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Although enormous effort has been made to further improve the operative techniques worldwide, the management of bladder exstrophy (BE) remains one of the most significant challenges in pediatric urology. Today it is universally agreed that successful and gentle initial bladder closure is decisive for favorable long-term outcome with regard to bladder capacity, renal function and continence. Due to a number of reasons, including a lack of comparable multicenter studies, a range of concepts is currently used to achieve successful primary closure. We review the literature of the last 15 years on the current concepts of bladder exstrophy repair with regard to the time of primary closure (initial vs. delayed closure), the concepts of primary closure (single-stage vs. staged approach; without osteotomy vs. osteotomy) and their outcomes. There is a worldwide lack of multicenter outcome studies with adequate patient numbers and precisely defined outcome parameters, based on the use of validated instruments. The modern staged repair (MRSE) in different variations, the complete primary reconstruction of exstrophy (CPRE), and the radical soft-tissue mobilization (RSTM) had been the most extensively studied and reported procedures. These major concepts are obligatory stable now for more than 20 years. Nevertheless, there are still a lot of open-ended questions e.g., on the potential for development of the bladder template, on continence, on long-term orthopedic outcome, on sexuality and fertility and on quality of life. Management of BE remains difficult and controversial. Further, clinical research should focus on multi-institutional collaborative trials to determine the optimal approach.

Recent Trends in the Management of Bladder Exstrophy: The Gordian Knot Has Not Yet Been Cut