Purpose: The major goals of complete primary repair of exstrophy (CPRE) are the re-creation of normal anatomy which allows bladder cycling and to reduce the number of future procedures necessary to achieve continence. It is unclear whether CPRE is associated with a higher bladder neck fistula rate than the traditional staged repair. We review a multi-institutional experience with the CPRE technique to evaluate the rate of bladder neck fistula.
Materials and methods: A retrospective review of 18 boys and 4 girls who underwent CPRE at 4 tertiary pediatric urology referral centers during the last 6 years was performed. All pertinent technical aspects were reviewed, including timing of procedure, whether osteotomies were performed, and number of layers used to reconstruct the bladder neck and urethra. Complications were noted, especially that of bladder neck fistula.
Results: Mean followup was 22.6 months. Of the patients 14 (64%) underwent primary closure within the first 48 hours of life and only 1 required osteotomies. The remaining 8 patients underwent closure between 5 days and 3 months of age (mean 24.6 days) and all required osteotomies. Bladder neck fistula occurred postoperatively at the pubic junction in 9 males (41%). Four cases had a 2 layer closure that was covered with a single layer small intestinal submucosa onlay (Surgisis) and no patient had a fistula. Fistulas developed in 62.5% of patients with delayed closure vs 29% of those with immediate closure (p = 0.135). Two fistulas closed spontaneously and 7 required surgical closure at a mean of 7.5 months after the fistula occurred.
Conclusions: This multi-institutional study demonstrates that bladder neck fistulas occur in almost half of patients following CPRE by experienced pediatric urologists. While spontaneous closure is possible, most will eventually require repair. The long-term implications of this finding with regard to continence and the need for additional bladder neck procedures remain to be seen. We are encouraged by the preliminary results of small intestinal submucosa coverage and will continue to evaluate its use at the time of primary exstrophy closure.