[Feminizing genitoplasty in adrenal congenital hyperplasia: one or two surgical steps?]

The best time to perform a genitoplasty in a Congenital Adrenal Hyperplasia (CAH) girl is an issue that has been discussed extensively. The purpose of this study is to find criteria that may help in the decision.

Charts of all patients with diagnosis of CAH with 21 Hydroxylase deficit who underwent genitoplasty in our institution were reviewed (Jan 1996-Dec 2006). Demographic data, surgery performed and outcomes were analyzed.

In the 10 year-period, 25 patients fit the inclusion criteria; 22 had complete data. All patients were classified based on Prader's criteria; Prader 2 (n=3), Pra der 3 (n=13) and Prader 4 (n=6). Mean age at first surgery was 13.5 months (range 2-89 m). In Prader 2 patients, a reduction clitoroplasty with a "cut back" vaginoplasty was performed with no complications. All patients in the Prader 3 group underwent a reduction clitoroplasty. A vaginoplasty was done in 9/13; 5/9 at the same surgery session (4 stenotic) and the other 4 in a 2nd stage with good results; vaginoplasty is still pending for the other 4 girls. In the Prader 4 group, a vaginoplasty pull-through was performed in 4/6 using the posterior sagital approach; one at the reduction clitoroplasty stage which ended stenotic and need dilatations, and the other 3 in a 2nd surgery with a good outcome. The other 2/6 girls are awaiting a vaginoplasty. 22/22 had acceptable results after a mean follow-up of 63 months (range 12-144).

Congenital Adrenal Hyperplasia (CAH) shows different approaches may be used for different degrees of virilization. For less severe cases (Prader 3) a cut-back may be the surgery of choice for vaginoplasty, while in the more complex cases a flap with pull-through or a posterior sagital procedure could be useful. Based on this series, we recommend performing vaginoplasty in a 2nd stage surgery, avoiding complications and further procedures such as di lactations.