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AMENORRHEAAMENORRHEA

Prof.DR.Dr.H.M.Thamrin Tanjung, Sp.OG(K)Dr.M.Rusda Harahap, Sp.OG

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Defnitions

Primary amenorrhea

Failure of menarche to occur when expected in

relation to the onset of pubertal development.

No menarche by age 16 years with signs of pubertal

development.

No onset of pubertal development by age 14 years.

Secondary amenorrhea

Absence of menstruation for 3 or more months in a

previously menstruating women of reproductive

age.

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CNS-Hypothalamus-Pituitary

Ovary-uterus InteractionNeural control Chemical control

Dopamine

-!

Norepiniphrine

"!

En#orphines

-!

Hypothalamus

Gn-RH

Ant$ pituitary

%SH& 'H

Ovaries

(terus

Pro)esteroneEstro)en

Menses

?

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Pathophysiolo)y o*

Amenorrhea

Inadequate hormonal stimulation of the endomeriumAnovulatory amenorrhea

- Euestrogenic

- Hypoestrogenic

Inability of endometrium to respond to hormonesOvulatory amenorrhea

- Uterine absence - Utero-vaginal agenesis- XY-Females ( e.g T.F.S)

- amage! en!ometrium ( e.g "s#erman$s syn!rome)

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Euestro)enic Anovulatory

AmenorrheaNormal androgens Hypothalamic-pituitary

dysfunction (stress !eight

loss or gain e"ercisepseudocyesis#

Hyperprolactinemia

$emini%ing ovarian tumour

Non-gonadal endocrinedisease (thyroid adrenal#

&ystemic illness

High androgens 'O&

)usculini%ing ovariantumour

ushing*s syndrome

ongenital adrenalhyperplasia (late onset#

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Hypoestro)enicAnovulatoryAmenorrhea

Normal androgens- Hypothalamic-pituitary failure

- &evere dysfunction - Neoplasticdestructive infiltrative infectious + trumatic conditions involving hypothalamus or

pituitary- Ovarian failure

-,onadal dysgenesis - 'remature ovarian failure

- n%yme defect - .esistant ovaries - .adiotherapy chemotherapy

High androgens

- )usculini%ing ovarian tumour- ushing*s syndrome

- ongenital adrenal hyperplasia(late onset#

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A)NO..HOA

AN APPROACH FOR DIAGNOI H%ST&'Y

HYS%"* EX"+%,"T%&,

U*T'"S&U, EX"+%,"T%&,

Eclu!e regnancy

Eclu!e ryptomenorr#ea

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ryptomenorrhea

!utflow obstruction to menstrual blood

- Imperforate hymen- Transverse Vaginal septum with functioning

uterus- Isolated Vaginal agenesis with functioning

uterus

- Isolated Cervical agenesiswith functioninguterus

- Intermittent a+#ominal pain- Possi+le #i,culty ith micturition

- Possi+le loer a+#ominal sellin)

-.ul)in) +luish mem+rane at the introitus ora+sent

va)ina only #imple!

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Imper*orate hymen

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Once Pre)nancy an# cryptomenorrheaare e/clu#e#:

0he patient is a +ioassay *or En#ocrine a+normalities

%our cate)ories o* patients arei#entife#

1$ Amenorrhea ith a+sent orpoor secon#ary se/Characters

2$ Amenorrhea ith normal2ry

se/ characters3$ Amenorrhea ith si)ns o* an#ro)en e/cess

4$ Amenorrhea ith a+sentuterus

an# va)ina

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%SH Serum level

'o 5normal

Hi)h

Hypo)ona#otropichypo)ona#im 6ona#al#ys)enesis

AMENORRHEAA+sent or poor secon#ary se/

Characteristics

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AMENORRHEANormal secon#ary se/

Characteristics

- $&H /H 'rolactin 0&H-'rovera 12 mg 'O daily

" 3 days

4 5leeding No bleeing'rolactin0&H

$urther

6or7-up

(ndocrinologist#

- )ild hypothalamic

dysfunction

- 'O (/H8$&H# .evie! $&H result

And history (ne"t slide#

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%SH

'o 5 normalHi)h

Hypothalamic-pituitary%ailureOvarian*ailure

If 9 :3 yrs or primary

amenorrhea 7aryoptype

If 9 ;3 yrs .8O

autoimmune disease

77 Ovarian+iopsy

head CT- scan or MRI

- Severe #ypot#alamic

!ysunction

- %ntracranial pat#ology

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Amenorrhea

(tero-va)inal a+sence

8aryotype

49-::

Mullerian

A)enesisMR8H syn#rome!

