Congenital abnormaleties of the uterus

84
CONGENITAL ANOMALIES OF THE UTEUUS PRESENTED BY: OUSMANE BECHIR HASSABALLAH IAN DONALD INTER SCHOOL FOLLOWSHIP OF ULTRASOUND Batch 6 10/1012015

Transcript of Congenital abnormaleties of the uterus

Page 1: Congenital abnormaleties of the uterus

CONGENITAL ANOMALIES OF THE UTEUUS

PRESENTED BY

OUSMANE BECHIR HASSABALLAH

IAN DONALD INTER SCHOOL FOLLOWSHIP OF ULTRASOUND

Batch 6

101012015

Introduction

Uterine malformations result from partial or

complete failure of one of three mechanisms

either separately or combined - agenesis

fusion and resorption Agenesis results in

either a complete absence of the uterus or a

unicornuate uterus a failure to fusion gives

rise to uterine didelphys or a bicornuate uterus

and a septated uterus is due to a failure of

resorption

incidence The incidence of muellerian anomalies has

historically varied widely due to the different

populations studied small sample sizes

prospective versus retrospective study designs

different classification systems and the type of

test used to make the diagnosis The need for a

standard classification of muellerian anomalies

was self-evident

In the general population the incidence of uterine

anomalies are estimated to be 3-4 (Sorensen 1988)

This increases to 73 in infertile patients ( Saravelos

2008)

Why is this important

Majority have no problem conceiving but

have higher rates of

ndash 1 Spontaneous Abortion

ndash 2 Premature Delivery

ndash 3 Infertility

ndash 4 Abnormal Fetal Lie

ndash 5 Dystocia at delivery

ndash 6 Dysmenorrhea endometriosis

ndash 7 Cervical incompetence

Uterine Anomalies

Absence of Uterus Fusion anomalies

Muumlllerian duct anomaly classification

The American Fertility Society (AFS)

classified muellerian anomalies according to

the major uterine anatomic types The AFS

classes of muellerian anomalies are

Hypoplasiaagenesis

Unicornuate

Didelphys

Bicornuate

Septate

Arcuate

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

The Muumlllerian duct anomaly classification is a

seven point system that can be used to describe a

number of embryonic Muumlllerian duct anomalies

class I uterine agenesisuterine hypoplasia

ndash a vaginal (uterus normal variety of

abnormal forms)

ndash b cervical

ndash c fundal

ndash d tubal

ndash e combined

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 2: Congenital abnormaleties of the uterus

Introduction

Uterine malformations result from partial or

complete failure of one of three mechanisms

either separately or combined - agenesis

fusion and resorption Agenesis results in

either a complete absence of the uterus or a

unicornuate uterus a failure to fusion gives

rise to uterine didelphys or a bicornuate uterus

and a septated uterus is due to a failure of

resorption

incidence The incidence of muellerian anomalies has

historically varied widely due to the different

populations studied small sample sizes

prospective versus retrospective study designs

different classification systems and the type of

test used to make the diagnosis The need for a

standard classification of muellerian anomalies

was self-evident

In the general population the incidence of uterine

anomalies are estimated to be 3-4 (Sorensen 1988)

This increases to 73 in infertile patients ( Saravelos

2008)

Why is this important

Majority have no problem conceiving but

have higher rates of

ndash 1 Spontaneous Abortion

ndash 2 Premature Delivery

ndash 3 Infertility

ndash 4 Abnormal Fetal Lie

ndash 5 Dystocia at delivery

ndash 6 Dysmenorrhea endometriosis

ndash 7 Cervical incompetence

Uterine Anomalies

Absence of Uterus Fusion anomalies

Muumlllerian duct anomaly classification

The American Fertility Society (AFS)

classified muellerian anomalies according to

the major uterine anatomic types The AFS

classes of muellerian anomalies are

Hypoplasiaagenesis

Unicornuate

Didelphys

Bicornuate

Septate

Arcuate

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

The Muumlllerian duct anomaly classification is a

seven point system that can be used to describe a

number of embryonic Muumlllerian duct anomalies

class I uterine agenesisuterine hypoplasia

ndash a vaginal (uterus normal variety of

abnormal forms)

ndash b cervical

ndash c fundal

ndash d tubal

ndash e combined

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 3: Congenital abnormaleties of the uterus

incidence The incidence of muellerian anomalies has

historically varied widely due to the different

populations studied small sample sizes

prospective versus retrospective study designs

different classification systems and the type of

test used to make the diagnosis The need for a

standard classification of muellerian anomalies

was self-evident

In the general population the incidence of uterine

anomalies are estimated to be 3-4 (Sorensen 1988)

