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AMENORRHEA The absence of menstrual flow, amenorrhea can be classified as primary or secondary.

With primary amenorrhea, menstruation fails to begin before age 16. With secondary amenorrhea, it begins at an appropriate age, but later ceases for 3 or more months in the absence of normal physiologic causes, such as pregnancy, lactation, or menopause. Pathologic amenorrhea results from anovulation or physical obstruction to menstrual outflow, such as from an imperforate hymen, cervical stenosis, or intrauterine adhesions. Anovulation itself may result from hormonal imbalance, debilitating disease, stress or emotional disturbances, strenuous exercise, malnutrition, obesity, or anatomic abnormalities, such as a congenital absence of the ovaries or uterus. Amenorrhea may also result from drug or hormonal treatments. History and physical examination Begin by determining whether the amenorrhea is primary or secondary. If it's primary, ask the patient at what age her mother first menstruated because age of menarche is fairly consistent in families. Form an overall impression of the patient's physical, mental, and emotional development because these factors as well as heredity and climate may delay menarche until after age 16. If menstruation began at an appropriate age but has since ceased, determine the frequency and duration of the patient's previous menses. Ask her about the onset and nature of any changes in her normal menstrual pattern, and determine the date of her last period. Find out if she has noticed any related signs, such as breast swelling or weight changes. Determine when the patient last had a physical examination. Review her health history, noting especially any long-term illnesses, such as anemia, or use of hormonal contraceptives. Ask about exercise habits, especially running, and whether she experiences stress on the job or at home. Probe the patient's eating habits, including the number and size of daily meals and snacks, and ask if she has gained weight recently. Observe her appearance for secondary sex characteristics or signs of virilization. If you're responsible for performing a pelvic examination, check for anatomic aberrations of the outflow tract, such as cervical adhesions, fibroids, or an imperforate hymen. Medical causes Adrenal tumor. Amenorrhea may be accompanied by acne, thinning scalp hair, hirsutism, increased blood pressure, truncal obesity, and psychotic changes. Asymmetrical ovarian enlargement in conjunction with the rapid onset of virilizing signs is usually indicative of an adrenal tumor. Adrenocortical hyperplasia. Amenorrhea precedes characteristic cushingoid signs, such as truncal obesity, moon face, buffalo hump, bruises, purple striae, hypertension, renal calculi, psychiatric disturbances, and widened pulse pressure. Acne, thinning scalp hair, and hirsutism typically appear. Adrenocortical hypofunction. In addition to amenorrhea, adrenocortical hypofunction may cause fatigue, irritability, weight loss, increased pigmentation (including bluish black discoloration of the areolas and mucous membranes of the lips, mouth, rectum, and vagina), vitiligo, nausea, vomiting, and orthostatic hypotension.

Amenorrhea-lactation disorders. Amenorrhea-lactation disorders, such as ForbesAlbright and Chiari-Frommel syndromes, produce secondary amenorrhea accompanied by lactation in the absence of breast-feeding. Associated features include hot flashes, dyspareunia, vaginal atrophy, and large, engorged breasts. Anorexia nervosa. Anorexia nervosa is a psychological disorder that can cause either primary or secondary amenorrhea. Related findings include significant weight loss, a thin or emaciated appearance, compulsive behavior patterns, a blotchy or sallow complexion, constipation, reduced libido, decreased pleasure in once-enjoyable activities, dry skin, loss of scalp hair, lanugo on the face and arms, skeletal muscle atrophy, and sleep disturbances. Understanding disruptions in menstruation A disruption at any point in the menstrual cycle can produce amenorrhea, as illustrated in the flowchart below.

Congenital absence of the ovaries. Congenital absence of the ovaries results in primary amenorrhea and the absence of secondary sex characteristics. Congenital absence of the uterus. Primary amenorrhea occurs with congenital absence of the uterus. The patient may develop breasts. Corpus luteum cysts. Corpus luteum cysts may cause sudden amenorrhea as well as acute abdominal pain and breast swelling. Examination may reveal a tender adnexal mass and vaginal and cervical hyperemia.

