Background: The new technique of complete primary repair of bladder exstrophy (CPRE) has offered the promise of improved bladder functional outcomes, yet longitudinal follow up of exstrophy patients who have undergone this form of closure is sparse. We present our median 5 year data on patients who have undergone CPRE.
Methods: Patients who had CPRE were analyzed retrospectively and followed prospectively using case report forms, radiologic and clinical data, and semi-structured parental and patient interviews. We reviewed parameters including clinical events, scarring on renal scan, presence of vesicoureteral reflux, surgical procedures, urodynamic studies, urinary and fecal continence status and episodes of urinary tract infection.
Results: From 1994 to 2007, CPRE was performed within 72 hours of life in 20 boys and 8 girls. One patient underwent CPRE elsewhere. The median follow up time was 5 years (range 1-13 yrs) The 28 patients underwent a total of 209 surgical procedures of which 28% were minor endoscopic cases. 4 males (20%) underwent bladder neck reconstruction (BNR) at a median age of 4.5 years (range 4-8 yrs). 2 boys and one girl had Deflux™ injection to the bladder neck at 3-5 yrs of age. To date no patient has undergone bladder augmentation. 9 patients have had 1 to 4 episodes of pyelonephritis and 6 have cortical defects on renal scan. All patients were voiding or incontinent per urethra. One requires intermittent catheterization. Parents did not report any fecal incontinence or soiling in children over 4 years old. Four of 20 children (20%) older than 4 years have urinary continence periods of 3 hours or greater. Relative to males, females had better urinary continence and decreased need for BNR. These differences did not reach statistical significance.
Conclusion: CPRE has been shown to be safe and efficacious. Prospective follow-up of patients after CPRE reveals that few patients (20%) attain continent periods of >3hrs. Long-term follow-up of patients after CPRE continues to be necessary in order to establish the long-term effects of this procedure.