This increases to 73 in infertile patients ( Saravelos

2008)

Why is this important

Majority have no problem conceiving but

have higher rates of

ndash 1 Spontaneous Abortion

ndash 2 Premature Delivery

ndash 3 Infertility

ndash 4 Abnormal Fetal Lie

ndash 5 Dystocia at delivery

ndash 6 Dysmenorrhea endometriosis

ndash 7 Cervical incompetence

Uterine Anomalies

Absence of Uterus Fusion anomalies

Muumlllerian duct anomaly classification

The American Fertility Society (AFS)

classified muellerian anomalies according to

the major uterine anatomic types The AFS

classes of muellerian anomalies are

Hypoplasiaagenesis

Unicornuate

Didelphys

Bicornuate

Septate

Arcuate

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

The Muumlllerian duct anomaly classification is a

seven point system that can be used to describe a

number of embryonic Muumlllerian duct anomalies

class I uterine agenesisuterine hypoplasia

ndash a vaginal (uterus normal variety of

abnormal forms)

ndash b cervical

ndash c fundal

ndash d tubal

ndash e combined

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 4: Congenital abnormaleties of the uterus

Why is this important

Majority have no problem conceiving but

have higher rates of

ndash 1 Spontaneous Abortion

ndash 2 Premature Delivery

ndash 3 Infertility

ndash 4 Abnormal Fetal Lie

ndash 5 Dystocia at delivery

ndash 6 Dysmenorrhea endometriosis

ndash 7 Cervical incompetence

Uterine Anomalies

Absence of Uterus Fusion anomalies

Muumlllerian duct anomaly classification

The American Fertility Society (AFS)

classified muellerian anomalies according to

the major uterine anatomic types The AFS

classes of muellerian anomalies are

Hypoplasiaagenesis

Unicornuate

Didelphys

Bicornuate

Septate

Arcuate

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

The Muumlllerian duct anomaly classification is a

seven point system that can be used to describe a

number of embryonic Muumlllerian duct anomalies

class I uterine agenesisuterine hypoplasia

ndash a vaginal (uterus normal variety of

abnormal forms)

ndash b cervical

ndash c fundal

ndash d tubal

ndash e combined

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 5: Congenital abnormaleties of the uterus

Uterine Anomalies

Absence of Uterus Fusion anomalies

Muumlllerian duct anomaly classification

The American Fertility Society (AFS)

classified muellerian anomalies according to

the major uterine anatomic types The AFS

classes of muellerian anomalies are

Hypoplasiaagenesis

Unicornuate

Didelphys

Bicornuate

Septate

Arcuate

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

The Muumlllerian duct anomaly classification is a

seven point system that can be used to describe a

number of embryonic Muumlllerian duct anomalies

class I uterine agenesisuterine hypoplasia

ndash a vaginal (uterus normal variety of

abnormal forms)

ndash b cervical

ndash c fundal

ndash d tubal

ndash e combined

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 6: Congenital abnormaleties of the uterus

Muumlllerian duct anomaly classification

The American Fertility Society (AFS)

classified muellerian anomalies according to

the major uterine anatomic types The AFS

classes of muellerian anomalies are

Hypoplasiaagenesis

Unicornuate

Didelphys

Bicornuate

Septate

Arcuate

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

The Muumlllerian duct anomaly classification is a

seven point system that can be used to describe a

number of embryonic Muumlllerian duct anomalies

class I uterine agenesisuterine hypoplasia

ndash a vaginal (uterus normal variety of

abnormal forms)

ndash b cervical

ndash c fundal

ndash d tubal

ndash e combined

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 7: Congenital abnormaleties of the uterus

