Amenorrhea Dr Ferdous Mehrabian Professor of Isfahan university
Amenorrhea Dr Ferdous Mehrabian Professor of Isfahan university
Amenorrhea ® Primary ® Absence of menses by age 16 with normal secondary sexual characteristics ® Absence of menses by age 14 without secondary sexual development ® Secondary ® Absence of menses for 6 months in a previously menstruating female
Events of Puberty ® Thelarche ® (breast development) Requires estrogen ® Pubarche/adrenarche (pubic hair development) ® Requires androgens ® Menarche ® Requires: ® Gn. RH from the hypothalamus ® FSH and LH from the pituitary
Are there secondary sexual characteristics?
Primary Amenorrhea ® Is there normal development of secondary sexual characteristcs? NO Think hypogonadism or hypogonadotropism
Amenorrhea with Immature Secondary Characteristics FSH Serum level Low / normal Hypogonadotropic hypogonadism High Gonadal dysgenesis
Primary Amenorrhea ® Hypogonadism ® 30% have genetic abnormality ® Gonadal dysgenesis, Turner’s syndrome, mosaicism ® Enzyme deficiencies ® Kallmann’s syndrome, CNS tumors ® Irradiation ® Chemotherapy ® Galactosemia
Primary Amenorrhea with Immature Sexual Characteristics ® Hypogonadism ® Gonadal (gonadal failure) dysgenesis ® Irradiation ® Chemotherapy ® Galactosemia ® Note: gonadotropins (FSH/LH) will be high, similar to menopause
Gonadal Dysgenesis ® Chromosomally abnormal - Classic turner’s syndrome (45 XO) - Turner variants (45 XO/46 XX), (46 X-abnormal X) - Mixed gonadal dygenesis (45 XO/46 XY) ® Chromosomally normal - 46 XX (Pure gonadal dysgeneis) - 46 XY (Swyer’s syndrome)
Primary Amenorrhea with Immature Sexual Characteristics ® Hypogonadotropism ® Hypothalamic dysfunction ® Kallmann syndrome ® Anorexia nervosa ® Space-occupying lesion of CNS ® Marijuana use ® Pituitary damage (surgery/radiation) ® Constitutional delay
Are there secondary sexual characteristics?
Primary Amenorrhea ® Is there normal development of secondary sexual characteristics? YES ® Think ® Pregnancy ® Mullerian anomaly ® Androgen insensitivity
Primary Amenorrhea with Normal Secondary Characteristics ® Mullerian Anomalies ® Mullerian agenesis (Mayer-Rokitansky- Kuster-Hauser syndrome) ® Imperforate hymen ® Transverse vaginal septum
Mayer-Rokitansky-Kuster-Hauser Syndrome (utero-vaginal agenesis) ® 15% of primary amenorrhea ® Normal secondary development & external female genitalia ® Normal female range testosterone level ® Absent uterus and upper vagina & normal ovaries
Imperforate Hymen
Androgen Insensitivity ® 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
Primary Amenorrhea ® Evaluation ® Pregnancy ® Physical test exam to determine presence of uterus ® FSH ® Karyotype
Primary Amenorrhea ® Treatment ® Cyclic estrogen/progestin ® Remove ® gonadal streaks if XY or mosaic Increased (52%) risk of gonadoblastomas, dysgerminomas, and yolk sac tumors ® Pulsatile Gn. RH for ovulation induction in select patients ® Surgical resection of intrauterine, cervical, and vaginal adhesions/septa
Secondary Amenorrhea ® Pregnancy! ® CNS disorders ® Pituitary gland ® Thyroid ® Ovary ® Uterus ® Systemic ® disorders Renal failure, liver disorders, DM ® Medications: anti-psychotics, reserpine
Secondary Amenorrhea ® CNS disorders ® Chronic hypothalamic anovulation ® Stress ® Increased exercise levels ® Anorexia nervosa ® Head trauma ® Space-occupying lesions
Secondary Amenorrhea ® Pituitary ® ® Hyperprolactinemia ® Prolactinoma ® Medications ® PCOS ® Renal failure Hypoprolactinemia ® Pituitary resection ® Sheehan’s syndrome ® Thyroid ® disorders Hyper- or hypothyroidism
Secondary Amenorrhea ® Ovulation disorders ® Polycystic ovarian syndrome ® Premature ovarian failure ® Uterine abnormalities ® Asherman’s syndrome ® Cervical stenosis ® Drug-induced amenorrhea ® Hormonal contraceptives ® Gn. RH analogues
Asherman’s Syndrome
Secondary Amenorrhea History Nutrition/exercise habits, weight change ® Sexual/contraceptive practice ® History of uterine/cervical surgery ® ® Physical exam ® Height/weight ® Hirsutism ® Galactorrhea ® Estrogen status of tissues ® Laboratory ® Bh. CG PRL & TSH progesterone challenge FSH if high karyotype
Negative Pregnancy. test TSH , PROLACTIN, Progesterone challenge test without withdrawal bleeding hypoestrogenic anovulation +ve. est/progest challenge test FSH norm. Repeat+serum estrogen level hypothalamicpituitary failure FSH>30 -40 repeat Pre. Ov Failure compromised outflow tract -ve. est/progest challenge test Normal FSH HSG OR hysteroscopy Asherman’s
Secondary Amenorrhea ® Treatment ® Discovery goals and treatment of underlying disorder ® Hormone ® Menses replacement every 1 -3 months ® Pregnancy ® Ovulation induction ® Gn. RH pump ® FSH/LH
Amenorrhea ® 26 yo Gravida 0 with menarche at age 14 presents with one-year history of amenorrhea.
Amenorrhea ® Sexually ® No active, using condoms recent change in weight, skin, hair ® Occasional heat intolerance ® No cyclic pain ® No gynecologic surgery ® Regular menses (every 28 -30 days) prior to past year
Amenorrhea ® Exam ® Overweight ® No galactorrhea ® Normal hair distribution ® Normal pelvic exam ® Pregnancy ® Progestin test challenge, TSH, serum prolactin ® Estrogen/progestin cycle, FSH
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