Feminizing genitoplasty for congenital adrenal hyperplasia: What happens at puberty?

Purpose: We document the postpubertal outcome of feminizing genitoplasty. Materials and Methods: A total of 14 girls, mean age 13.1 years, with conge nital adrenal hyperplasia were assessed under anesthesia by a pediatric uro logist, plastic/reconstructive surgeon and gynecologist. Of these patients 13 had previously undergone feminizing genitoplasty in early childhood at 4 different specialist centers in the United Kingdom. Results: The outcome of clitoral surgery was unsatisfactory (clitoral atrop hy or prominent glans) in 6 girls, including 3 whose genitoplasty had been performed by 3 different specialist pediatric urologists. Additional vagina l surgery was necessary for normal comfortable intercourse in 13 patients. Fibrosis and scarring were most evident in those who had undergone aggressi ve attempts at vaginal reconstruction in infancy. Conclusions: These disappointing results, even in the hands of specialists, highlight the importance of late followup and challenge the prevailing ass umption that total correction can be achieved with a single stage operation in infancy. Although simple exteriorization of a low vagina can reasonably be combined with cosmetic correction of virilized external genitalia in in fancy, we now believe that in some cases it may be best to defer definitive reconstruction of the intermediate or high vagina until after puberty. The psychological issues surrounding sexuality in these patients are inadequat ely researched and poorly understood.