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

The Muumlllerian duct anomaly classification is a

seven point system that can be used to describe a

number of embryonic Muumlllerian duct anomalies

class I uterine agenesisuterine hypoplasia

ndash a vaginal (uterus normal variety of

abnormal forms)

ndash b cervical

ndash c fundal

ndash d tubal

ndash e combined

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 8: Congenital abnormaleties of the uterus

class II unicornuate uterusunicornis

unicollis ~6-25

ndash a communicating contralateral rudimentary

horn contains endometrium

ndash b non-communicating contralateral

rudimentary horn contains endometrium

ndash c contralateral horn has no endometrial

cavity

ndash d no horn

ndash class III uterus didelphys ~5-11

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 9: Congenital abnormaleties of the uterus

Muumlllerian duct anomaly classificationDr Ayush Goel and Dr Frank Gaillard et al

class IV bicornuate uterus next commonest

type ~10-39

ndash a complete division all the way down to

internal the os

ndash b partial division not extending to the os

class V septate uterus commonest anomaly

~34-55

ndash a complete division all the way down to the

internal os

ndash b incomplete division

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 10: Congenital abnormaleties of the uterus

class VI arcuate uterus ~7

class VII in utero Diethylstilbestrol (DES)

exposure T shaped uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 11: Congenital abnormaleties of the uterus

Classification into 4 groups

1 Agenesis of uterusvagina Rokitansky-

Kuster-Hauser Syndrome

2-Unilateral development Unicornate

uterus

3 Lateral Fusion defects (obstructive or

non-obstructive)

4-Defects in Vertical Fusion (obstructive or

non-obstructive)

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 12: Congenital abnormaleties of the uterus

Uterine agenesis

is the extreme of Mullerian duct anomalies

(Class I) where there is complete absence of

uterine tissue above the vagina

Epidemiology

The uterine agenesis-hypoplasia spectrum

accounts for ~10-15 of all Muumlllerian duct

anomalies

Clinical presentation

Clinical presentation is characterised by primary

amenorrhoea with normal hormonal levels

guaranteed by fully functional gonads

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 13: Congenital abnormaleties of the uterus

Pathology

Complete absence of the Mullerian ducts is

termed Mayer-Rokitansky-Kuster-Hauser

(MRKH) syndrome which includes absence

of the vagina as well

Associations

renal tract anomalies including renal

agenesis

skeletal vertebral anomalies

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 14: Congenital abnormaleties of the uterus

The uterus is small measuring just 38 x 12 cm hypoplasia

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 15: Congenital abnormaleties of the uterus

Figure 2 Pelvic ultrasound longitudinal showing the urinary bladder and

absent uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 16: Congenital abnormaleties of the uterus

Figure 3 Pelvic ultrasound showing an ectopic pelvic kidney indenting

on the urinary bladder superiorly

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 17: Congenital abnormaleties of the uterus

Figure 4 Intravenous urography showing pevicaeal system of the ectopic

kidney

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 18: Congenital abnormaleties of the uterus

Figure 5 Enhanced axial CT of the pelvis showing contrast in the urinary

bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 19: Congenital abnormaleties of the uterus

Sagittal and transverse ultrasound images show a markedly hypoplastic

uterus measuring less than 48 x 16 x 27 cm in a middle-aged female

patient with primary infertility

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 20: Congenital abnormaleties of the uterus

Unicornuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

A unicornuate uterus or unicornis unicollis is a

type of Mullerian duct anomaly (class II) This

type can account for ~10 (range 6-13) of

uterine anomalies and infertility is seen in

~125 (range 5-20) of cases

Pathology

There is failure of one muumlllerian duct to elongate

while the other develops normally The

embryologic predominance of the unicornuate

uterus to be on the right has not been explained It

may or may not have rudimentary horn

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 21: Congenital abnormaleties of the uterus

Sub-classification

It can be classified into to the following types

according to the

type a with rudimentary horn the American

Fertility Society 3

ndash a1 horn contains endometrium

a1a communicating contralateral

rudimentary horn contains endometrium

(10)

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 22: Congenital abnormaleties of the uterus

a1b non-communicating contralateral

rudimentary horn contains

endometrium (22)

ndash a2 contralateral horn has no endometrial

cavity (33)

type b no horn (35)