An#o)en

Insenitivity0S%syn#rome!

$ 6ona#al

re)ressioon$ 0estocular en;yme

#e*enciecy$ 'ey#i) cella)enisis

49-:

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Normal %SH& 'H> -ve +lee#in)history is suggestive of amenorrhea

trumaticaAshermans syndrome

istory of pregnancy associated !"C

Rarely after C# $ myomectomy T%&endometritis$ 'ilhar(ia

!iagnosis ) #* or hysterescopy

Treatment ) lysis of adhesions+ !"C orhysterescopy , estrogen therapy . I/C!or catheter0

#ome will prescri'e a cycle of 1strogen and

2rogesterone challenge &efore #* orysterescopy

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Asherman?s syn#rome

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Amenorrhea

Si)ns o* an#ro)en e/cessTestosterone, DHEAS, FSH, and LH

DHEAS 500-700 mug/dLDHEAS 700 mug/dLTEST! "00 ng/dL

Serum #7-$H

%rogesterone &e'e&

Late (AHAdrena&

)*+erunt.on

/S ? R1 or (T

$'ar.an$r adrena&

tumor

Lo2er e&e'at.ons %($S 3H.g) LH / FSH4

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Amenorrhea

'.I)A.< A)NO..HA

= Ovarian failure ;>?

= Hypogonadotrophic ;@?

Hypogonadism=

= 'O& 1?

= ongenital lesions

(other than dysgenesis# @?= Hypopituitarism ;?

= Hyperprolactinaemia ;?

= 6eight related ;?

&ONBA.< A)NO..HA

= 'olycystic ovary syndrome ;2?

= 'remature ovarian failure :C?

= 6eight related amenorrhoea 1C?

= Hyperprolactinaemia 1@?

= "ercise related amenorrhoea :?

= Hypopituitarism :?

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6ona#al #ys)eneis

Chromosomally incompetent

- Classic turners syndrome 34560

- Turner variants 3456738550$385-a'normal 50 - Mi9ed gonadal dygenesis 34567385:0

Chromosomally competent

- 3855 2ure gonadal dysgeneis0

- 385: #wyers syndrome0

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6ona#al #ys)enesis

(&ass.

Turners

Turner

6ar.ant

True gonada&

D*sgenes.s

.ed

D*sgenes.s

+)enot*+e Fema&e Fema&e Fema&e Am8.guous

Gonad Strea9 Strea9 Strea9 - Strea9- Testes

H.g)t S)ort - S)ort

- :orma&

Ta&& S)ort

Somat.st.gmata

(&ass.a& :.&

9ar*ot*+e ;$ ;;/;$ ora8norma& ;

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0urner?s syn#rome

#e9ual infantilism and short stature%

Associated a'normalities$ we''ednec;$coarctation of the aorta$high-arched pallate$cu'itus valgus$ 'road shield-li;e chest with

wildely spaced nipples$ low hairline on the nec;$short metacarpal 'ones and renal anomalies%

igh aryotype - ?@ 34$ 5@- B@ mosaic forms 38557345@0

Treatment) RT

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osa. 3

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Ovarian #ys)enesis

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None-#ys)enesis ovarian

*ailure #teroidogenic en(yme defects D-

hydro9ylase0

6varian resistance syndrome Autoimmune oophoritis

2ostinfection eg% Mumps0

2ostoopherectomy 2ostradiation

2ostchemotherapy

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Premature ovarian *ailure

#erum estradiol E 4@ pg7ml and

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Polycystic ovary syn#rome

The most common cause of chronic anovulation

yperandrogenism + =7

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Hypo)ona#otrophic

Hypo)ona#ismNormal hight

D Normal e"ternal and internalgenital organs (infantile#

D /o! $&H and /HD ).I to .8O intra-cranial pathology=

D ;2-@2? anosmia (7allmann*ssyndrome#

D &ometimes constitutional delayD 0reat according to the cause (H.0#

potentially fertile=

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Constitutional pu+ertal #elay

