Bladder exstrophy: navigating long-term outcomes

Abstract

Bladder exstrophy (BE) is a rare congenital malformation of the genitourinary tract, as well as surrounding structures, within the spectrum of the exstrophy-epispadias complex. This review aims to summarize the current literature on repair procedures, specifically the modern staged repair and complete primary reconstruction, along with their associated complications and complication rates. A particular focus is placed on long-term management strategies to address these complications as patients transition into adulthood. Additionally, this review highlights the management of sexual health for both males and females, fertility considerations, and overall quality of life, emphasizing that patient care extends beyond voiding function. By incorporating these broader aspects, the review provides a global perspective on patient management, which is crucial for the entire multidisciplinary care team. Due to the rarity of BE, the available data is inherently limited, and findings may not be generalizable to every patient. Despite these challenges, this review synthesizes the existing literature through comprehensive searches conducted on PubMed and Google Scholar, thematically organizing findings to identify well-recognized complications and their management. The importance of multidisciplinary care, extending beyond pediatric urology and including adult urologists, is critically emphasized to ensure a seamless transition of care as patients age. Finally, this review underscores the need for ongoing research to establish standardized long-term management protocols, particularly as patients with BE are living longer lives due to advances in surgical techniques and medical care.

Keywords: Bladder exstrophy (BE); long term outcomes; quality of life (QoL).