An Eighteen Year Multi-institutional Report of Urinary Continence Following Complete Primary Repair of Bladder Exstrophy
Dana A. Weiss, MD1, Aseem R. Shukla, MD1, Joseph G. Borer, MD2, Bryan S. Sack, MD2, John V. Kryger, MD3, Elizabeth B. Roth, MD3, Travis W. Groth, MD3, Jennifer R. Frazier, MPH1, Michael E. Mitchell, MD3, Douglas A. Canning, MD1.
1Children's Hospital of Philadelphia, Philadelphia, PA, USA, 2Boston Children's Hospital, Boston, MA, USA, 3Children's Hospital of Wisconsin, Milwaukee, WI, USA.
Surgical repair of bladder exstrophy poses the challenge of achieving continence while preserving the upper tracts. Assessment of this goal has been elusive due to varied definitions of "dry." In an effort to better understand what it means, to patient and physician, to be "continent" or "dry" we devised and applied a categorization strata to our collective experience with complete primary repair of bladder exstrophy (CPRE). Based on previous single-institution experience, we hypothesized that about a third of patients with CPRE can void spontaneously with acceptable continence.
Prospectively maintained IRB approved databases were retrospectively queried at each institution to identify patients who underwent CPRE between 10/1996 and 1/2013. Exclusion criteria included: closure at other institutions, staged repairs, epispadias only, cloacal exstrophy, and no follow up since January 2014. Age at closure, continence at last follow up, bladder emptying, pads usage, uroflow findings, bladder capacity, and use of anticholinergic medications were evaluated.
We categorized patients into 2 groups: voiding or catheterizing. Each of these groups was stratified based on surgical procedures (see table). Patients were assigned a continence score of 1-5 (see table for categorizations). Voiding patients in groups 1 and 2 were subdivided by age > or < 10 years to determine if they had achieved his or her final continence potential.
A total of 73 patients who had undergone CPRE were identified and 57 met inclusion criteria. 40 void spontaneously, while 17 perform clean intermittent catheterization (CIC). Of the 40 voiding patients, 28 underwent CPRE only with no further surgeries for continence. 9/48 (18.7%) are category 4 or 5, 5/48 (10.4%) are category 3, and 11/48 (22.9%) are category 1 or 2. 7 of these 11 are still under 10 years of age. Six voiding patients underwent either deflux or bladder neck reconstruction and are category 3 or 4. Of the 17 CIC patients who catheterize, 2 underwent CPRE alone and perform CIC to empty, but are category 4 with this regimen, while 3 underwent only an appendicovesicostomy (APV) and are category 4 or 5. Two are category 5 after undergoing an augmentation cystoplasty and APV, and 5 are category 4 or 5 after having a bladder neck closure. One patient with bladder neck closure is category 2 and 3 are category 3.
Bladder exstrophy is a complex entity that does not lend itself to binary outcomes such as dry or wet, and the ability to void is an important outcome. It is only by specifically categorizing patient outcomes can we better understand our current and future successes and failures in achieving continence with voiding rather than simply being dry with catheterization. While almost 1/3 of patients can void with continence, it remains to be seen whether future results in a multi-institutional consortium will equal or surpass our previous outcomes.
|VOIDING n = 40||CIC n = 17|
|Dryness, continence definition stratification /// BE SURGICAL GROUP||CPRE only|
n = 28
|CPRE + BNR|
n = 4
|CPRE + BN Injxn|
n = 8
n = 3
|CPRE + Mitrofanoff n = 3||CPRE + Mitrofanoff + Augment|
n = 2
|CPRE + Mitrofanoff +|
Augment + BN Closure/BNR
n = 9
|1 wet||7 (4<10yrs)||1 (1<10yrs)||2 (0<10yrs)||1*||0||0||0|
|2 dry < 2 hours||4 (3<10yrs)||1 (0<10yrs)||1 (1<10yrs)||0||0||0||1|
|3 dry >= 2 hours||5||0||4||0||0||0||3|
|4 dry day only||4||2||0||2||1||0||1|
|5 dry day and night||5||0||0||0||2||2||4|
|0 – no info||3||0||1||0||0||0||0|
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