Management of the Ambiguous Genitalia

Hwang, Yun Joong; Woo, Young Nam

Abstract

It is well known that proper gender assignment and treatment to a neonate born with ambiguous genitalia are extremely important. We reviewed seven patients with ambiguous genitalia who were surgically managed at our department during recent 5 years. The median age was 12.1 years (from 3 to 24 years) and patients consist of three female pseudohermaphroditism (adrenogenital syndrome), one true hermaphroditism, one male pseudohermaphroditism and two mixed gonadal dysgenesis. Three patients were managed with clitoral recession and vaginoplasty, each of them with clitoral recession vaginoplasty and gonadectomy, with clitoral recession and gonadectomy, with clitoral recession, with gonadectomy and bilateral mastectomy. One patient with adrenogenital syndrome was raised as male, but re-assigned and surgically corrected as female at her age of 16 years. Another one patient with true hermaphroditism was raised as male who underwent excision of female internal genitalia, gonadectomy and bilateral mastectomy in considering of patient's gender identity, appearance of external genitalia and parent's proposal although the karyotype was 46 XX. We suggest that gender assignment and surgical correction must be done as early as possible after full evaluation of fertility feasibility, karyotype, sex ability and patient and parent's proposal.