Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 26-33: Primary Amenorrhea + Key Features Download Section PDF Listen +++ ++ Menarche ordinarily occurs between ages 11 and 15 years (average in United States, 12.7 years) Primary amenorrhea is failure of any menses to appear Evaluate at age 14 if no menarche or breast development or if height in lowest 3%, or at age 16 if still no menarche Causes Hypothalamic-pituitary (low-normal FSH) Idiopathic delayed puberty Pituitary tumor Hypothalamic amenorrhea (eg, stress, weight change, exercise) Anorexia nervosa Hypothyroidism Cushing syndrome GnRH or gonadotropin deficiency Hyperandrogenism (low-normal FSH) Adrenal tumor or adrenal hyperplasia Polycystic ovary syndrome Ovarian tumor Exogenous androgenic steroids Uterine causes (normal FSH) Absence of uterus Imperforate hymen Ovarian causes (high FSH) Turner syndrome Autoimmune ovarian failure Pseudohermaphroditism Complete androgen insensitivity syndrome: individuals are born with normal external female genitalia, although some may have labial or inguinal swellings due to cryptorchid testes Partial androgen insensitivity syndrome: individuals have variable degrees of ambiguous genitalia Pregnancy (high hCG) + Clinical Findings Download Section PDF Listen +++ ++ Nausea, breast engorgement suggest pregnancy Headaches or visual field abnormalities suggest pituitary tumor Obesity and short stature suggests Cushing syndrome Hirsutism and virilization suggest excessive testosterone Short stature suggests growth hormone or thyroid hormone deficiency Short stature and gonadal dysgenesis indicate Turner syndrome Tall stature suggests eunuchoidism or acromegaly Anosmia suggests Kallmann syndrome Perform pelvic examination to assess for hymen patency and presence of a uterus + Diagnosis Download Section PDF Listen +++ ++ Serum FSH, LH, prolactin, total and free testosterone, TSH, T4, and pregnancy test Serum electrolytes Further hormone evaluation if patient is virilized or hypertensive MRI of hypothalamus and pituitary is used to evaluate teens with primary amenorrhea and low or normal FSH and LH—especially those with high PRL levels Pelvic duplex/color sonography is very useful Karyotyping + Treatment Download Section PDF Listen +++ ++ Treatment directed at underlying cause Hormone replacement therapy for females with permanent hypogonadism See Amenorrhea, Secondary & Menopause