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Amenorrhea In the name of GOD

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Page 1: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

AmenorrheaIn the name of GOD

Page 2: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Deffination Amenorrhea is the absence or cessation of

menses and may result from a wide variety of pathological condition:

AnatomicalEndocrineOrganic

Primary Amenorrhea : secondary sexual development fails to begin by age 14,or begins but fails to progress.

Secondary Amenorrhea : once menstural cycles have been established ,amenorrhea for an interval equivalent to 3 previous cycles,or 6 months.

Page 3: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

The Normal Menstrual Cycle1.Functional Hypothalamus to synthesize and

release GnRHin a pulstile pattern.2.Pitutitary gonadotrophs must have the capacity

to synthesize and secret gonadotropinsin response to GnRH.

3.Ovaries must contain follicles responsive to pituitiary FSH & LH stimulation.

4.Normal genital outflow tract and FunctionalEndometrium capable of respance to estrogen & progestrone.

Page 4: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse
Page 5: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Hypothalamus

Pitutitary

Ovaries

Genital outflow tract

Functional Endometrium

Page 6: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Genital Tract AbnormalitiesCongenital:

vaginal/mullerian agenesisandrogen insensitivity syndrome(AIS)imperforate hymentransverse vaginal septumcervical atresia

Acquired:asherman syndromecervical stenosis/obstraction

Page 7: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Abnormal Genital Tract Anatomy

A history of primary amenorrhea and absent or blind vaginaimperforate hymen , transverse vaginal septum , cervical atresia

a the time of menarche with complaint of cyclic perineal,pelvic or abdominal pressure or pain.

Page 8: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Imperforate Hymen

Page 9: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Abnormal Genital Tract Anatomy

Vaginal/mullerian agenesis

Asymtomatic and Normal breast and pubic hair development but absent vagina.

androgen insensitivity syndrome(AIS)

Normal breast and pubic hair development,absent or sparse growth of pubic hair and shot blind vagina.

Page 10: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Abnormal Genital Tract Anatomy

asherman syndrome

Dysmenorrhea and spotting

cervical stenosis/obstraction

Dysmenorrhea and hypomenorrhea,subfertility or recurrentearly pregnancy loss

Page 11: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Hypothalamus

Pitutitary

Ovaries

Genital outflow tract

Functional Endometrium

Congenital

Acquiredcervical stenosis/obstraction

asherman syndrome

cervical atresia

vaginal/mullerian agenesis

androgen insensitivity syndrome

imperforate hymen

transverse vaginal septum

Page 12: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Ovarian Disorders

Gonadal dysgenesis(30-40 %)Turner syndrome (45 XO)Swyer syndrome (46 XY)

No significant secondary sexual development

Premature ovarian failure (POF)Have significant secondary sexual development

Other:17- α hydroxylase deficencyAromatase deficencyGonadotropin-resistant ovary syndrome

Primaryamenorrhea

Secodaryamenorrhea

Page 13: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Normal Genital Tract Anatomy

Increased FSH: ovarian failure(Gonadal dysgenesis,Premature ovarian failure)

Page 14: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Hypothalamus

Pitutitary

Ovaries

Genital outflow tract

Functional Endometrium

Congenital

Acquiredcervical stenosis/obstraction

asherman syndrome

cervical atresia

vaginal/mullerian agenesis

androgen insensitivity syndrome

imperforate hymen

transverse vaginal septum

Gonadal dysgenesis

Premature ovarian failure

Other

Page 15: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Pituitary Disorders

Pituitary tumorsFunctional tumors (Prolactin,GH,TSH,ACTH)

Empty Sella syndromeSheehan syndrome

Page 16: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Normal Genital Tract Anatomy

1- TSH

hypo and hyper thyroidism

2-Prolactinprolactin-secreting pituitary adenomaother pituitary and hypothalamic tumorsdrugsbreast or chest wall stimulationhypothyroidismOCPothers…

3-FSHdecreased or normal

Page 17: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Hypothalamus

Pitutitary

Ovaries

Genital outflow tract

Functional Endometrium

Congenital

Acquiredcervical stenosis/obstraction

asherman syndrome

cervical atresia

vaginal/mullerian agenesis

androgen insensitivity syndrome

imperforate hymen

transverse vaginal septum

Gonadal dysgenesis

Premature ovarian failure

Other

Pituitary tumorsFunctional tumors (Prolactin,GH,TSH,ACTH)

