DELAYED PRIMARY REPAIR OF BLADDER EXSTROPHY: ULTIMATE EFFECT ON GROWTH

Nima Baradaran, Raimondo M. Cervellione, Andrew A. Stec and John P. Gearhart

Division of Pediatric Urology, Brady Urological Institute, The Johns Hopkins Medical Institutions, Baltimore, Maryland (NB, JPG), Department of Pediatric Urology, Royal Manchester Childrens Hospital, Manchester, United Kingdom (RMC), and Department of Urology, Medical University of South Carolina, Charleston, South Carolina (AAS)

NEONATAL primary bladder exstrophy closure protect the bladder mucosa from environmental influences and offers the advantage of early bladder cycling leading to better bladder growth. prenatal diagnosis is not 100% accurate and still frequently missed.

Common reason for delayed exstrophy closure bladder template inadequate for a safe neonatal closure POSTPONE Primary closure of a bladder with a small template increases risk of dehisence bladder growth and urinary continence.

We examined longitudinal growth of the bladder in children who underwent delayed primary closure of bladder exstrophy due to either a small bladder template or a delayed referral, and compared bladder growth in these patients to children undergoing neonatal primary closure.

PATIENTS AND METHODS

bladder exstrophy database : patients born with classic bladder exstrophy between 1970 2006 6 years of follow up to assess longitudinal bladder growth. patients whose primary bladder closures were performed at our institution with available cystographic bladder capacity measurements between age 1 and their first continence procedure.

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3 treatment groups were identified according to the timing of bladder closure and indication for delayed closure : neonatal closure repaired within 30 days after birth. after 30 days due to small bladder template due to late referral to our institution

compare bladder growth trends among the 3 groups: cystographic BC. Gravity cystogram patient under anesthesia instillation of contrast ( 6Fr catheter at 30 cm above the symphysis ) bladder overinflated catheter balloon prevent leakage.

Statistical analysis : Stata, version 10. Descriptive statistics : Mann-Whitney and chisquare tests ( continuous and categorical measurements), p<0.05 statistically significant.

RESULTS

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740 classic bladder exstrophy cases: 18 delayed primary closure secondary to a small template, 15 delayed closure due to late referral 82successfully closed neonatally. 453 primary closure was performed elsewhere 145 had a history of primary closure failure 27 did not have repeated bladder capacity measurements excluded

Children electively delayed repair template underwent closure at an older age compared to those with delayed referral (median 10 months vs 6 months). A Cantwell-Ransley epispadias repair was performed in 23 of 28 boys (82%) at closure and pelvic osteotomy was performed in 32 of 33 patients (97%) at delayed primary repair

Consecutive cystographic BC measurements children undergoing closure due to small bladder templates had smaller capacities compared to those undergoing neonatal closure at all yearly measurements before their continence procedure. (except at age 2). Multivariate analysis (age at closure, gender and osteotomy status, age and bladder growth/capacity).

Delayed closure due to small template 36 cc smaller bladder volumes (p= 0.012) VS neonatal closure. Rate of annual bladder growth is not statistically significantly different between delayed closure with normal vs small templates (p= 0.7). Children undergoing delayed closure due to late referral (normal template) have overall 29 cc smaller bladders (p= 0.13)

Predicted annual bladder capacity growth in children undergoing neonatal closure (solid line) vs delayed primary closure of bladder exstrophy due to small bladder template (dotted line) or late referral (dashed line).

BNR

Neonatal group 42 of 82 patients (51%) underwent BNR at a median age of 5.5 years (range 3.2 to 14). 35 patients (83%) gained volitional voiding after BNR and 7 (17%) required continent diversion with bladder augmentation because of failed BNR. 9 patients(11%) were not suitable candidates for BNR, and underwent bladder augmentation and urinary diversion. Combined with BNR failures, 16 patents (19.5%) in this group needed diversion, 26 (32%) are still waiting for a continence procedure and 5 were lost to followup.

in the group with normal template who underwent delayed closure: 6 patients (40%) are still awaiting a continence procedure, 1 (6%) underwent a failed BNR and required diversion, and 2 (12%) underwent augmentation without BNR. Six patients in this group were lost to follow up.

DISCUSSION

Early bladder closure better bladder cycling during the newborn period and ultimately results in optimal bladder growth. primary exstrophy repair successfully performed in a delayed fashion. However,whether this delay negatively affects bladder growth .bladder potential is unknown

closure delay due to a small template vs normal template the difference in bladder size does not reach statistical significance and decreases with age. History of a failed primary closure is the ONLY independent risk factor precluding the bladder from optimal growth

Benefit of primary neonatal closure minimalization of environmental exposure of the bladder mucosa prevent metaplasia. (no evidence, microscopic changes in the bladder after delayed closure reverse to normal postoperatively)

bladders with long environmental exposures due to primary closure failure no plastic changes in any of the cellular layers were observed leaving the bladder extruded for a time does not seem to be harmful to the overall health and development of the detrusor muscle or bladder musculature

The ultimate goal of exstrophy repair achieve urinary continence and preserve renal function. The goal of this study to evaluate longitudinal BC. Therefore, patients who did not have annual measurements available were excluded.

CONCLUSIONS

Patients with small bladder templates delayed bladder closure smaller cystographic capacities compared to those with larger bladder templates (neonatal bladder closure). The timing of the closure did not modify the rate of bladder growth. Delayed exstrophy closure is a valid option

Recommend neonatal closure Further basic science studies about exact changes that occur to the bladder epithelium and musculature after birth and before definitive closure optimal condition for growth

TERIMA KASIH