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at menarche,
Menstrual
gestational
MEDICHIE
Disorders
complica- ify the degree of estrogen stim- mal steroid production from those
J
tions and medications, childhood de- ulation. Bimanual examination, who have hypothalamic or pituitary
velopment, chronic systemic illness, followed by ultrasonographic exami- disorders. Chromosomal analysis is
nutrition, and family history of ge- nation of the pelvis, if necessary, indicated when FSH and LH levels
netic anomalies. Physical examina- will establish the presence or absence are high.
tion should be meticulous and of a uterus and ovaries. Gonadal dysgenesis is the most
include charting of height, weight, common cause of primary amenor-
and sexual maturation rating; com- DIFFERENTIAL DIAGNOSIS AND rhea. It usually is due to Turner syn-
MANAGEMENT
plete neurologic examination, includ- drome (45,XO kaiyotype) or some
ing the cranial nerves; examination Patients who have primary amenor- other abnormality in X chromosome
of the skin, hair, and genitalia for rhea can be divided into four groups, structure or number. The ovaries fail
signs of hirsutism or virilization; pal- depending on pubertal maturation and to develop, leaving remnant fibrous
pation of the thyroid; and palpation internal genitalia (Figure 1). bands called gonadal streaks. Patients
of the abdomen and groin for who have gonadal dysgenesis are
No breast development-Intact uterus
masses. sexually immature, amenorrheic, and
Pelvic examination, if done in a These individuals lack ovarian estro- have high FSH and LH levels (hyper-
sensitive manner, need not be a trau- gen but have normal development of gonadotropic hypogonadism). Indi-
matic experience. At absolute mini- the Mullerian system during fetal viduals who have Turner syndrome
mum, a careful examination of the life. The differential diagnosis in- often have other stigmata, such as
external genitalia must be performed. cludes gonadal dysgenesis, hypothal- short stature, webbed neck, or chest
Use of the speculum should be pre- amic-pituitary disorder, and genetic and limb abnormalities. Patients who
ceded by digital vaginal examination defects in ovarian steroid production. have mosaic Turner syndrome or
to avoid injuring the patient with an Measurement of the serum levels of other X chromosome abnormalities
absent vagina or an outflow obstruc- follicle stimulating hormone (FSH) may be phenotypically normal except
tion. If a normal vagina and cervix and luteinizing hormone (LH) will for sexual immaturity.
are present, microscopic examination differentiate patients who have gona- Gonadal dysgenesis also may oc-
of the cervical mucus may help clar- dal (ovarian) dysgenesis and abnor- cur in individuals who have normal
Breasts (-)
Breasts (-)
Uterus (+)
Uterus (-)
High
Hypogonadotropic
Lesions
T hypogonadism
of the central nervous system
Gonadal
45,XO
I
dysgenesis
(Turner syndrome)
Gonadal enzyme deficiency Agonadism
(testicular regression)
Breasts (+)
Uterus (-) Uterus (+)
Mullerian
ti agenesis Androgen
insensitivity (testicular obstruction
Rule
ft
out vaginal
(eg, imperforate
outlet
hymen,
Hypothalmic-pituitary-ovarian
disturbance
1
(evaluate as
axis
FIGURE 1. Diagnostic approach to patients who have primaty amenorrhea. Adapted from Maschak CA, Kletzky OA, Davajan V. Mishell DR
Jr. Clinical and laboratoty evaluation of patients with primaty amenorrhea. Obstet Gynecol. 1981;5 7: 71 9. Reprinted with pennission from the
American College of Obstetrics and Gynecology.
ADOLESCENT MEDICINE
Menstrual Disorders
in females who have already estab- amenorrhea focuses on the hypothala- ing, weight fluctuation, bowel habits,
lished menstruation. Although men- mic-pituitary-ovarian axis. This be- exercise, medication and drug use,
ses often are irregular in young gins with a complete history and headache, visual change, and galac-
adolescents, they should stabilize physical examination (Figure 2). torrhea. Family history of menstrual
within 1 to 2 years of menarche. irregularities, eating disorders, diabe-
Amenorrhea occurring more than 18 INITIAL EVALUATION tes, and thyroid disease also may be
months after menarche should be Maintenance of normal menses re- helpful.
