Amenorrhea

• DR Manal Behery

• Professor OB&GYNE

• Zagazig University

• 2014

Amenorrhea

Absence Of Menstruation.

ORIGIN from Greek

Classification of amenorrhea

AMENORRHEAAMENORRHEA

PHYSIOLOGICALPHYSIOLOGICAL PATHOLOGICAL

Pre-pubertyPregnancy relatedMenopause

Primary

Secondary

Events of Puberty

•1-Thelarche :

• the breast development

• Requires estrogenestrogen

•2-Pubarche/adrenarche :

pubic hair development

Requires androgensandrogens

Stage1 stage2

Prepupertal: elevation of papilla- no pubic hair

Elevation of breast and papilla on a small mount,increase in areola – libial hair

Marshall and tunner staging

Stage 3 stage4

Further breast enlargment,

hair extend to mons pubis

Secondary mound of areola and papilla ,hair extend laterl

And increases pigmention

Stage5

Recession of arola to contour of the breast

Adult pubertal hair with striaght upper borded

Requires:

GnRHGnRH from the hypothalamus

FSHFSH and LHLH from the pituitary

Estrogen and progesteroneEstrogen and progesterone from the ovaries

Normal outflow tract

Events Of Puberty

3-Menarche :

Hypothalamo-Pituitary-Ov-Ut Axis

CNSHypothalamus

Pituitary

Ovary

UterusOutflow tract

The Hypothalamic-Pituitary-Ovarian Axis

http://www.shen-nong.com/eng/images/exam/missedperiods/img_mp1a.gif

CNS-hypothalamus-pituitaryovary-uterus interaction

Neural control Chemical control

Dopamine)-(

Norepinephrine)+(

Endorphins)-(

Hypothalamus

GnRH

Ant. pituitary

FSH, LH

Ovaries

Uterus

ProgesteroneEstrogen

Menses

–± ?

Functional menses

Compartment ICompartment Ioutflow tract ( uterine target

organ)

Compartment ICompartment IIIOvary ( Estrogen -Progesterone)

Compartment IIICompartment IIIAnterior Pituitary

Compartment IVCompartment IVCNS ( Hypothalamic )

Clinically

PrimaryPrimary SecondarySecondary

Common causes of Amenorrhea

Primary  • Gonadal failure )45%( • Congenital absence of uterus and vagina )20%(

• Constitutional delay )15%(

Secondary 

Chronic anovulation )40%(

• Hypothyroidism / hyperprolactinemia )20%( • Weight loss/anorexia )16%(

Constitutional pubertal delay

• Common cause (15%) Positive family history

• Under stature and delayed bone age ( X-ray Wrist joint)

•• Diagnosis by exclusion and follow up

• Prognosis is good(late developer)

• No drug therapy is required – Reassurance (? HRT)

primary amenorrhea

Evaluation Categories

• 1-Breast Absent – Uterus Present

• 2-Breast Present – Uterus Absent

• 3-Breast Present – Uterus Present

• 4-Breast Absent – Uterus Absent

46 XX

Typical features of Turner Syndrome Turner Syndrome 1st commen cause (45% of causes )1st commen cause (45% of causes )

Turner's S.

•A craniopharyngioma is a benign tumor that develops near the pituitary gland.

• most commonly in childhood and adolescence and

•in later adult life.compresses the pituitary stalk or gland, the tumor can cause partial or complete pituitary hormone deficiency.

• Family history: Consider watchful waiting

• Request: FSH, LH- Raised: Karyotype: 45 XO Turner syn

46 XX Premature ovarian failure

- Low: Constitutional delayConsider: anorexia

exerciseillnesscoeliac diseasehypothalamic/pituitary

- Intermediate: Anatomical - ultrasound

Secondary sexual characteristicsabsent 14y

Mayer-R )okitansky-Kuster-Hauser syndrome(

Utero-vaginal Agenisis Mayer-Rokitansky-Kuster-Hauser syndrome

• Second most common cause of Primary amenorrhea.

• Normal breasts and Sexual Hair Normal looking external female genitalia

• Karyotype 46-XX

• 15-30% renal abnormalities.

• Treatment : Vaginal creation (Dilatation VS Vaginoplasty)

• Normal breasts but no sexual hair

• Normal looking female external genitalia

• Absent uterus and upper vagina

• Karyotype 46, XY

• Male range testosterone level

• Treatment : gonadectomy after puberty + HRT

Androgen insensitivityTesticular feminization syndrome

• Absent/abnormal then karyotype: -46 XX Mullerian agenesis

-46 XY Androgen insensitivity

• Present • +no outflow obstruction

-As for 2o amenorrhoea

Secondary sexual characteristics Present by 16 yearsUltrasound uterus

1-Rule out pregnancy!

