388 / 492
VR-27
(VS)
THE MULTI-INSTITUTIONAL BLADDER EXSTROPHY CONSORTIUM: TECHNICAL
STANDARDIZATION OF COMPLETE PRIMARY REPAIR OF EXSTROPHY IN THE
GIRL
Joseph G. BORER
1
, Evalynn VASQUEZ
1
, Douglas A. CANNING
2
, John V. KRYGER
3
, Travis GROTH
3
, Dana WEISS
2
, Aseem
SHUKLA
2
, Alexandra BELLOWS
1
and Michael E. MITCHELL
3
1) Boston Children's Hospital, Urology, Boston, USA - 2) Children's Hospital of Philadelphia, Urology, Philadelphia, USA -
3) Children's Hospital of Wisconsin, Urology, Milwaukee, USA
INTRODUCTION
To improve our experience and proficiency in the care of bladder exstrophy (BE), we formed the Multi-Institutional BE
Consortium (MIBEC). In this video, we describe the standardization of complete primary repair of BE (CPRE) in the
female as developed through this collaboration.
MATERIAL AND METHODS
Three institutions alternately served as hosts with commentary, critique and teaching by collaborating surgeons via
direct observation or real-time video transmission. Employing the MIBEC method and protocol, CPRE with bilateral iliac
osteotomy was performed between 1-3 months of age. Patients were prospectively followed for outcomes including
complications.
RESULTS
From February 2013-February 2015, MIBEC surgeons performed CPRE in 13 consecutive girls at median age of 1.9
months (0.1-51.6 months) for 10 classic BE and 3 epispadias patients. There was no dehiscence. Hydronephrosis of mild
grade was present in 3 girls and moderate in 5; 5 had unilateral or bilateral hydroureter. Pyelonephritis of ≥1 episode
occurred in 5 girls, and 4 girls had varying degrees of urinary retention. Two were managed successfully with temporary
clean intermittent catheterization (CIC), and 2 developed complete retention; 1 with a stenotic bladder outlet will
require diversion to vesicostomy, and 1 with meatal stenosis resulting in bladder rupture continues CIC after repair.
Changes in the sequence of perineal reconstruction and urethral maturing, relative to symphyseal approximation, have
been made due to obstructive complications. A more gradual tapering of the bladder neck into the proximal urethra has
also been incorporated.
CONCLUSIONS
CPRE in girls is an evolving process with each patient benefiting from the last. We have modified our technique through
the MIBEC to improve outcomes and decrease complications.