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

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.

Keywords: congenital adrenal hyperplasia; high urogenital sinus; intermediate urogenital sinus; urinary continence after urogenital reconstruction; urogenital sinus mobilization.

Figure 1

Genitography is very useful for the study of vaginal morphology, dimension, and relation to the urethra. Depending on vaginal confluence in the UGS was classified in (A) low, (B) intermediate, and (C) high variant. The white arrows show the confluence of vagina in the UGS.

Figure 2

Dissection of the posterior vaginal wall, separating it from the rectal wall before sectioning the UGS. A rectal finger is very useful to facilitate vaginal exposure.

Figure 3

“Total urogenital mobilization”: TUM in lithotomy position in an intermediate UGS.

Figure 4

Mobilization the UGS in the lithotomy position without opening it in a high variant. A balloon catheter has been previously inserted in vagina.

Figure 5

Prone position. The modified anterior sagittal transrectal approach (ASTRA). Note that the balloon catheter was previously inserted in vagina. The midline perineal incision is extended around the anocutaneous junction between 4 and 8 h to get better exposure. The rectum is retracted using sutures and a retractor to avoid opening the anterior rectal wall as proposed in the ASTRA technique.

Figure 6

(A,B) The vagina opened over the balloon, the foley catheter repositioned in the proximal urethra. The long arrow shows the length of the UGS.

Figure 7

Diagram of the UGS transected ventrally (patient is in prone position) and everted to reach the anterior wall of the vagina. In this way, the proximal part of it stays as urethra.

Figure 8

UGS everted to reach the anterior vaginal wall.

Figure 9

Postoperative aspect of a vaginoplasty after an aggressive dissection of the posterior vaginal wall bringing the mucosa out to a wide perineal flap.