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 23: Congenital abnormaleties of the uterus

Associations

renal abnormalities

ndash renal anomalies are more commonly

associated with a unicornuate uterus than

with other muumlllerian duct anomalies and are

present in 40 of cases eg renal agenesis

ndash the renal anomaly is always ipsilateral to the

rudimentary horn

cryptomenorrhea within endometrium

containing rudimentary horn that does not

communicate with the endometrial cavity

primary infertility 4

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 24: Congenital abnormaleties of the uterus

Ultrasound

Can be diffcult to detect on 2D ultrasound

The uterus may be seen tapering to one side

Hysterosalpingogram (HSG)

MRI

Radiographic features

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 25: Congenital abnormaleties of the uterus

Complications

Of the Mullerian duct anomalies a

unicornuate uterus is considered to have the

second worse obstetric outcome (worst with

a septate uterus)

Spontaneous abortion rates are reported to

range from 41-62 Reported premature

birth rates range from 10-20 Fetal

survival rate is ~40 (range 38-57)

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 26: Congenital abnormaleties of the uterus

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the

coronal plane showing the characteristic fusiform banana-shaped uterus

with a single left horn and a single endometrial cavity

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 27: Congenital abnormaleties of the uterus

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 28: Congenital abnormaleties of the uterus

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus

The cavity (at C) only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 29: Congenital abnormaleties of the uterus

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 30: Congenital abnormaleties of the uterus

Unicornuate uterus A multiplanar display of the left unicornuate uterus is

shown This diagnosis is difficult to establish with 2DUS because the uterus

may appear grossly normal or slightly laterally deviated With 3DUS the

diagnosis is confidently made because the coronal plane (C) shows clearly that

there is only a single cornual angle (arrow) The cervical canal has a normal

appearance A Axial view B sagittal view C coronal view

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 31: Congenital abnormaleties of the uterus

Unicornuate Uterus Ultrasound Pregnant uterus ndash fetus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 32: Congenital abnormaleties of the uterus

Uterus didelphysDr Jeremy Jones and Dr Frank Gaillard et al

is a type of Muumlllerian duct anomaly (class III)

where there is complete duplication of uterine horns

as well as duplication of the cervix with no

communication between them

Epidemiology

Didelphic uteri account for approximately ~8

(range 5-11) of Muumlllerian duct anomalies

Clinical presentation

Many patients are asymptomatic although some

may occasionally experience dyspareunia as a result

of the vaginal septum

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 33: Congenital abnormaleties of the uterus

Pathology

It results from failed ductal fusion that occurs

between the 12th and 16th week of pregnancy and

is characterized by two symmetric widely

divergent uterine horns and two cervices The

uterine volume in each duplicated segment is

reduced As with most uterine anatomical

anomalies there is an increased incidence of

fertility issues and Muumlllerian abnormalities in

general are over represented in infertile women

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 34: Congenital abnormaleties of the uterus

The chance of seeing a pregnancy to term is

significantly reduced down to only 20

with a third of pregnancies ending in

abortion and over half in premature

deliveries Only 40 of pregnancies

resulted in living children 2

Along with unicornuate uterus uterus

didelphys has the greatest impact on

reproductive performance reference required

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 35: Congenital abnormaleties of the uterus

Associations

renal agenesis

vaginal septum which can include transverse

vaginal septum

ndash there is a vaginal septum in 75 of cases and

obstruction to one horn is possible from occasional

transverse septae

Radiographic features

Classically shows two widely spaced uterine corpora

each with a single Fallopian tube Separate divergent

uterine horns with large fundal cleft (as distinct from

a septate uterus)

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 36: Congenital abnormaleties of the uterus

Hysterosalpingogram (HSG)

Pelvic ultrasound

Separate divergent uterine horns are

identified with a large fundal cleft

Endometrial cavities are uniformly separate

with no evidence of communication Two

separate cervices need to be documented

MRI

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 37: Congenital abnormaleties of the uterus

2-dimensional image of a didelphic uterus with a

gestational sac (arrow) in the left horn

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 38: Congenital abnormaleties of the uterus

Ultrasound image showing duplication of uterus corpus characterizes this

Mullerian Duct Anomaly Didelphys uterus (Type III) or Complete

bicornuate (Type IVa) Ultrasound scan

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 39: Congenital abnormaleties of the uterus

