Abstract
Aim: 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.Methods: 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).Results: 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 G1; 8 years (5 months–17 years) and 6.34 cm (4–12 cm) in G2 and 8.3 years (2–14 years) and 11.5 cm (11–12 cm) in G3. 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.Conclusion: Urogenital sinus mobilization for vaginoplasty in girls with CAH does not compromise voiding function or urinary continence.
Highlights
We called IT to those that needing an aggressive dissection to get to the vagina, still have enough urethra proximal to the vaginal confluence
The description of the technique of total urogenital mobilization (TUM) by Peña in 1997 [1] prompted us to apply the same principle to the correction of other forms of urogenital sinus (UGS) such as that found in congenital adrenal hyperplasia (CAH) [2]
MATERIALS AND METHODS Retrospective chart review of patients with CAH operated at our institution with intermediate and severe form of UGS since October 1996 that have been followed until they were toilet trained
Summary
The description of the technique of total urogenital mobilization (TUM) by Peña in 1997 [1] prompted us to apply the same principle to the correction of other forms of urogenital sinus (UGS) such as that found in congenital adrenal hyperplasia (CAH) [2]. We have applied the TUM technique to cases in which the urethra proximal to the confluence, as evaluated by contrast studies had adequate length. The advantage of this technique is that it avoids the often difficult separation of the urethra from the vagina. Some authors have raised concerns about a possible adverse effect the TUM could have on future urinary continence. We report the results regarding urinary continence in patients who were toilet trained at the time of surgery or who have become old enough to have sphincter control
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