2-Exclude cryptomenohrea

✴1-Pregnancy

✴2-Cryptomenorrhea :imperforated hymen, vaginal septum,

✴3-Causes 2nd ry Amenorrhea :hypothalamic, pituitary ,other endocrionpathy

Very rare

17,20 -desmolase deficincy

agonadieisim

17 alfa-hydroxylase deficincy (46, XY )

Compartment I: Disorders of the Outflow Tract

• Eugonadism

MRI: complete low transverse septum with obstruction

MRI: complete slightly higher transverse septum with obstruction

Compartment II: Disorders of the Ovary

• Hypergonadotropic hypogonadism

Compartment III: Disorders of the Anterior Pituitary

• Hypogonadotropic hypogonadism

Microadenoma vs macroadenoma

Compartment IV: Hypothalamic (Kallmann’s syndrome) • Hypogonadotropic

hypogonadism

• Congenital disorder characterized by:

• 1) Anosmia or hyposmia

• 2) Primary amenorrhea

• Caused by defect in synthesis and/or release of gonadorelin (LH releasing hormone)

History and physical examination completed for a patient with primary amenorrhea

Secondary sexual characteristics present

No Yes

Measure FSH and LH levels

Uterus absent or abnormal

Uterus present or normal

Karyotype analysis Outflow obstruction

FSH and LH < 5 IU/ L

Hypogonadotropic hypogonadism

Hypergonadotropichypogonadism

Karyotype analysis 46 ,XY

46 ,XX

Androgen Sensitivity Syndrome

NoYes

Evaluate for

secondary amenorrhea

Imperforate hymen or transverse

vaginal septum

Perform ultrasonography of uterus

Evaluation of Primary Amenorrhea

FSH > 20 IU/ L and

LH > 40 IU/ L

Secondary amenorrhea

In women of reproductive age, pregnancy is the most common cause of secondary amenorrhea .

Pregnancy

The reality of this must be ascertained before any intervention is instituted for non-obstetric amenorrhea.

Compartment I OCompartment I Outflow tract ( uterine target organ)

pregnancy

Asherman's Syndrome

Compartment II Compartment II Ovary

PCO

Premature Ovarian Failure

Resistance Ovarian Syndrome

Radiation & Chemotherapy .

(Hypergonadotropic Hypogonadism )

Polycystic ovary syndrome• The most common cause of chronic

anovulation

Classic 45-XO Premature ovarian failure

Turner’s syndrome Mosaic )46-XX / 45-XO(

Compartment III Compartment III Anterior Pituitary

Hyper – prolactinemia

Tumors

Sheehan Syndrome

Compartment IV Compartment IV CNS ( Hypothalamic )

Hypothyrodism

Stress anxiety

Anorexia

Excessive Exercise

Drugs

Anorexia NervosaAnorexia nervosa

• A psychological disease characterized by

• Intense fear of gaining weight or being fat, despite being underweight

• Disturbance in one’s experience of body weight, size, and shape

• the refusal to maintain normal body weight, and amenorrhea

V. Others1.Thyroid hypo or hyperthyroidism

2. Adrenal disease Adrenogenital SCushing SAddison s disease

3. Ch. illness, badly controlled DM.

HistoryHistoryA good history can reveal the etiologic diagnosis A good history can reveal the etiologic diagnosis

in up to 85% of cases of amenorrhea.in up to 85% of cases of amenorrhea.

ASSESSMENTASSESSMENT

Present History

1.Sexual activity, risk of pregnancy ,

2.Type of contraceptive used .

3.Galactorrhoea

4.Androgenic symptoms: weight gain, acne, hirsutism

5.Menopausal symptoms: night sweats, hot flushes

6.Issues with eating or excessive exercise .

Past history

1.Drug use: Dopamine antagonists for psychiatric conditions. Antihypertensive,anticonsulsionvant

Genital tract surgery: intrauterine instrumentation

3 --radio or chmotherrapy

4 -infection : mumps or TB oophritis

Clinical assessment

1 -β - hCG

2 -TSH

3 -Prolactin

TOP

Bottom

1. Provera 10 mg PO once daily 7-10 days or

2. Norethindrone 5 mg PO once daily for 7-10 days or

3. Progesterone 200 mg IM for one dose .

Progesterone Challenge Test :

Step 1

1. Premarin 1.25 mg orally daily for 21 days

2. Oral Contraceptive for 2 Cycles

3. Estradiol 2 mg orally daily for 21 days and Follow with 7-10 days of Progesterone

Estrogen progesterone challenge test

Step 2

• Asharman syndrome)intrauterine synechea)

Step 3

TreatmentI. Treatment of the cause.1-if Y chromosome is present gonadectomy is

indicated2- Create outflow tract or at least a sexually functional

vagina

II. No obvious cause:

. Conception is not required: COCP.

. Conception is required: induction of ovulation

Thanks