Neurological Considerations for "Nerve-Sparing" Cosmetic Genital Surgeries Performed on Children with XX Chromosomes Diagnosed with 21-Hydroxylase Congenital Adrenal Hyperplasia and Clitoromegaly.

Abstract

Congenital adrenal hyperplasia (CAH) is most often caused by adrenal deficiency of 21-hydroxylase (21-OH). The resulting increase in androgens can cause clitoromegaly in fetuses with XX chromosomes. 21-OH CAH is the most common reason for cosmetic clitoroplasty in childhood. "Nerve-sparing" (NS) clitoral reduction surgeries are described as offering optimal cosmesis, while sparing sensation and nerve function. The methods used to demonstrate NS surgery efficacy, however, such as electromyography and optical coherence tomography, do not evaluate the small-fiber axons that comprise the majority of axons in the clitoris and that transduce sexual pleasure. Although some data show sparing of a portion of the main dorsal nerve trunk of the clitoris, the overall neurobiological consequences of elective clitoral reductions have received little attention. NS surgeries remove dorsal nerve branches that transduce sexual sensation, as well as the corpora cavernosa and cavernous nerve, which provide clitoral autonomic function. While most outcome studies focus on surgeons' perceptions of cosmetic results, studies that assess small-fiber function indicate significant nervous system and sexual impairment. Studies assessing children's clitoral function after surgery with vibrational testing have been ethically condemned. Decades of advocacy against medically unnecessary childhood genital surgeries have highlighted the subsequent physical and psychological harm. Recent studies with CAH patients indicate gender diversity and a lower prevalence of female gender identification than is often cited to justify feminizing surgery. The most effective and ethical NS technique for CAH may be acceptance of gender, sexual, and genital diversity as the infant develops into childhood, adolescence, and adulthood.