534 obstetrics and Gynecology Board review •••
❍ What is the diagnosis of a female patient with XY karyotype and palpable Müllerian structures but lack of
sexual development?
Swyer syndrome (remove gonads immediately at diagnosis as they have a high incidence of malignancy).
❍ What is the cause of Swyer syndrome in at least 10% to 15% of affected individuals?
Mutation in the SrY gene.
❍ What is the most common cause of secondary amenorrhea?
Pregnancy.
❍ Describe the initial laboratory evaluation for secondary amenorrhea.
hCG, TSH, PrL, progestin withdrawal, and FSH. Consider testosterone, DHEAS, and pelvic ultrasound for
investigating polycystic ovarian disease.
❍ How is a patient’s estrogen status and competence of the outflow tract assessed?
Administer medroxyprogesterone acetate (MPA) 5 or 10 mg for 5 to 10 days, to determine whether the patient
bleeds after withdrawal of the medication. Alternatively, can use 200 mg IM progesterone in oil or micronized
progesterone 300 mg QD.
❍ How much bleeding is needed for a positive withdrawal response in a progestational challenge?
Any amount of bleeding no matter how scant.
❍ What percentage of women with amenorrhea and low estrogen levels (from exercise, weight loss, ovarian
failure, etc) will actually have a withdrawal bleed from a progestin challenge test, i.e. false-positive results?
40% to 50%.
❍ If no withdrawal bleed occurs, what is the next step?
To add estrogen prior to the progestin withdrawal.
(1.25 mg conjugated estrogen or 2 mg of estradiol daily for 21 days with 10 mg of Provera added for the last 5 days).
❍ What is the treatment of Asherman syndrome?
Hysteroscopy with lysis of adhesions, +/- method to keep the uterine cavity open and high-dose estrogen for 2 months
(add MPA 10 mg daily after the third week) and broad-spectrum antibiotics pre-op and 10 days afterward.
❍ Name the methods often used to keep the uterine cavity open.
IUD or pediatric Foley filled with 3 mL of fluid left in for 7 days.
❍ What percentage of patients with Asherman syndrome have achieved a successful pregnancy after surgical
lysis of the intrauterine adhesions?
70% to 80%.
❍ What complications may these patients then experience with their pregnancy?
Preterm labor, placenta accreta, previa, and postpartum hemorrhage.