Long-term Follow-up of Exstrophy-epispadias Complex from a Lower-middle Income Country: A Case Report and Review of the Literature

Bladder exstrophy-epispadias complex (EEC) is a rare congenital defect where the abdominal muscles and bones fail to close in the mid-pelvis. It is crucial to understand the health-related quality of life (QOL) of exstrophy patients who have undergone multiple correctional surgeries. We herein discuss a case of bladder EEC that was repaired through a series of procedures at a resource-limited hospital in Karachi, Pakistan. A 21-year-old male, who was born with EEC, underwent bladder augmentation, Mitrofanoff procedure, bladder neck reconstruction, ureter implantation and a right nephrectomy in a single one-stage procedure during late childhood for urinary incontinence. However, this required a further revision because the urinary incontinence persisted, with difficulty in catheterizing the Mitrofanoff channel. On follow-up after 10 years, our patient currently describes normal QOL with near-normal sexual function. Validated questionnaires for QOL, erectile dysfunction, incontinence and prostatic function were used to assess the patient’s post-operative status in these domains. Through our report, we conclude that such patients can have a normal QOL by means of a holistic multidisciplinary management, which includes timely surgical corrections along with an additional focus on the psycho-social and sexual aspects of this condition.