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Amenorrhea Dr.F Mehrabian MD. Amenorrhea  Primary  Absence of menses by age 16 with normal secondary sexual characteristics  Absence of menses by age.

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Presentation on theme: "Amenorrhea Dr.F Mehrabian MD. Amenorrhea  Primary  Absence of menses by age 16 with normal secondary sexual characteristics  Absence of menses by age."— Presentation transcript:

1 Amenorrhea Dr.F Mehrabian MD

2 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

3 Events of Puberty  Thelarche (breast development)  Requires estrogen  Pubarche/adrenarche (pubic hair development)  Requires androgens  Menarche  Requires:  GnRH from the hypothalamus  FSH and LH from the pituitary  Estrogen and progesterone from the ovaries  Normal outflow tract

4 Are there secondary sexual characteristics?

5 Primary Amenorrhea  Is there normal development of secondary sexual characteristcs? NO Think hypogonadism or hypogonadotropism

6 Amenorrhea with Immature Secondary Characteristics FSH Serum level Low / normalHigh Hypogonadotropic hypogonadism Gonadal dysgenesis

7 Primary Amenorrhea  Hypogonadism  30% have genetic abnormality  Gonadal dysgenesis, Turner’s syndrome, mosaicism  Enzyme deficiencies  Kallmann’s syndrome, CNS tumors  Irradiation  Chemotherapy  Galactosemia

8 Primary Amenorrhea with Immature Sexual Characteristics  Hypogonadism (gonadal failure)  Gonadal dysgenesis  Irradiation  Chemotherapy  Galactosemia  Note: gonadotropins (FSH/LH) will be high, similar to menopause

9 Gonadal Dysgenesis  Chromosomally abnormal - Classic turner’s syndrome (45XO) - Turner variants (45XO/46XX),(46X- abnormal X) - Mixed gonadal dygenesis (45XO/46XY)  Chromosomally normal - 46XX (Pure gonadal dysgeneis) - 46XY (Swyer’s syndrome)

10 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

11 Are there secondary sexual characteristics?

12 Primary Amenorrhea  Is there normal development of secondary sexual characteristics? YES  Think  Pregnancy  Mullerian anomaly  Androgen insensitivity

13 Primary Amenorrhea with Normal Secondary Characteristics  Mullerian Anomalies  Mullerian agenesis (Mayer-Rokitansky- Kuster-Hauser syndrome)  Imperforate hymen  Transverse vaginal septum

14 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  Karyotype 46-XX  15-30% renal, skeletal and middle ear anomalies

15 Imperforate Hymen

16 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

17 Primary Amenorrhea  Evaluation  Pregnancy test  Physical exam to determine presence of uterus  FSH  Karyotype

18 Primary Amenorrhea  Treatment  Cyclic estrogen/progestin  Remove gonadal streaks if XY or mosaic  Increased (52%) risk of gonadoblastomas, dysgerminomas, and yolk sac tumors  Pulsatile GnRH for ovulation induction in select patients  Surgical resection of intrauterine, cervical, and vaginal adhesions/septa

19 Secondary Amenorrhea  Pregnancy!  CNS disorders  Pituitary gland  Thyroid  Ovary  Uterus  Systemic disorders  Renal failure, liver disorders, DM  Medications: anti-psychotics, reserpine

20 Secondary Amenorrhea  CNS disorders  Chronic hypothalamic anovulation  Stress  Increased exercise levels  Anorexia nervosa  Head trauma  Space-occupying lesions

21 Secondary Amenorrhea  Pituitary disorders  Hyperprolactinemia  Prolactinoma  Medications  PCOS  Renal failure  Hypoprolactinemia  Pituitary resection  Sheehan’s syndrome  Thyroid disorders  Hyper- or hypothyroidism

22 Secondary Amenorrhea  Ovulation disorders  Polycystic ovarian syndrome  Premature ovarian failure  Uterine abnormalities  Asherman’s syndrome  Cervical stenosis  Drug-induced amenorrhea  Hormonal contraceptives  GnRH analogues

23 Asherman’s Syndrome

24 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  BhCG  PRL & TSH  progesterone challenge  FSH  if high  karyotype

25 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>30-40 Normal FSH HSG OR hysteroscopy Asherman’s FSH norm. repeat Repeat+serum estrogen level PreOv Failure hypothalamic- pituitary failure anovulation

26 Secondary Amenorrhea  Treatment goals  Discovery and treatment of underlying disorder  Hormone replacement  Menses every 1-3 months  Pregnancy  Ovulation induction  GnRH pump  FSH/LH

27 Amenorrhea  26 yo Gravida 0 with menarche at age 14 presents with one-year history of amenorrhea.

28 Amenorrhea  Sexually active, using condoms  No recent change in weight, skin, hair  Occasional heat intolerance  No cyclic pain  No gynecologic surgery  Regular menses (every days) prior to past year

29 Amenorrhea  Exam  Overweight  No galactorrhea  Normal hair distribution  Normal pelvic exam  Pregnancy test  Progestin challenge, TSH, serum prolactin  Estrogen/progestin cycle, FSH

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