Urinary Continence Following Repair of Intermediate and High Urogenital Sinus (UGS) in CAH. Experience with 55 Cases.

[congenital adrenal hyperplasia]

To evaluate postoperative urinary continence in patients with congenital adrenal hyperplasia (CAH ) with intermediate (IT ) and high urogenital sinus (UGS ) who underwent a UGS mobilization maneuver .We called IT to those that although needing an aggressive dissection to get to the vagina , still have enough urethra proximal to the vaginal confluence . Very low variants are excluded from this analysis . Dissection always started in the posterior wall of the UGS with an aggressive separation from the anterior rectal wall . If the wide portion of the vagina was reached dissection stopped and the UGS opened ventrally widening to the introitus . Nineteen patients were treated using this maneuver (Group 1 ). When more dissection was required the anterior wall of the UGS was dissected and carefully freed from the low retropubic space . Then the UGS was opened either ventrally or dorsally . Thirty three patients required this approach (Group 2 ). Combined procedures were used in three patients with high UGS (Group 3 ).Mean age at the time of the repair and length of the UGS were 12 .2 years (4 months -18 years ) and 3 .75 cm (3 -8 cm ) for G 1 ; 8 years (5 months -17 years ) and 6 .34 cm (4 -12 cm ) in G 2 and 8 .3 years (2 -14 years ) and 11 .5 cm (11 -12 cm ) in G 3 . All patients had been regularly followed . Mean age at last follow up was 14 .3 , 17 , and 9 .9 years for Groups 1 , 2 , and 3 , respectively . All patients continue to void normally and are continent . All patients have two separate visible orifices in the vulva . Only three are sexually active .Urogenital sinus mobilization for vaginoplasty in girls with CAH does not compromise voiding function or urinary continence .