Polycystic ovary disease

Alternative names
Polycystic ovaries; Polycystic ovarian syndrome (PCOS); Stein-Leventhal syndrome; Polyfollicular ovarian disease.

Definition

Polycystic ovary disease is characterized by enlarged ovaries with multiple small cysts, an abnormally high number of follicles at various states of maturation, and a thick, scarred capsule surrounding each ovary.

The syndrome was originally reported by Stein and Leventhal in 1935 when they described a group of women with amenorrhea (absence of menses), infertility, hirsutism (unwanted hair growth in women), and enlarged polycystic ovaries.

Today, it is known that those with polycystic ovaries may have some, but not necessarily all, of the “classic” symptoms included in Stein-Leventhal syndrome.

Causes, incidence, and risk factors

Polycystic ovary disease is an endocrine disorder, which means normal hormone cycles are disrupted. Hormones direct many functions throughout the body. For example, hormones regulate reproductive functions, including the normal development of ova (eggs) in the ovaries. It is not completely understood why or how hormone cycles are disrupted, although there are several working theories.

In polycystic ovary disease, under-developed follicles accumulate in the ovaries. Follicles are sacs within the ovaries that contain ova. The ova in these follicles fail to mature and, therefore, cannot be released from the ovaries. Instead, they accumulate as cysts in the ovary. This can contribute to infertility. The lack of follicular maturation and inability to ovulate are likely caused by low levels of follicle stimulating hormone (FSH) and higher-than-normal levels of androgens (male hormones) produced in the ovary.

Insulin resistance also seems to be a key feature in polycystic ovarian syndrome. In addition to other hormones, insulin helps regulate ovarian function. When someone is insulin resistant, this means that cells throughout the body do not readily respond to insulin circulating in the blood. For this reason, the amount of insulin remains high in the blood (called hyperinsulinemia). High levels of insulin can contribute to lack of ovulation, high androgen levels, infertility, and early pregnancy loss.

Polycystic ovaries are two to five times larger than normal ovaries, and they have a white, thick, tough outer covering. Women are usually diagnosed when in their 20s or 30s.

Many women with polycystic ovary disease have irregular menses and may have scanty menstruation (oligomenorrhea) or no menses at all (amenorrhea).

Women diagnosed with this disorder frequently have a mother or sister with similar symptoms commonly associated with PCOS (polycystic ovarian syndrome).

Conception is frequently possible with proper surgical or medical treatments. Following conception, pregnancy is usually uneventful.

Symptoms

If you have polycystic ovary disease, you are likely to experience some of the following 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)  
  • Weight gain, even Obesity  
  • Insulin resistance and Diabetes  
  • Infertility  
  • Increased hair growth (hirsutism); distribution of body hair may be in a male pattern  
  • Virilization - development of male sex characteristics in a female. This may include an increase in body hair, facial hair, a deepening of the voice, male-pattern baldness, and clitoral enlargement.  
  • Decreased breast size  
  • Aggravation of Acne

Signs and tests

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

Tests include:

     
  • FSH levels - low or normal  
  • LH levels - generally high  
  • Androgen (testosterone) levels - high  
  • Estrogen (primarily estrone and estradiol) levels - relatively high  
  • Urine 17-ketosteroids - possibly high  
  • Vaginal ultrasound and, possibly, Abdominal ultrasound  
  • MRI  
  • Laparoscopy  
  • Ovarian biopsy

Other blood tests that may be done as part of the initial evaluation to look for other potential causes of the symptoms include:

     
  • Serum HCG (pregnancy test) negative  
  • Thyroid function tests  
  • Prolactin levels

Treatment

Medications used to treat the symptoms of polycystic ovary disease include Birth control pills, 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.

Weight reduction, which may be very difficult, is also very important. For those with polycystic ovaries who are overweight, Weight loss can reduce insulin resistance, stimulate ovulation, and improve fertility rates. Sometimes, part of the treatment for polycystic ovaries is use of insulin sensitizing medication like metformin.

Expectations (prognosis)

Pregnancy may be achieved with appropriate treatment.

Complications

     
  • Sterility  
  • Obesity-related conditions, like High blood pressure and Diabetes  
  • Increased the risk of Endometrial cancer - this is because the endometrium (lining of the uterine wall that sheds when you menstruate) can get thicker and thicker (hyperplasia) due to the lack of ovulation  
  • Possible increased risk of Breast cancer

Calling your health care provider

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

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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