The Surgical Correction of Urogenital Sinus in Patients with DSD: 15 Years after Description of Total Urogenital Mobilization in Children

The surgical treatment of persistent cloaca is a serious challenge. The operation is technically difficult and the final results for urinary and fecal function are far from excellent. The repair of a cloaca includes, among other maneuvers, the separation of the vagina from the urinary tract. This step is a serious technical challenge and is very time consuming. Devascularization of these structures is the main source of complications such as urethro-vaginal fistula, vaginal stricture, and acquired vaginal atresia. To avoid these complications and to facilitate the cloacal repair, a new technical variation using total urogenital mobilization was performed in 11 patients. In this procedure, after the rectum is separated from the vagina, both the urethra and the vagina are mobilized together as a single unit. The surgical time spent during the reconstruction was reduced by approximately 70%. All patients recovered well from the operations and have been followed up for 1 to 14 months. The blood supply of the vagina and urethra in all cases remained excellent. No patient developed urethrovaginal fistula, vaginal stricture, or acquired vaginal atresia. The cosmetic appearance in these patients is superior to the one achieved with previous techniques. Although this maneuver may not render better urinary or fecal control, the urethra is more accessible for catheterization. These preliminary results suggest that the total urogenital mobilization maneuver provides a definite technical advance in the repair of cloaca malformations.

The treatment of urogenital sinus malformations is complex and controversial. Despite numerous and significant contemporary surgical advances, the dissection of the urogenital sinus remains technically challenging. Based on total urogenital mobilization, we describe a technique whereby this dissection is limited to the pubourethral ligament. Our short-term results with partial urogenital mobilization (PUM) performed on 15 patients are retrospectively reviewed. There were no intraoperative complications and the short-term cosmetic results are excellent. No patients have developed voiding dysfunction or urinary tract complications. While total urogenital mobilization is a very effective procedure, we believe that the PUM approach limits potential morbidity in the reconstruction of these complex problems.

A new 1-stage procedure for clitorovaginoplasty in severely masculinized female pseudohermaphrodites is presented. The technique consists of clitoral reduction with preservation of the neurovascular supply to the glans and vaginal formation by joining the inverted penile skin and the divided distal "urethra" (urogenital sinus) to form a cylinder that is then inserted into the perineum and anastomosed to the true vagina. For high and small vaginas Monfort's transtrigonal approach is suggested. This operation, 4 of which have been performed to date, can be done at any age, and it offers excellent cosmetic and functional results.