Complete repair of bladder exstrophy: Preliminary experience with neonatesand children with failed initial closure

Purpose: The surgical repair of bladder exstrophy remains challenging for t he pediatric urologist. We present our preliminary experience with complete primary repair of exstrophy in neonates and children with failed initial c losure. Materials and Methods: Between November 1998 and April 1999, 9 boys and 2 g irls with bladder exstrophy underwent complete repair. This procedure was p erformed in the first 72 hours of life in 4 boys and at age 3 months in 1 g irl. Complete repair with osteotomy was performed after failed initial clos ure in 5 boys and 1 girl at a mean age of 28 months (range 15 to 36). The b ladder and urethra were closed in continuity and epispadias was repaired by total penile disassembly. All patients were kept in a spica cast for 3 wee ks. Ureteral stents and suprapubic tube were removed 10 and 14 days, respec tively, after surgery. Ultrasound was performed preoperatively and every 3 months postoperatively, voiding cystourethrography was done 6 to 12 months after surgery. Results: Mean followup is 14 months (range 12 to 17). All repairs were succ essful including 1 case of penile scrotal duplication. Concomitant augmenta tion was done in 2 girls due to a small bladder plate. Complete closure res ulted in hypospadias in 3 of the 9 boys. No patient had dehiscence or fistu la. Serial followup ultrasound revealed no hydronephrosis and normal renal growth. Febrile urinary tract infection occurred in 1 case 2 months after s urgery and was managed conservatively. Bladder capacity was 200 and 270 ml. , at 6 months, respectively, in the 2 patients with an augmented bladder an d both are dry on intermittent catheterization. The 4 patients in whom the closure was performed at birth are voiding with dry intervals with mean bla dder capacity of 15 cc at 1 year (range 60 to 90). The 5 older children had a mean bladder capacity of 120 cc (range 70 to 150) at 6 months, of whom 2 are completely continent and 3 have 1 to 3 hours of dry intervals. Conclusions: Complete repair of bladder exstrophy is feasible in neonates a nd children after failed initial closure with minimal morbidity. There is n o short-term evidence of worsening reflux or hydronephrosis. Longitudinal. followup with adequate recording is required for continence evaluation. Thi s technique may minimize the future need of bladder neck reconstruction and augmentation in patients with exstrophy.