considered abnormal and warrants quires adequate body fat composi- The physical examination should
investigation. tion. Because weight loss or failure begin with measurement of height
Pregnancy always must be in- to gain weight is a common sign of and weight and an assessment of
cluded in the differential diagnosis of illness during adolescence, secondary body habitus. Common stigmata of
secondary amenorrhea. Sensitive amenorrhea may be an indication of anorexia nervosa include cachexia,
questioning need not imply inherent poor nutrition, stress, or systemic ill- lanugo, parotid enlargement, brady-
distrust or disbelief of the adolescent ness. Common diseases presenting cardia, hypotension, and hypother-
who denies sexual activity. Instead, during adolescence that may be asso- mia. Fundoscopic examination, gross
it acknowledges the strong sociocul- ciated with secondary amenorrhea in- visual fields, and examination of the
tural pressures that may lead an ado- clude anorexia nervosa, inflammatory cranial nerves should be done as part
lescent to give an inaccurate sexual bowel disease, diabetes mellitus, thy- of an initial screening for a pituitary
history. roid disease, and pituitary adenomas. lesion. Breast examination should in-
Once pregnancy has been ex- The history,therefore,should include clude an attempt to elicit galactor-
cluded, evaluation of secondary questioning about caloric intake, diet- rhea. If light microscopy of the
Rule out
Ashermann
syndrome
Abnormal
Lesion of1
I central nervous
system
FIGURE 2. Evaluation of secondary amenorrhea. CT, computed tomography; DHEA-S, dehydroepiandrosterone sulfate; LH, luteinizing
hormone; MRI, magnetic resonance imaging; TEST, testosterone; TSH, thyroid stimulating hormone. Modified from Speroff L, Glass RH,
Kase NG. Clinical Gynecologic Endocrinology and Infertility. 4th ed. Baltimore, MD: Williams and Wilkins; 1989:178
presumed ovarian failure to rule out replacement therapy should be University of Pennsylvania School of Medi-
cute.
Turner mosaicism or other chromo- instituted.
somal abnormalities. The presence of Anorexia nervosa is a special case tAssociate Professor of Medicine and Pediat-
rics, University of Pennsylvania School of
any portion of a Y chromosome man- of hypothalamic amenorrhea. Al- Medicine, and Director, Craig-Dulcimer Pro-
dates gonadectomy to eliminate the though the mechanisms of amenor- grunt in Adolescent Medicine, Hospital of the
risk of malignant transformation of rhea are similar to those of exercise- University of Pennsylvania, Childrens Hospi-
the gonad. Ovarian failure also may induced amenorrhea (ie, low body tal of Philadelphia.
result from autoimmune disease, fat, stress, beta-endorphin release), Correspondence and reprint requests should be
chemotherapy, or radiation. the complex psychological and mcdi- addressed to Dr Polaneczky, Department of
Obstetrics and Gynecology, University of
Low serum levels of gonadotropins cal problems associated with anorexia Pennsylvania, 5 Penn Tower, 34th Street and
point to a defect in the hypothalamus nervosa are difficult to manage. A Civic Center Boulevard Philadelpia, PA 19104-
or pituitary (hypogonadotropic hypo- team approach employing psychiatric 4283.
gonadism or hypothalamic amenor- counseling, close medical supervi-
rhea). Although the incidence of sion, nutritional consultation, and
pituitary tumors is low in the absence hormonal replacement provides the
of galactorrhea or an elevated serum best outcome.
prolactin, radiologic evaluation There is increasing evidence that
should be considered in cases where osteoporosis associated with chronic
no obvious cause for hypothalamic estrogen deficiency may begin during
dysfunction can be found. Hypothala- the adolescent years. For this reason,
mic amenorrhea comprises a hetero- estrogen replacement therapy should
geneous group of disorders as not be delayed until adulthood in the
discussed previously, including sys- patient who has well-established hy-
temic illness, anorexia nervosa, exer- pogonadism. Progesterone should be
cise-induced amenorrhea, and administered with the estrogen to
stressful situations (eg, going away minimize the risk of endometrial car-
to school). It is believed that stress cinoma. When such a combined regi-
leads to alterations in hypothalamic men is used, the benefits appear to
GnRh secretion, possibly due to ele- far outweigh the risks.
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