What is Stein Leventhal Syndrome?
Stein Leventhal Syndrome is a hormonal disorder affecting female reproductive system. In this syndrome, elevation of androgen hormone in female leads to irregular ovulation. The irregular ovulation is one of the major factors for infertility of the affected female. Other different associated menstrual symptoms are prominent for children.
The synonym of Stein Leventhal Syndrome is polycystic ovarian syndrome. The reason behind this synonymous name can be described as this syndrome was first illustrated by Irving F. Stein, Sr., and Michael L. Leventhal. These American gynecologists described that the presence of ovarian cysts is the cause of reproductive hormonal abnormalities and resultant of that cause anovulation. [1,2,3]
Signs & Symptoms
The associated symptoms of Stein Leventhal Syndrome are:
- Infertility due to irregular ovulation
- Irregular menstruation or absence of menstruation
- Pain in pelvis
- Skin problems, such as oily skin, acne
- Thickened skin with dark brown or black patchy skin
- Hair problems like dandruff, thinning hair or baldness
- Premature development of pubic hair
- Excessive midline body hair with facial hair growth
- Enlargement of the clitoris
- Altered voice (male like)
- Decreased breast size
The included sign are elevated levels of androgens, hirsutism and acne. The diagnostic findings associated with Stein Leventhal Syndrome are as follows:
- Enlarged ovaries with 4 -6 cm diameter.
- Grayish- white surface
- Single layered germinal epithelium is replaced by the thickened capsule
- Several distinct cysts with minimum 2 mm to maximum 10 mm diameter
The onset of the sign and symptoms or progression of the condition varies. The syndrome can develop before puberty or after puberty and even after menopause also. Symptomatic approach differs with the onset of syndrome. The symptomatic diagnosis become difficult in development of the syndrome after menopause. Frequent diagnostic results showed that generally in between age of 20s or 30s women diagnosed with Stein Leventhal Syndrome. (2,3,4,5)
The two major hormones estrogen and progesterone, involve in releasing mature eggs from the ovaries are estrogen and progesterone. Normal, healthy women release one or more eggs during each cycle (ovulation). Stein Leventhal Syndrome is associated with hormonal alteration that cause difficulty to the ovaries in releasing mature eggs.
The responsible factor for development of the Stein Leventhal Syndrome is a metabolic abnormality of androgens and estrogen and abnormal rate of androgen production. The elevated level of androgen hormone, including testosterone, androstenedione, and dehydroepiandrosterone sulfate (DHEA-S) often found in the affected females. But in some cases, females may also have normal androgen levels.
The exact cause of elevated androgen level is not yet confirmed by the scientific study. Some risk factors which have direct or indirect relationship with elevation of androgen. Following are the considerable risk factors:
- Peripheral insulin resistance
- Gonadotropin dynamic dysfunction
- Increased stimulatory effect of luteinizing hormone (LH)
- Elevated levels of plasminogen activator inhibitor-1 (PAI-1) (1,3,4,6)
Physical examination, including pelvic examination is an initial diagnostic test. The following findings are associated with Stein Leventhal Syndrome:
- Inflamed ovaries
- Rare findings showed that inflated clitoris
Other findings of physical examination are:
- Increased weight
- Increased blood pressure
The different types of blood test are conducted for estimating the following measures:
- Estrogen level
- FSH level
- LH level
- Male hormone (testosterone) level
Apart from the above mentioned blood tests, the following blood tests may require to conduct:
- Fasting glucose and other related tests to estimate the glucose intolerance and insulin resistance
- Lipid profile
- Prolactin level
- Pregnancy test (serum HCG)
- Thyroid function tests
Diagnosing staff check waist size, BMI (body mass index)
The most common findings for above mentioned test results are high blood sugar level (diabetes), high cholesterol level (hypercholesteromia) and obesity of the affected women.
Doctor also recommend to imaging and surgical intervention for detail observational purposes:
- Vaginal ultrasound
- Pelvic laparoscopy (4)
- The losing weight and control blood sugar level are two important management criteria in Stein Leventhal Syndrome. In general, dietary measure and exercise regimen are advisable treatment approach.
- Diabetes controlling medicine like Glucophage is prescribed if the above mentioned measure fails to provide desirable effect. Glucophage assist to control blood sugar level, weight management and irregular menstrual cycle management.
- LH-releasing hormone (LHRH) analogous medicine also prescribed to manage estrogen and progesterone hormone balance to normalize ovulation.
- Clomiphene citrate is the drug which assisting ovulation to become pregnant.
- Doctor also recommended that spironolactone or flutamide pills and eflornithine cream to limit the excessive hair growth in body.
- Contraceptive pills can help to control hirsutism and menstrual irregularities for women who completed their family planning or do not wish to become pregnant.
- Laparoscopic pelvic surgery may suggest to remove or correct an ovary to treat infertility, though the recurrence is common in most of the cases. (2,4)
- Richard Scott Lucidi (2015), Polycystic Ovarian Syndrome; Retrieve from: http://emedicine.medscape.com/article/256806-overview#a4
- Definition of Stein-Leventhal syndrome; Retrieve from: http://www.medicinenet.com/script/main/art.asp?articlekey=5553
- Polycystic ovary syndrome; Retrieve from: https://medlineplus.gov/ency/article/000369.htm?session=5H3vSHbClmlGc4HsySKW4YrBBP
- Rechard S. Legro, Stein Leventhal Syndrome; Retrieve from: https://www.britannica.com/science/Stein-Leventhal-syndrome
- Irving F. Stein, Sr., The Stein–Leventhal Syndrome — A Curable Form of Sterility; N Engl J Med 1958; 259:420-423August 28, 1958DOI: 10.1056/NEJM195808282590904; http://www.nejm.org/doi/full/10.1056/NEJM195808282590904
- Hoyt KL, Schmidt MC.; Polycystic ovary (Stein-Leventhal) syndrome: etiology, complications, and treatment; Clin Lab Sci. 2004 Summer;17(3):155-63. Retrieve from: http://www.ncbi.nlm.nih.gov/pubmed/15314890