Complications

infertility

unilateral hydrocolpos haematocolpos (if a

vaginal septum is present)

endometriosis

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 40: Congenital abnormaleties of the uterus

Bicornuate uterusDr Frank Gaillard et al

A bicornuate uterus is a type of uterine

duplication anomaly It can be classified as

a class IV Mullerian duct anomaly

Epidemiology

Overall congenital uterine anomalies occur in

~15 of females (range 01-3) Bicornuate

uteri are thought to represent ~25 (range 10-

39) of Mullerian duct anomalies

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 41: Congenital abnormaleties of the uterus

Clinical presentation

In most cases a bicornuate uterus is

incidentally discovered when the pelvis is

imaged The most common symptomatic

presentation is with early pregnancy loss

and cervical incompetence 6

Pathology

A bicornuate uterus results from an

abnormal development of the

paramesonephric ducts There is a partial

failure of fusion of the ducts resulting in a

uterus divided into two horns

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 42: Congenital abnormaleties of the uterus

Associations

associated longitudinal vaginal septum may be

present in ~25 of cases

as with other Mullerian duct anomalies

abnormalities of the renal tract may also be

present

Subtypes

Bicornuate uterus is divided according to the

involvement of the cervical canal

bicornuate bicollis - two cervical canals central

myometrium extends to external cervical os

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 43: Congenital abnormaleties of the uterus

bicornuate unicollis - one cervical canal

central myometrium extends to internal

cervical os

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 44: Congenital abnormaleties of the uterus

Radiographic features

General

The preferred methods of imaging uterine

anomalies are ultrasound

hysterosalpingogram or MRI The external

uterine contour is concave or heart-shaped

and the uterine horns are widely divergent

The fundal cleft is typically more than 1cm

deep and the intercornual distance is

widened

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 45: Congenital abnormaleties of the uterus

The uterus is seen as comprising of caudally

fused symmetric uterine cavities with some

degree of communication between the two

cavities (usually at the uterine isthmus) Although

not a specific finding the angle between the

horns of the bicornuate uterus is usually more

than 105deg

Fluoroscopy - hysterosalpinogram (HSG)

A divided uterus can be seen but it is difficult to

differentiate between septate and bicornuate

anomalies since the uterine fundal contour is not

visible

MRI

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 46: Congenital abnormaleties of the uterus

Treatment and prognosis

Surgical intervention is usually not indicated in

absence of reproductive difficulties

In women with a history of recurrent pregnancy

loss and in whom no other infertility issues have

been identified a Strassman metroplasty can be

considered

In patients with cervical incompetence

placement of a cervical cerclage may increase

fetal survival rates 9 Indeed the association

between cervical incompetence and bicornuate

uterus is so high that prophylactic cerclage may

be appropriate in some instances

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 47: Congenital abnormaleties of the uterus

Practical points

Septate uterus increases the risk of early

pregnancy loss and hysteroscopic

intervention to resect the septum is

sometimes pursued In this situation

differentiation between a septate uterus or a

bicornuate uterus is critical outcome

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 48: Congenital abnormaleties of the uterus

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 49: Congenital abnormaleties of the uterus

Surface rendering of a bicornuate uterus The serosal indentation

(arrow) is evident

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 50: Congenital abnormaleties of the uterus

Bicornuate uterus with unilateral pregnancy

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 51: Congenital abnormaleties of the uterus

Septate uterusDr Matt A Morgan and Dr Natalie Yang et al

A septate uterus is a common type of congenital

uterine anomaly and it may lead to an increased rate

of pregnancy loss The main imaging differential

diagnoses are arcuate uterus and bicornuate uterus

Epidemiology

It is considered the commonest uterine anomaly

(accounts for ~55 of such anomalies) It is classified

as a class V Mullerian duct anomaly Septate uterus

is the most common anomaly associated with

reproductive failure (67)

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 52: Congenital abnormaleties of the uterus

