Summary objectives: 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.
Methods: 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.
Results: 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).
Conclusions: 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.