Endocrine Abstracts

Objective: Successive female pseudohermaphroditism born to gestational hyperandrogenism accompanied by maternal virilization is extremely rare in literature.

Patient(s): A housewife, age 29, G2P1A1, revealed no hyperandrogenism before pregnancy. She gave her first child birth complicated by maternal virilization and female fetal pseudohermaphroditism due to hyperandrogenemia of bilateral 6-cm ovarian luteoma at age 27. Peak maternal serum testosterone level as high as 11539 ng/dl (normal: 20–86) was evident. Spontaneous regression of ovarian size and hyperandrogenemia during the puerperium revealed the natural course of pregnancy luteomas, not true neoplasms. She returned to regular menstruation without symptoms and signs of hyperandrogenemia the following two years except irreversible deepening voice in the aftermath of high androgen exposure. She conceived her second pregnancy at age 29. Elevation of maternal serum androgen level commenced as early as 5 weeks gestation, followed by rising androgen level that positively corresponds to acne formation and emerging facial hair by increasing gestational age. A 46 XX karyotype was confirmed after chorion villi sampling at 12 weeks gestation. Both parents made a fully informed decision to terminate the pregnancy until 14 2/7 weeks gestation. Maternal testosterone level reached 751 ng/dl while ovarian size is normal at termination.

Result(s): The abortus revealed apparently clitoral hypertrophy. The patient returns to normal androgen level two weeks later and free from virilization afterward, leaving lowering of her voice.

Conclusion(s): Placenta may be protective by virtue of its high capacity to convert androgens to estrogen. Conversion of testosterone to oestradiol was inadequate to protect from high maternal testosterone concentration and, undoubtedly, this fetus would have virilised if female in our observation (1). The risk for male fetus is unknown. Expectant management is the treatment option as there are no pharmacological options which are safe in pregnancy. Imprudent surgical intervention should be withheld in this regard.