| Causes | Incidence | Clinical Findings | Treatment |
|---|---|---|---|
| Structural/anatomical abnormalities | |||
| Mullerian agenesis | 1/4,000 to 1/10,000 | Amenorrhea with normal external genitalia, but absent upper genital tract | Support, counseling |
| Rare; increasingly worsening cyclical pelvic pain (if rudimentary uterus present) | Surgical removal of remnant | ||
| Inability to have intercourse | Surgical creation of neovagina | ||
| Androgen insensitivity | 1/60,000 | Amenorrhea with normal external genitalia, vaginal “dimple,” absent uterus and cervix, testes in abdominal cavity or inguinal canal | Support, counseling Possible surgical creation of neovagina Removal of testes (age 20) |
| Imperforate hymen | 1/1000 | Amenorrhea with cyclic abdominal pain, hematocolpos, hematometra, hemoperitoneum | Surgical removal of the hymenal tissue |
| Bluish and bulging hymen | |||
| Primary hypogonadism | |||
| Gonadal dysgenesis | Varies depending on etiology | Varies depending on etiology | Varies depending on etiology |
| Premature ovarian failure | 10% to 28% of women with primary amenorrhea | History of ovarian injury (surgical, chemotherapy, radiation therapy), autoimmune disease, or chromosomal disorder | Support, counseling, risk reduction for osteoporosis and cardiovascular disease |
| Estrogen replacement | |||
| Fertility assistance | |||
| HPA axis dysfunction | |||
| Functional hypothalamic amenorrhea | Most common form of amenorrhea in adolescence | Low levels of estrogen, LH, and FSH | Correct underlying cause of hypothalamic dysfunction |
| Anorexia nervosa, excessive exercise, excessive stress | Promote bone mineral density | ||
| OCPs | |||
| Kallman syndrome | Rare genetic disorder | Amenorrhea, lack of pubertal development, anosmia | Hormone replacement therapy, BMD monitoring |
| Hyperprolactinemia | Cause of 1% of primary and 15% of secondary amenorrhea | Elevated prolactin level, hypothyroidism, antipsychotic medication use | MRI of pituitary fossa for refractory or excessive prolactin level (>100 ng/mL) |
| Galactorrhea is rare | Bromocriptine | ||
| Multifactorial | |||
| PCOS | Oligomenorrhea, hirsutism, acne, obesity, insulin resistance | Weight loss, exercise, OCPs for menstrual regulation, insulin sensitizing medication (eg, metformin) to treat hyperinsulinemia |
Data from Refs. 42⇓–44.
BMD, bone mineral density; FSH, follicle-stimulating hormone; HPA, hypothalamic-pituitary axis; LH, luteinizing hormone; MRI, magnetic resonance imaging; OCP, oral contraceptive pill; PCOS, polycystic ovarian syndrome.