Hermaphrodism: Recommendations Concerning Case Management

Many a physician might understandably be nonplussed upon being presented for the first time with a case like the following: C.L. (HLH#B-6544), aged 6 years and 2 months, living as a boy, had been referred to he pediatric endocrine clinic of the Harriet Lane Home from another hospital where a diagnosis of female pseudohermaphrodism due to congenital adrenal hyperplasia had been established two years earlier.1 Recommendations were sought regarding future management; the question of a reassignment of sex was raised. The parents gave a story of harrowing indecision following the child's birth. Originally given a girl's name, the baby was 6 months old when a specialist consultant unequivocally declared the child to be a cryptorchid hypospadiac male. Laparotomy was not performed at that time and the appearance of pubic hair was dismissed as unimportant. Thenceforth the child's legal status was changed to that of a male and he was given a boy's name. At the age of 6, should this child have been left to live as a boy, or reassigned to live as a girl?2