Common cause B@0

/nder stature and delayed'one age

5-ray Hrist Goint0

2ositive family history

!iagnosis 'y e9clusion andfollow up

2rognosis is good

late developer0

o drug therapy isreJuired K Reassurance .RT0

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Sheehan?s syn#rome

2ituitary ina'ility to secrete gonadotropins

2ituitary necrosis following massive

o'stetric hemorrhage is most commoncause in women

!iagnosis ) istory and 1B$

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@ei)ht-relate# amenorrhoea

Anore"ia Nervosa 1o or :o Amenorrhea is often first sign

A body mass inde" (5)I# 91 7g8mEmenstrual irregularity and amenorrhea

Hypothalamic suppression Abnormal body image intense fear of !eight

gain often strenuous e"ercise

)ean age onset 1;-1@ yrs (range 12-:1 yrs#

/o! estradiol

ris7 of osteoporosis

5ulemics less commonly have amenorrheadue to fluctuations in body !t but any

disordered eating pattern (crash diets# can

cause menstrual irregularity=

0reatment F body !t= ('sychiatrist referral#

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E/ercise-associate#

amenorrhoea Common in women who participate

in sports e%g% competitiveathletes$ 'allet dancers0

1ating disorders have a higherprevalence in female athletes thannon-athletes

ypothalamic disorder caused 'ya'normal gonadotrophin-releasinghormone pulsatility$ resulting inimpaired gonadotrophin levels$particularly =$ and su'seJuentlylow oestrogen levels

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Contraception relate#

amenorrhea 2ost-pill amenorrhea is not an entity

!epot medro9yprogesterone acetate

/p to ?@ of women will have amenorrhea after

year of use% It is reversi'le oestrogen deLciency0 A minority of women ta;ing the progestogen-only

pill may have reversi'le long term amenorrhoeadue to complete suppression of ovulation

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Cushin)?s syn#rome

Clinical suspicion : Hirsutism,truncal obesity, purple striae,BP

If Suspicion is high :

dexamethasone suppression test! mg P" !! pm # and obtaineserum cortisol le$el at % am :

& ' (g) dl excludes cushing*s

+ hours total urine free cortisolle$el to confirm diagnosis

+ forms adrenal tumour or .C/Hhypersecretion pituitary or ectopicsite#

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(tero-va)inal A)enisisMayer-Roitansy-8uster-Hauser

syn#rome 13? of 1ry amenorrhea

Normal breasts and &e"ual Hair

development + Normal loo7ing e"ternal

female genitalia Normal female range testosterone level

Absent uterus and upper vagina + Normal

ovaries

Garyotype @>-

13-;2? renal s7eletal and middle earanomalies

0reatment F &0.I/ Jaginal creation

( Bilatation ! Jaginoplasty#

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An#ro)en insensitivity

0esticular *emini;ation syn#rome

5-lin;ed trait

A'sent cytosol receptors

ormal 'reasts 'ut no se9ual

hair ormal loo;ing female e9ternal

genitalia

A'sent uterus and upper vagina

>aryotype 38$ 5:

Male range testosterone level

Treatment ) gonadectomy afterpu'erty , RT

. Vaginal creation dilatation

V#Vaginoplasty 0

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6eneral Principles o*mana)ement o* Amenorrhea

%"ttempts to restore ovulatory unction

%% t#is is not possible H'T (oestrogen and

progesterone) is given to #ypo-estrogenicamenorr#eic /omen (toprevent osteoporosis; atherogenesis)

%erio!ic progestogen s#oul! be ta0en by euestrogenic

amenorr#eic /omen (to avoid endometrial cancer)%% Y c#romosome is present gona!ectomy is in!icate!

%+any cases re1uire re1uent re-evaluation

l

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Hormonal treatmentPrimary Amenorrhea itha+sent secon#ary se/ual

characteristics

0o achieve pubertal development

remarin 2mg 3-42 5 provera 36mg 32-42

X 7 mont#s8 4.2mg premarin X 7 mont#s an!

3.42mg premarin X 7 mont#s

)aintenance therapy 6.942mg premarin 5 provera &' rea!y H'T

preparation &' 76:g oral contraceptive pill

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Summary

"lt#oug# t#e /or0-up o amenorr#ea may seem to be

comple; a careully con!ucte! p#ysical eamination /it# t#e

#istory; an! *oo0ing to t#e patient as a bioassay or en!ocrine

abnormalities; s#oul! permit t#e clinician to narro/ t#e!iagnostic possibilities an! an accurate !iagnosis can be

obtaine! 1uic0ly.

+anagement aims at restoring ovulatory cycles i possible;

replacing estrogen /#en !eicient an! rogestogegen toprotect en!ometrium rom unoppose! estrogen.

Fre1uent re-evaluation an! reassurance o t#e patient.

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0HAN8