Pathology

Septate uterus is considered a type of uterine

duplication anomaly It results from partial or

complete failure of resorption of the uterovaginal

septum after fusion of the para-mesonephric

ducts The septum is usually fibrous but can also

have varying muscular components

Subtypes

a partial septum (sub-septate uterus) involves the

endometrial canal but not the cervix

a septum is considered complete if it extends to

either the internal or external cervical os 10

septum extends into the vagina septate uterus and

vagina

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 53: Congenital abnormaleties of the uterus

Associations

As with other uterine anomalies concurrent

renal anomalies may be associated

Radiographic features

General

the external uterine fundal contour may be

convex flat or mildly (lt 1 cm) concave

acute angle lt75 degrees between uterine

cavities

endometrial canals are completely separated by

tissue iso-echoic to myometrium with

extension into endocervical canal

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 54: Congenital abnormaleties of the uterus

Ultrasound

The echogenic endometrial stripe is separated at the

fundus by the intermediate echogenicity septum

The septum extends to the cervix in a complete

septate uterus The external uterine contour must

demonstrate a convex flat or mildly concave

(ideally lt1cm) configuration and may best be

appreciated on coronal images of the uterus

Colour Doppler

May show vascularity in the septum in 70 of

cases and if present may be associated with a higher

rate of obstetric complications

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 55: Congenital abnormaleties of the uterus

Fluoroscopy - Hysterosalpingogram

Accuracy of hysterosalpingogram alone is

only 55 for differentiation of septate uterus

from bicornuate uterus

Pelvic MRI

MRI is considered the current imaging

modality of choice

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 56: Congenital abnormaleties of the uterus

Complications

90 miscarriage rate

ndash a patient with a septate uterus does not

usually not have difficulty conceiving but

a septate uterus is associated with the

highest rate of pregnancy loss of the

Muumlllerian duct anomalies

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 57: Congenital abnormaleties of the uterus

Treatment and prognosis

The distinction between septate uterus and bicornuate

uterus has important management implications In

septate uterus but not in bicornuate uterus the

septum can be shaved off during hysteroscopy

(metroplasty) to form a single uterine cavity without

perforating the uterus 4

Reproductive outcome has been shown to improve

after resection of the septum with reported decreases

in the spontaneous abortion rate from 88 to 59

after hysteroscopic metroplasty

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 58: Congenital abnormaleties of the uterus

Differential diagnosis

Considerations a hysterosalpingogram include

bicornuate uterus the shape of the external

uterine contour is crucial to differentiate a

septate uterus from a bicornuate uterus because

widely different clinical and interventional

approaches are assigned to each anomaly

On ultrasound or MRI images also consider

arcuate uterus small myometrial indentation in

the fundus with normal fundal contour

thick intra-uterine adhesion band

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 59: Congenital abnormaleties of the uterus

Septate Uterus

Most COMMON anomaly 55

May be complete incomplete

bull25 early abortions

bull5 - 7 late abortions amp Premature labors

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 60: Congenital abnormaleties of the uterus

3-dimensional coronal view of a septated uterus (a) distance

between ostia (+ +) (b) length of septum (x x)

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 61: Congenital abnormaleties of the uterus

(12 weeks) Longitudinal section of the fetus showing its location belong the

uterine septum within the amniotic cavity that had spread to the both uterine

horns

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 62: Congenital abnormaleties of the uterus

The fetal head situated in the right uterine horn

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 63: Congenital abnormaleties of the uterus

Transverse plane through the uterus The uterine septum is hypoechoic and

hypovascular At this level the placenta (PL) begins to spread on the posterior

wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 64: Congenital abnormaleties of the uterus

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-September

20115(3)243-256

Figs A to C Septate uterus seen on 3D VCI-C (A) septal vessels on 3D

power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 65: Congenital abnormaleties of the uterus

Arcuate uterusDr Yuranga Weerakkody and Dr Natalie Yang et al

An arcuate uterus is a mildly variant shape of

the uterus It is technically one of the Muumlllerian

duct anomalies but is often classified as a

normal variant It is the uterine anomaly that is

least commonly associated with reproductive

failure Arcuate uterus can be characterized with

ultrasound or MRI

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 66: Congenital abnormaleties of the uterus

