The Management of Bladder Exstrophy: The Manchester Experience

Aim: Bladder exstrophy (BE) is a severe congenital malformation with life-long implications. This article discusses the current surgical management and describes the development of the nationally commissioned bladder exstrophy service in Manchester, UK. Methods: Outcome of BE surgery in Manchester was retrospectively reviewed. A Medline search was also undertaken and the published outcomes reviewed for the Modern Staged Repair of Bladder Exstrophy (MSRE), the Complete Primary Repair of Exstrophy (CPRE), and Radical Soft-Tissue Mobilisation (RSTM). Results: Sixty-seven infants with BE were treated in the period 2000-2012. Twenty-six infants underwent primary closure during the neonatal period, and in twenty-one this was successful. The remaining forty-one infants underwent delayed closure, and all were successful. Twenty-six children underwent MSRE, and continence with urethral micturition was achieved in sixteen (62%) (ten alone and six with urethral clean intermittent catheterisation). A further five (19%) are continent following bladder neck closure, cystoplasty, and continent diversion. Seven (26%) of the twenty-six patients are completely dry overnight. Twenty infants underwent primary ureteric reimplantation, and none have renal scarring. By contrast, renal scarring (unilateral n = fourteen; bilateral n = five) was found in nineteen of thirty-seven infants who did not undergo reimplantation. Conclusions: Specialised experience has allowed demonstrable improvement in bladder exstrophy outcomes throughout the period of the study. (C) 2014 Elsevier Inc. All rights reserved.