Early one-stage surgical reconstruction of the extremely high vagina in patients with congenital adrenal hyperplasia.

High vaginal atresia is a very rare anomaly seen in the most severely masculinized females with congenital adrenal hyperplasia. These children have a foreshortened vagina conjoining the urogenital sinus proximal to the external urethral sphincter. In the past, they have undergone early clitoral recession and labioscrotal reduction, followed by vaginal pull-through at 2 to 4 years of age. Cumulative experience with repair of this anomaly has led us to attempt earlier one-stage intervention and to develop techniques that circumvent previously encountered vaginal stenoses. One-stage reconstruction of three older children (ages 2 to 9 years) involved: closure of the urethrovaginal fistula, mobilization of the vagina from the rectum and urethra, use of bilateral buttock flaps to augment the anterior vaginal wall, augmentation of the posterior wall with an inverted perineal U flap, clitoral recession, and advancement of labioscrotal and clitoral shaft flaps inferiorly to create labia majora and minora (respectively). The introiti were quite capacious after employing such flaps, did not require postoperative dilatation, and were free of strictures or urethrovaginal fistulae during long-term follow-up. Three younger patients were seen for initial evaluation at 8 to 12 months of age, when early one-stage reconstruction was undertaken. Paradoxically, these repairs were technically less difficult and did not require buttock flap augmentation because an island of anterior perineal skin could be rotated in to reach the anterior vaginal wall. A nerve stimulator was used to identify the external urethral sphincter, while the vagina was aggressively mobilized and advanced forward beyond the site of fistula closure on the urethra to avert formation of a urethro-vaginal fistula.2 +