Polycystic Ovary (Stein-Leventhal) Syndrome: Etiology, Complications, and Treatment

CLINICAL PRESENTATION

PCOS may present with a wide variety of signs and symptoms (Table 1). The most common clinical presentations include menstrual irregularities, symptoms of hyperandrogenism, infertility, and obesity. Menstrual irregularities range from oligomenorrhea to amenorrhea.5 Women affected with PCOS usually begin menarche at a normal age (10 to 16 years of age), but their cycles remain irregular generally progressing to amenorrhea.1 In a review reported by Goldzieher and Axelrod, amenorrhea was reported in 47% of patients.7 Hunter reports a study where 70% of women with PCOS reported menstrual irregularities.1 However, there is a small percentage of women affected by PCOS who report regular menstrual cycles.

Signs of androgen excess include course hair growth in androgen-dependent areas of the body (sideburns, chin, upper lip, peri-areola, chest, lower abdominal midline and thigh), as well as truncal obesity, and acne.1,7 These signs may range from mild to moderate with approximately 70% of women reporting some form of hirsutism. Signs of virilization, though rare, may occur, including clitormegaly, voice changes, increased muscle mass, and temporal baldness.1

Obesity is another complicating factor of PCOS, but an explanation for the high prevalence of obesity among women with PCOS is unknown. It is generally seen as a central or truncal accumulation of adipose tissue and is evidenced by an increased hip-to-waist ratio (>0.8).9 Obesity may also contribute to menstrual irregularities/infertility and androgen excess as these conditions have been shown to improve with weight loss.2 Obese women with PCOS appear to have a higher prevalence of hirsutism than women of normal weight with PCOS:70% to 73% vs. 56% to 58% respectively.7 Menstrual irregularities were also more prevalent in obese women than non-obese:78% to 88% vs. 68% to 72% respectively.7 Women who do have hyperandrogenism along with obesity are at greater risk for dyslipidemia, i.e., increased triglycerides and low-density lipoproteins (LDL), and decreased high-density lipoproteins (HDL).5 It is important to remember however, that obesity does not necessarily have to be present for dyslipidemia to be present.

Acanthosis nigricans (AN), described as velvety, raised, pigmented skin typically found on the posterior neck, axillae, and within the mammary folds, is a skin condition often associated with PCOS. AN is evident in 1% to 3% of women with PCOS and more commonly found in those who are obese.7 An investigation for AN should be part of any physical exam for a patient with suspected PCOS. If found, AN should additionally alert the practitioner to the possibility of diabetes, hypertension, hyperlipidemia, and cancer.10 The symptom group of hirsutism, AN, and insulin resistance is known as the HAIRAN (hyperandrogenic-insulin resistance-acanthosis nigricans) syndrome and is likely a more severe form of PCOS.7,10

The classic descriptors of amenorrhea, hirsutism, infertility, and obesity do not all have to be present for the diagnosis of PCOS. Because there is a lack of agreement as to what actually constitutes PCOS, the definition from the 1990 National Institutes of Health/National Institute of Child Health and Human Development conference may be useful in determining if a patient has PCOS.11 This definition proposes the following criteria: a patient must have ovulatory dysfunction and evidence of clinical and laboratory substantiated hyperandrogenism without the presence of other causes of hyperandrogenism.