Complete repair of exstrophy: further experience with neonates and children after failed initial closure.

Abstract

The surgical repair of bladder exstrophy remains challenging for the urologist. Recently, complete primary repair has been used in neonates. We present our experience with this approach in neonates and children after failed initial closure. Between November 1998 and November 2000, 17 boys and 2 girls with bladder exstrophy underwent complete repair. Complete primary repair was performed in the first 72 hours of life in 4 boys. Complete repair with osteotomy was at a mean age +/- SD of 23 +/- 21 months (range 1 to 74) in 15 patients including 7 with failed initial closure. The bladder and urethra were closed in continuity with epispadias repair by total penile disassembly. All patients were kept in spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days after surgery, respectively. Ultrasound was performed before surgery and 3 months thereafter, and voiding cystourethrography was obtained 3 months postoperatively and then annually in all cases. Complete closure resulted in hypospadias in 10 boys (59%). There was no major complication. Mean followup +/- SD was 17 +/- 8 months (range 5 to 33). Temporary suprapubic urinary leakage was noted initially in 10 cases (52%) but no patient had persistent fistula. Initial postoperative renal ultrasound revealed hydronephrosis in 11 renal units (29%). However, at last followup only 1 renal unit (2%) showed pelvicaliceal dilatation. Two patients (10%) had a febrile urinary tract infection and were treated conservatively. Reflux was noted in 24 renal units (63%) but did not require surgery. The 4 boys in whom the closure was performed at birth had regular voiding with 60 to 90-minute dry intervals and mean bladder capacity +/- SD was 85 +/- 35 cc. The 15 older children had a mean bladder capacity of 74 +/- 37 cc and 5 (33%) had regular voiding with 30 to 90-minute dry intervals. Complete repair of exstrophy is feasible in neonates and older children including, those with failed initial closure with minimal morbidity. There is a short-term evidence of favorable outcome in newborns. Longitudinal followup is required to determine the future need of bladder neck reconstruction and augmentation in older patients.