Abstract
Should the Genitoplasty of Girls with CAH be Done in One or Two Stages?
Highlights
The debate over the timing of surgical repair in girls with congenital adrenal hyperplasia (CAH) is intense and was in part started by patient advocacy groups largely composed of dissatisfied adults operated in childhood
One must keep in mind that girls with 21-hydroxylase deficiency, the most common form of CAH, are genotypically female, and have a normal potential for fertility and sexual function, and to date there have been no instances of gender dysphoria reported in this population
There is only one report suggesting that vaginoplasty done after puberty carries a lower rate of stenosis [9]
Summary
The debate over the timing of surgical repair in girls with congenital adrenal hyperplasia (CAH) is intense and was in part started by patient advocacy groups largely composed of dissatisfied adults operated in childhood. If we have to ban early genital reconstruction in girls with CAH, surgeons must be rational and consistent in their behavior and ban hypospadias repair (not to speak of most circumcisions) in infants and children who cannot give consent. Two recent articles in Frontiers in Pediatrics [2, 3] advocate or suggest seriously considering the surgical correction of the genital ambiguity in girls with CAH, in two stages: one early stage, to feminize the appearance of the external genitals, and vaginoplasty as a second stage after puberty.
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