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Stein-Leventhal syndrome


Alternative names

Polycystic ovaries; Sclerocystic ovarian disease; Polycystic ovarian syndrome (PCOS)

Definition

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 (excessive hair), and infertility . Many women who have this condition also have diabetes with insulin resistance.

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 be released from the ovaries. The ova accumulate as cysts in the ovary, contributing to infertility.

Polycystic ovaries are two to five times larger than normal ovaries, and they have a white, thick, tough outer covering. This condition is commonly called Stein-Leventhal syndrome, and usually develops shortly after puberty.

A woman with polycystic ovaries stops menstruating, menstruates erratically, or may not have ever menstruated. She will gain weight, eventually becoming obese . She may develop excessive amounts of facial or body hair (hirsutism). Some women exhibit virilization , or development of male characteristics.

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. 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 in the brain by the pituitary gland, may stimulate the underdeveloped ova to mature and be released from the ovary.

Women diagnosed with this disorder frequently have a mother or sister 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 usually uneventful.

Symptoms

  • Abnormal, irregular, or scanty menstrual periods (oligomenorrhea)
  • Absent menses ( amenorrhea ), usually (but not always) after having one or more normal menstrual periods during puberty ( secondary amenorrhea )
  • Obesity
  • Infertility
  • Increased hair growth ( hirsutism )
  • Decreased breast size
  • Aggravation of acne
  • Unusual growth and distribution of body hair in a male pattern (virilization)

Signs and tests

In a pelvic examination, the health care provider may note an enlarged clitoris (very rare finding) and enlarged ovaries.

Tests include:

  • LH (luteinizing hormone)-to-FSH ratio increased
  • Vaginal ultrasound
  • Laparoscopy
  • Ovarian biopsy
  • Androgen ( testosterone ) levels elevated
  • Urine 17-ketosteroids (possibly 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.

Complications

  • Sterility
  • Complications secondary to obesity
  • Poor self-image and social life due to androgenic features (excessive facial hair, small breast size)
  • Diabetes
  • Complications secondary to oligo-ovulation, such as endometrial hyperplasia and endometrial cancer

Calling your health care provider

Call for an appointment with your health care provider if you are experiencing the symptoms of this disorder.

Update Date: 10/18/2003

Francisco L. Gaudier, M. D., Maternal Fetal Medicine, Jacksonville, FL. Review provided by VeriMed Healthcare Network.

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Last updated: Tue, 06 Jan 2009 00:20:03 GMT
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