FEMINIZING GENITOPLASTY FOR CONGENITAL ADRENAL HYPERPLASIA: WHAT HAPPENS AT PUBERTY?

TLDR

The study underscores the inadequate research and poor understanding of psychological issues related to sexuality in girls with congenital adrenal hyperplasia undergoing feminizing genitoplasty. The authors evaluated 14 postpubertal girls with congenital adrenal hyperplasia, assessing outcomes of prior feminizing genitoplasty performed at four UK centers through multidisciplinary anesthesia‑based examinations. Most patients required additional vaginal surgery, with fibrosis and scarring especially in those who had aggressive infant reconstruction, indicating that single‑stage infancy correction is often insufficient and that delaying intermediate or high vaginal reconstruction until after puberty may be preferable.

Abstract

We document the postpubertal outcome of feminizing genitoplasty.A total of 14 girls, mean age 13.1 years, with congenital adrenal hyperplasia were assessed under anesthesia by a pediatric urologist, 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.The outcome of clitoral surgery was unsatisfactory (clitoral atrophy or prominent glans) in 6 girls, including 3 whose genitoplasty had been performed by 3 different specialist pediatric urologists. Additional vaginal surgery was necessary for normal comfortable intercourse in 13 patients. Fibrosis and scarring were most evident in those who had undergone aggressive attempts at vaginal reconstruction in infancy.These disappointing results, even in the hands of specialists, highlight the importance of late followup and challenge the prevailing assumption 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 infancy, 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 inadequately researched and poorly understood.