Hypothalamic tumor. In addition to amenorrhea, a hypothalamic tumor can cause endocrine and visual field defects, gonadal underdevelopment or dysfunction, and short stature. Hypothyroidism. Deficient thyroid hormone levels can cause primary or secondary amenorrhea. Typically vague, early findings include fatigue, forgetfulness, cold intolerance, unexplained weight gain, and constipation. Subsequent signs include bradycardia; decreased mental acuity; dry, flaky, inelastic skin; puffy face, hands, and feet; hoarseness; periorbital edema; ptosis; dry, sparse hair; and thick, brittle nails. Other common findings include anorexia, abdominal distention, decreased libido, ataxia, intention tremor, nystagmus, and delayed reflex relaxation time, especially in the Achilles tendon. Mosaicism. Mosaicism results in primary amenorrhea and the absence of secondary sex characteristics. Ovarian insensitivity to gonadotropins. A hormonal disturbance, ovarian insensitivity to gonadotropins leads to amenorrhea and an absence of secondary sex characteristics. Pituitary tumor. Amenorrhea may be the first sign of a pituitary tumor. Associated findings include headache; visual disturbances, such as bitemporal hemianopsia; and acromegaly. Cushingoid signs include moon face, buffalo hump, hirsutism, hypertension, truncal obesity, bruises, purple striae, widened pulse pressure, and psychiatric disturbances. Polycystic ovary syndrome. Typically, menarche occurs at a normal age, followed by irregular menstrual cycles, oligomenorrhea, and secondary amenorrhea. Or, periods of profuse bleeding may alternate with periods of amenorrhea. Obesity, hirsutism, slight deepening of the voice, and enlarged, oysterlike ovaries may also accompany this disorder. Pseudoamenorrhea. An anatomic anomaly, such as imperforate hymen, obstructs menstrual flow, causing primary amenorrhea and, possibly, cyclic episodes of abdominal pain. Examination may reveal a pink or blue bulging hymen. Pseudocyesis. With pseudocyesis, amenorrhea may be accompanied by lordosis, abdominal distention, nausea, and breast enlargement. Testicular feminization. Primary amenorrhea may signal this form of male pseudohermaphroditism. The patient, outwardly female but genetically male, shows breast and external genital development but scant or absent pubic hair. Thyrotoxicosis. Thyroid hormone overproduction may result in amenorrhea. Classic signs and symptoms include an enlarged thyroid (goiter), nervousness, heat intolerance, diaphoresis, tremors, palpitations, tachycardia, dyspnea, weakness, and weight loss despite increased appetite.

Turner's syndrome. Primary amenorrhea and failure to develop secondary sex characteristics may signal this syndrome of genetic ovarian dysgenesis. Typical features include short stature, webbing of the neck, low nuchal hairline, a broad chest with widely spaced nipples and poor breast development, underdeveloped genitalia, and edema of the legs and feet. Uterine hypoplasia. Primary amenorrhea results from underdevelopment of the uterus, which is detectable on physical examination. Other causes Drugs. Busulfan, chlorambucil, injectable or implanted contraceptives, cyclophosphamide, and phenothiazines may cause amenorrhea. Hormonal contraceptives may cause anovulation and amenorrhea after they're discontinued. Radiation therapy. Irradiation of the abdomen may destroy the endometrium or ovaries, causing amenorrhea. Surgery. Surgical removal of both ovaries or the uterus produces amenorrhea. Special considerations In patients with secondary amenorrhea, physical and pelvic examinations must rule out pregnancy before diagnostic testing begins. Typical tests include progestin withdrawal, serum hormone and thyroid function studies, and endometrial biopsy. Patient counseling Explain the treatment and expected outcomes, encourage the patient to discuss her fears, and refer her for psychological counseling if needed. PEDIATRIC POINTERS Adolescent girls are especially prone to amenorrhea caused by emotional upsets, typically stemming from school, social, or family problems. GERIATRIC POINTERS In women older than age 50, amenorrhea usually represents the onset of menopause.

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