Stein Leventhal Syndrome

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Contents 1 Introduction 2 Epidemiology 3 Pathology 4 Clinical-manifestations 5 Laboratory 6 Radiology 7 Complications 8 Management 9 More General Terms 10 Additional Terms 11 Internet Database 12 References

Introduction

• Hyperandrogenism with chronic anovulation & polycystic ovaries. Primarily a clinical diagnosis. [3]

Epidemiology

• most common cause of hyperandrogenicity in women
• by transvaginal ultrasonography, 20-25% of all women have > 8-10 subcapsular cysts in both ovaries
• prevalence 6.6% [7a]; 10% of reproductive age women [8]

Pathology

• enlarged ovaries with increased stroma & thickened capsule
• anovulation secondary to increased androgen which is converted to estrogen in adipose tissue
• hyperestrogen state stimulates pituitary resulting in increased LH:FSH ratio propagating anovulatory state
• loss of LH surge
• risk of endometrial hyperplasia is increased
• insulin-resistance

• defects in phosphorylation pattern of insulin receptor

Clinical-manifestations

• amenorrhea or oligomenorrhea since menarche

• infertility, irregular or missed menstrual periods

• hyperandrogenism a hirsutism (70%), acne

• onset at puberty
• gradual onset, slow progression

• truncal obesity (50%), insulin resistance
• ancanthosis nigricans

Laboratory

• serum FSH is normal
• serum LH is normal or increased
• progesterone challenge test induces withdrawal bleeding
• serum testosterone is normal or modestly (< 2-fold) increased
• increased serum prolactin (20-25%)
• serum DHEA-sulfate is mildly increased in 25% of patients
• serum estradiol levels are normal
• serum estrone levels are increased
• very high free testosterone & serum androstenedione suggest androgen-producing neoplams
• increased serum insulin
• increased serum triglycerides [5]
• increased total cholesterol & LDL cholesterol [5]

Radiology

• pelvic &/or abdominal ultrasound

• symptom onset after menarche
• suspicion of ovarian or adenal mass [3]

Complications

• increased risk of endometrial hyperplasia & carcinoma
• hypertension is common
• diabetes is common
• increased risk for depression [10]
• excess risk for adverse pregnancy outcomes [12]

Management

• oral contraceptives after ruling out pregnancy

• with or without spironolactone
• useful for hyperadrogenism ( hirsutism & acne)

• spironolactone 50 mg daily reduces androgen levels [7]
• long-acting GnRH analogues
• androgen inhibitors

• flutamide
• cyproterone

• monitor for diabetes mellitus type-2

• metformin is the drug of choice
• metformin 500 mg TID may restore ovulation & fertility [4,6]
• pioglitazone may also be useful [11]

• clomiphene used to stimulate ovaulation in women trying to become pregnant
• monitor blood pressure
• weight reduction
• screen for depression [10]

More General Terms

• chronic endocrine disease
• ovarian disease

Additional Terms

• amenorrhea (oligomenorrhea)
• flutamide (Eulexin, Niftholide)
• oral contraceptive (OC)
• progesterone challenge test

Internet Database

OMIM: 184700

References

1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 389
2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 245
3. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998, 2006
4. Journal Watch 21(6):51, 2001 Vandermolen DT et al Metformin increases the ovulatory rate and pregnancy rate from clomiphene citrate in patients with polycystic ovary syndrome who are resistant to clomiphene citrate alone. Fertil Steril 75:310, 2001 PMID: [1]
5. Journal Watch 22(3):22, 2002 Legro RS et al Prevalence and predictors of dyslipidemia in women with polycystic ovary syndrome. Am J Med 111:607, 2001 PMID: [2]
6. Journal Watch 24(1):10, 2004 Lord JM et al Metformin in polycystic ovary syndrome: systematic review and meta-analysis. BMJ 327:951, 2003 [3] PMID: [4]
7. Journal Watch 24(14):115, 2004
   - Azziz R, Woods KS, Reyna R, Key TJ, Knochenhauer ES, Yildiz BO. The prevalence and features of the polycystic ovary syndrome in an unselected population. J Clin Endocrinol Metab. 2004 Jun;89(6):2745-9. PMID: [5]
   - Ganie MA, Khurana ML, Eunice M, Gulati M, Dwivedi SN, Ammini AC. Comparison of efficacy of spironolactone with metformin in the management of polycystic ovary syndrome: an open-labeled study. J Clin Endocrinol Metab. 2004 Jun;89(6):2756-62. PMID: [6]
8. Prescriber's Letter 11(10): 2004 Pharmacologic Treatment of Polycystic Ovary Syndrome Detail-Document#: [7] (subscription needed) [8]
9. The American Association of Clinical Endocrinologists (AACE) Position statement on metabolic and cardiovascular consequences of polycystic ovary syndrome [9]
10. Dokras AS et al. Increased risk for abnormal depression scores in women with polycystic ovary syndrome: A systematic review and meta-analysis. Obstet Gynecol 2011 Jan; 117:145. PMID: [10]
11. Prescriber's Letter 18(5): 2011 Metformin vs Pioglitazone for Polycystic Ovary Syndrome Detail-Document#: [11] (subscription needed) [12]
12. Roos N et al. Risk of adverse pregnancy outcomes in women with polycystic ovary syndrome: Population based cohort study. BMJ 2011 Oct 13; 343:d6309 PMID: [13]
    - Macklon NS. Polycystic ovary syndrome: Independently increases the risk of adverse pregnancy and birth outcomes. BMJ 2011 Oct 13; 343:d6407 PMID: [14]
13. National Guideline Clearinghouse Polycystic ovary syndrome American College of Obstetricians and Gynecologists (ACOG). ngc-guideline: [15]
    - Long-term consequences of polycystic ovary syndrome. (Royal College of Obstetricians and Gynaecologists) ngc-guideline: [16]
    - Diagnosis and management of polycystic ovarian syndrome. University of Texas at Austin School of Nursing, Family Nurse Practitioner Program ngc-guideline: [17]
    - Ovulation induction in polycystic ovary syndrome. Society of Obstetricians and Gynaecologists of Canada ngc-guideline: [18]

Stein-Leventhal (polycystic ovary) syndrome