The Surgical Management of Infants and Children With Ambiguous Genitalia Lessons Learned From 25 Years

TLDR

Children with ambiguous genitalia present a wide spectrum of anatomic and physiologic anomalies, and successful management requires individualized, long‑term evaluation that integrates cosmetic, endocrinologic, social, psychological, and sexual factors. The study reports 25 years of surgical management of 91 children with ambiguous genitalia. Female sex assignment was made for 79 of the 91 patients, with 60 undergoing clitoral reconstruction and 42 receiving vaginal reconstruction; long‑term follow‑up showed satisfactory anatomic and functional outcomes for clitoral surgery alone, while extensive vaginal reconstruction yielded compromised functional results, underscoring the importance of prompt assignment, early accurate diagnosis, conservative clitoral reconstruction before age one, and tailored vaginal reconstruction.

Abstract

Over a 25-year period, 91 children with ambiguous genitalia have received surgical management. Female sex assignment was made for 79. Of these, 60 patients underwent extensive clitoral reconstruction consonant with the female assignment. Forty-two patients had vaginal reconstruction. Factors relating to success include: (1) prompt and appropriate sex assignment; (2) early and accurate diagnosis; (3) conservative reconstruction of the clitoris at an early age (less than 1 year); and (4) choice of vaginal reconstruction based on the severity of the malformation. Long-term follow-up demonstrates satisfactory anatomic and functional results when clitoral surgery alone was required. Functional results for patients with extensive vaginal reconstruction have been compromised. Physicians caring for children with congenital intersexual anomalies can expect to encounter a wide spectrum of anatomic and physiologic derangements. Cosmetic appearance alone is an inadequate measure of success because endocrinologic, social, psychological, and sexual factors must be blended into comprehensive evaluation of these patients. The management plan must be flexible and individualized, incorporating long-term follow-up to adulthood.