Empty Sella syndromeSheehan syndrome

Page 18: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

•Hypothalamic Disorders

The most common cause of amenorrhea

Congenital:Kallmann syndrome

Aquired:PCOSHypothalamic amenorrheaHypothalamic tumorsHyperprolactinemia

Page 19: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Normal Genital Tract Anatomydecreased or normal FSH:pituitary & hypothalamic disorders(PCOS,Hypothalamic Amenorrhea)

PCOS: 1, ovulatory dysfunction

2, clinical evidence of hyperandrogenism(hirsutism,acne,androgenic alopecia)3, exclusion of other disorders(hyperprolactinema,CAH,thyroid abnormalities)+ obesity& insulin resistant

Page 20: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Normal Genital Tract Anatomy

Hypothalamic Amenorrhea:absence of obesity or evidence of hyperandrogenism of PCOS.

Determination of Estrogen Status???????? serum estradiol cervical mucus progestin challenge test

Page 21: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Hypothalamus

Pitutitary

Genital outflow tract

Functional Endometrium

Congenital

Acquiredcervical stenosis/obstraction

asherman syndrome

cervical atresia

vaginal/mullerian agenesis

androgen insensitivity syndrome

imperforate hymen

transverse vaginal septum

Gonadal dysgenesis

Premature ovarian failure

Other

Pituitary tumorsFunctional tumors (Prolactin,GH,TSH,ACTH)

Empty Sella syndromeSheehan syndrome

PCOSHypothalamic amenorrhea

Hypothalamic tumorsHyperprolactinemia

Congenital disorders (Kallmann syndrome)

Ovaries

Page 22: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Evaluation of AmenorrheaMedical History

Physical examination

Diagnostic evaluation: TSHProlactinFSH

Page 23: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Medical History

Puberty : acceleration of growth

thelarcheadrenarchemenarrche

Page 24: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Medical History Vaginal dryness or hot flushes: estrogen deficency Progressive hirsutism or virilization: hyperandrogenism Bilateral galactorrhea: hyperprolactinemia Cyclic pelvic or lower abdominal pain or urinary

complaints: developmental anpmalies …

Page 25: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Physical Examination Short stature(less than 60 inches): gonadal dysgenesis Sexual infantilism/webbing of the neck,low set ear &

posterior hairline,widely spaced nipples,short fourth metacarpal,cubitus valgus : turner

Acne and hirsutism: hyperandrogenism Obesity or an increased waist to hipe ratio: insulin

resistant & chronic anovulation …

Page 26: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse
Page 27: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse
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Treatment (genital tract abnormalities)

Vaginal/mullerian agenesis: vaginal dilation & operative treatment.

AIS: operative treatment & removed the testes. Imperforate hymen: cruciate incision. Transverse vaginal septum and cervical atresia:

surgical management. cervical stenosis: uterine sounding & cervical

dilation Asherman syndrome: high-dose exogenous

estrogen treatment.

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Treatment(ovarian disorders)

Gonadal dysgenesis: 1-GH 2-Sex hormone replacement therapy

(low doses of estrogen & progestin)

POF: estrogen/progestin hormone replacement therapy

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Treatment(pituitary disorders)

Pituitary tumors:* Medical treatment(Bromocriptine & Cabergoline)* Sugery treatment.

Empty sella syndrome: like pituitary adenoma.

Sheehan syndrome: hormone replacement therapy.

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Treatment(hypothalamic disorders)

PCOSDesire pregnancy: 1-weight loss

2-Clomiphene citrate3-Metformin4-Gonadotropins

Not desire pregnancy : OCP Hypothalamic amenorrheaDesire pregnancy: 1-redused physical activity & increased weight

2-Clomiphene citrate3- Gonadotropins & HCG

Not desire pregnancy : OCP

Page 32: In the name of GOD Amenorrheacervical stenosis/obstraction asherman syndrome cervical atresia vaginal/mullerianagenesis androgen insensitivity syndrome imperforate hymen transverse

Negative Pregnancy.test

TSH ,PROLACTIN, Progesterone challenge test

withdrawal bleeding

without withdrawal bleeding

hypoestrogenic compromised outflow tract

+ve.est/progest challenge test

-ve.est/progest challenge test

FSH>30-40Normal FSH

HSG OR hysteroscopyAsherman’s

FSH norm.

repeatRepeat+serum estrogen level

PreOvFailure

hypothalamic-pituitary failure

anovulation

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tHaNkS…

References:Danforth’s OBSTETRICS & GYNECOLOGYBerek & Nonak’s GYNECOLOGY

By: Drtabatabai