THE USE OF COMBINED BLADDER AND EPISPADIAS REPAIR IN BOYS WITH CLASSIC BLADDER EXSTROPHY: OUTCOMES, COMPLICATIONS AND CONSEQUENCES

Abstract

Despite widespread use of modern staged reconstruction for classic bladder exstrophy, there remains a role for combined bladder closure and epispadias repair when primary closure is delayed or initial reconstruction has failed. The principle of combining bladder and urethral closure in 1 operation was first proposed more than 40 years ago, and represents a demanding technical procedure. We recount our experience to date with this approach. A total of 38 boys underwent combined bladder and epispadias repair using pelvic osteotomies. Five cases were delayed primary closures owing to a bladder template unsuitable for newborn closure. A total of 30 cases were previous failed bladder closures, with concurrent epispadias repair in 6. The 3 remaining cases were staged closures where the epispadias repair failed, leading to bladder prolapse via the posterior urethra. Overall, there was a major bladder prolapse in 25 cases, and separation of the pubic symphysis with dehiscence of anterior abdominal wall structures and bladder in 8. Mean age at surgery was 26.5 months for the 33 reclosures. A total of 10 boys had development of a urethrocutaneous fistula and 4 had development of strictures. A total of 19 patients required additional procedures of the bladder neck (endoscopic), urethra or penis. A later bladder neck reconstruction was undertaken in 19 boys, of whom 12 are continent. Seven boys underwent continent diversion and 5 are considering the procedure. A total of 14 boys are awaiting adequate capacity for bladder neck reconstruction. Ureteral reimplantation was performed in 22 patients, and no patient was rendered hypospadiac. When combined closure is applied to failed previous exstrophy repair one can expect at best a 50% continence rate without continent diversion. Furthermore, based on the experience at this institution the majority of patients require ureteral reimplantation, and many require additional surgery to the penis or urethra. However, with the application of modern reconstructive techniques continence and a cosmetically pleasing phallus can be expected in most cases, although at the expense of multiple surgical procedures.