Stein-Leventhal syndrome is an accumulation of many incompletely developed follicles in the ovaries. This condition is characterized by irregular menstrual cycles, scanty or absent menses , multiple small cysts on the ovaries (polycystic ovaries), mild hirsutism, and infertility . Many women who have this condition also have diabetes with insulin resistance.
Alternative Names
Polycystic ovaries; Sclerocystic ovarian disease; Polycystic ovarian syndrome (PCOS)
Causes, incidence, and risk factors
Abnormal ovarian function sometimes causes incompletely developed follicles (ova) to accumulate in the ovaries. These ova fail to mature and ovulate, therefore causing them to accumulate as cysts in the ovary and contributing to infertility in the woman. Polycystic ovaries are two to five times as large as normal ovaries, and they have a white, thick, very tough outer covering. This condition is commonly called Stein-Leventhal syndrome, and usually develops shortly after puberty. A woman with polycystic ovaries will stop menstruating (she may not have ever started) or will menstruate erratically. She will gain weight, eventually becoming obese , and may develop excessive amounts of facial or body hair ( hirsutism ). Some women exhibit virilization . Although the cause of Stein-Leventhal is not fully understood, there are several theories suggesting that problems with estrogen production and hypothalamic-ovarian feedback may be responsible. Normal ovarian function is dependent on a number of hormones and failure of one or more of these hormones to be produced at the right time, in the proper concentration, can interfere with normal development. Ovarian function will not proceed normally if a woman's body does not produce sufficient amounts of pituitary hormones. However, an increase in the amount of follicle-stimulating hormone ( FSH ), which is one of the hormones normally produced by the pituitary gland, is frequently successful in stimulating the underdeveloped ova to mature and be released from the ovary. Women diagnosed with this disorder frequently have a mother or sister(s) with similar symptoms commonly associated with PCOS (polycystic ovarian syndrome). However, there is not currently enough evidence to prove a genetic link to the disease. Conception is frequently possible with proper surgical or medical treatments. Following conception, pregnancy is normally uneventful.
Signs and tests
In a pelvic examination, the health care provider may note an enlarged clitoris (very rare finding) and enlarged ovaries. Tests:
LH (luteinizing hormone) to FSH (follicle stimulating hormone) ratio increased
Vaginal ultrasound Laparoscopy Ovarian biopsy
Androgen ( testosterone ) levels elevated
Urine 17-ketosteroids (may be elevated) Elevated LH Estrogen level relatively high FSH decreased Serum HCG ( pregnancy test ) negative This disease may also alter the results of the following tests:
Estriol - urine Estriol - serum
Treatment
Medications used to treat the symptoms of Stein-Leventhal syndrome include oral contraceptives, spironolactone, flutamide, and clomiphene citrate. Treatment with clomiphene induces the pituitary gland to produce more FSH , which in turn stimulates maturity and release of the ova. Occasionally, more potent ovulation induction agents (fertility drugs, human menopausal gonadotropins) are needed for pregnancy. A "wedge resection" of the ovaries has been used in the past to remove cysts . Finally, weight reduction , which may be very difficult, is also very important. Maintaining general good health and eliminating the complications of obesity are helpful.
Expectations (prognosis)
Pregnancy may be achieved with appropriate medical intervention.
Calling your health care provider
Call for an appointment with your health care provider if you are experiencing the symptoms of this disorder.