The management of bladder exstrophy: the Manchester experience

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.

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).

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.

Specialised experience has allowed demonstrable improvement in bladder exstrophy outcomes throughout the period of the study.