LAUSR

Successful pregnancy with congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency is an extremely rare condition. Only two cases have been reported in the literature. Described here is a 30-year-old woman diagnosed as a neonate with congenital adrenal hyperplasia related to 11-beta-hydroxylase deficiency classic type, who subsequently underwent clitoral resection and vaginoplasty. She was started on lifelong steroid therapy after surgery. She developed hypertension at 11 years of age and was on antihypertensive therapy from then on. In later life, she underwent division of vaginal scar tissue and perineal refashioning. She spontaneously conceived but her pregnancy was complicated by severe pre-eclampsia and delivery was required at 33 weeks of gestation by cesarean section. A healthy male infant was delivered. Management of these women is similar to those with more common causes of congenital adrenal hyperplasia, with careful monitoring throughout pregnancy for complications such as gestational diabetes, gestational hypertension, and intrauterine growth restriction.