Short-term outcomes of the multi-institutional bladder exstrophy consortium: Successes and complications in the first two years of collaboration

SummaryIntroduction/backgroundBladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care.ObjectiveOutcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term.Study designBoston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures.ResultsFrom February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below.DiscussionAbsence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence.ConclusionThis collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing.Summary Table. Diagnosis, timing of CPRE and complications per gender.GenderGirlsBoysClassic BE/epispadias10/312/2Median age at CPRE (range)1.9 months (0.1-51.6)2.9 months (0.4-28.8)SuccessesNo dehiscence 7 without complicationsNo dehiscence 11 without complicationComplications6 girls total 5 pyelonephritis 4 urinary retention (BOO) 2 temporary CIC 1 vesicostomy 1 bladder rupture3 boys total 1 pyelonephritis 2 urethrocutaneous fistula