Pathology

An arcuate uterus is

characterized by a mild

indentation of the

endometrium at the

uterine fundus It occurs

due to a failure of

complete resorption of

the uterovaginal septum

It is the most common

Mullarian duct anomaly

affecting 39 of the

general population

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 67: Congenital abnormaleties of the uterus

Radiographic features

General features include

normal fundal contour

no division of uterine horns

smooth indentation of fundal endometrial

canal - the depth of indentation is usually

considered to be lt1 cm

increased transverse diameter of uterine

cavity

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 68: Congenital abnormaleties of the uterus

Pelvic ultrasound

A normal external uterine

contour is noted with a

broad smooth indentation

on the fundal segment of

the endometrium No

division of the uterine

horns

Hysterosalpingogram

MRI

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 69: Congenital abnormaleties of the uterus

Differential diagnosis

septate uterus

ndash arcuate uterus and septate uterus exist on a

spectrum from least to most resorption of

the uterovaginal septum respectively

bicornuate uterus

ndash arcuate uterus can be distinguished from

a bicornuate uterus on the basis of its complete

fundal unification (ie the arcuate uterus has a

normal or slightly indented external fundal contour

whereas the bicornuate has a more marked fundal

indentation no more than 5 mm above the level of

the uterine horns)

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 70: Congenital abnormaleties of the uterus

Figs A to E Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed

coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode

(E)

Donald School Journal of Ultrasound in Obstetrics and Gynecology July-

September 20115(3)243-256

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 71: Congenital abnormaleties of the uterus

RKH Syndrome Diagnosis

Expected Menarche

Difficult to differentiate from imperforate

hymen

No uterus on exam US MRI

Laparoscopy IVP

Confused with Androgen Resistance

Syndrome with shallow pouch and no

uterus

Determine karyotype

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 72: Congenital abnormaleties of the uterus

Abnormalities of the

ovaries

1) agenesis or complete absence

2) Gonadal dysgenesis streak

gonads as in Turner syndrome

3) Failure of descent into the pelvis

4) Ovotestis ldquotrue hermaphroditerdquo

In which combined ovarian and

testicular tissues seen

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 73: Congenital abnormaleties of the uterus

conclusion

All uterine anomalies negatively affect the live

birth rate and result in a higher frequency of

obstetrical complications Depending upon

the type of muellerian anomaly cervical

incompetence spontaneous miscarriage

preterm delivery breech presentation

abnormal fetal lie and intrauterine growth

restriction are all increased to a variable

degree 167

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 74: Congenital abnormaleties of the uterus

3DUS enables the measurement of the length

of a uterine septum amp depth of fundal cleft

In addition 3D ultrasound in diagnosing

uterine congenital malformations has been

found to be a reproducible method(Salim 2003)

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 75: Congenital abnormaleties of the uterus

While transvaginal sonography is an excellent

screening examination for uterine anomalies it is

not as effective as 3D ultrasound in distinguishing

specific malformations For example Jurkovic et al

20 reported a 100 sensitivity and specificity for

the three-dimensional ultrasound detection of

uterine anomalies in contrast to 100 sensitivity

and 95 specificity for two-dimensional

ultrasound However the positive predictive value

of three-dimensional and two-dimensional

ultrasound for muellerian anomalies was 100 and

50 respectively

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 76: Congenital abnormaleties of the uterus

MRI has been the gold standard for categorizing

uterine anomalies because of its 98 - 100 accuracy

While ultrasound will remain the primary modality

utilized to evaluate muellerian anomalies MRI can

offer additional diagnostic information in patients with

equivocal ultrasound findings As a result laparoscopy

or open surgery are no longer required to make a

definitive diagnosis of a uterine anomaly

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 77: Congenital abnormaleties of the uterus

Thank you

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 78: Congenital abnormaleties of the uterus

At 6TH week gestation

Paramesonephric

or Mullerian Duct

develops lateral to

the Mesonephric

rdquowolffian ldquo Duct

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 79: Congenital abnormaleties of the uterus

The middle and caudal parts of

the Mullerian ducts undergoes

medial migration and fusion

The cranial 13 rarr tubes

The middle 13 rarr uterus and

cervix

Caudal 13 rarr upper 34 of

vagina

Development of the vagina

Page 80: Congenital abnormaleties of the uterus

Development of the vagina