Emad R. Sagr, MBBS, FRCSC, FACOG Consultant Obstetrics & Gynecology and Gynecology Oncology Security Forces Hospital Definitions 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. CNS-Hypothalamus-Pituitary Ovary-uterus Interaction Neural controlChemical control Dopamine (-) Norepiniphrine (+) Endorphines (-) Hypothalamus Gn-RH Ant. pituitary FSH, LH Ovaries Uterus ProgesteroneEstrogen Menses ? Incidence of Primary Amenorrhea Less than.1% Puberty Breast: / yrs. Pubic Hair:11.0 +/ yrs. Menarche12.9 +/- 1.2 yrs. Onset of Puberty and Menstruation Ratio of fat to both total body weight and lean body weight Moderate obesity (20 30 % above ideal body weight) = earlier menarch Malnutrition (anorexia nervosa, starvation) = delay Prepubertal strenuous exercise (less total body fat) = delay e.g. ballet dancers, swimmers, runners Diagnostic Evaluation by Compartments IOutflow Tract (uterus vagina) IIOvary IIIAnterior Pituitary IVCNS Hypothalamus (environment and psyche) Evaluation History/Physical Psychiatric, family history-genetic abnormalities, nutritional status, growth/development Secondary sexual characteristics Presence of breasts normal reproductive tract (uterus, vagina) Evaluation Categories Breast Absent Uterus Present Breast Present Uterus Present Breast Present Uterus Absent Breast Absent Uterus Absent Initial Tests for Amenorrhea Progesterone challenge TSH Prolactin TSH elevated hypothyroid Prolactin elevated (MRI 100 ng/ml) Progesterone Challenge Positive withdrawal bleed Normal prolactin Normal TSH Diagnosis = annovulation Treatment: monthly progesterone/O.C. Progesterone Negative Withdrawal FSH/LH FSH/LH normal estrogen/progesterone cycle If negative = end organ defect If FSH/LH high = ovarian failure Estrogen positive withdrawal, FSH normal or low, MRI sella = no path Diagnosis: hypothalamic amenorrhea Chromosome Evaluation for Ovarian Failure If the patient is under age 30 karyotype Y chromosome/excision of gonadal area Problem gonadal tumor malignant 30% do not develop virilization, therefore even normal appearing female needs karyotype to exclude Y After age 30 = premature menopause Specific Disorders IOutflow- imperforate hymen, ashermans mullerian agenesis, androgen insensitivity syndrome IIOvary - can be primary or secondary amenorrhea 40% of primary amenorrhea have gonadal streaks Of the 40%, 50% = 45,X 25% = mosaics 25% = 46 XX Secondary amenorrhea patients have many karyotypes Specific Disorders (continued) Turner syndrome Gonadal dysgenesis Gonadal agenesis Savage syndrome Premature ovarian failure Radiation therapy Alkylating agents Compartment III Anterior pituitary disorders Tumors large bitemperal hemianopsia Small tumors visual defects- rare Craniopharyngioma calcification x-ray may produce blurring of vision Acromegaly Cushings Pituitary prolactin adenomas (micro/macro) Sheehans syndrome Compartment IV CNS disorders Hypothalamic amenorrhea problem is a GNRH pulsatile secretion Anorexia/Bulemia/weight loss 25% (onset 10 30 years) Exercise Etiology of Amenorrhea Breast Absent Breast Present Uterus Absent Uterus Present 17, 20 desmolase deficiency 1. Gonadal failure turner 45X 17 a hydroxylase deficiency 46xy Gonadal dysgenisis Agonadism 17 a hydroxylase deficiency with 46XX 2. Hypothalamic failure 3. Pituitary failure AIS (T.F.) Hypothalamic, pituitary, ovarian pt uterine etiology Mullerianagenesis