A. Treatment of primary amenorrhea is directed at correcting the underlying pathology; helping the woman to achieve fertility, if desired; and prevention of complications of the disease.

B. Congenital anatomic lesions or Y chromosome material usually requires surgery. Surgical correction of a vaginal outlet obstruction is necessary before menarche, or as soon as the diagnosis is made after menarche. Creation of a neovagina for patients with mullerian failure is usually delayed until the women is emotionally mature. If Y chromosome material is found, gonadectomy should be performed to prevent gonadal neoplasia. However, gonadectomy should be delayed until after puberty in patients with androgen insensitivity syndrome. These patients have a normal pubertal growth spurt and feminize at the time of expected puberty.

C. Ovarian failure requires counseling about the benefits and risks of hormone replacement therapy.

D. Polycystic ovary syndrome is managed with measures to reduce hirsutism, resume menses, and fertility and prevent of endometrial hyperplasia, obesity, and metabolic defects.

E. Functional hypothalamic amenorrhea can usually be reversed by weight gain, reduction in the intensity of exercise, or resolution of illness or emotional stress. For women who want to continue to exercise, estrogen-progestin replacement therapy should be given to those not seeking fertility to prevent osteoporosis. Women who want to become pregnant can be treated with gonado-tropins or pulsatile GnRH.

F. Hypothalamic or pituitary dysfunction that is not reversible (eg, congenital GnRH deficiency) is treated with either exogenous gonadotropins or pulsatile GnRH if the woman wants to become pregnant.

References: See page 184.

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