Abstract
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.
Highlights
Today the diagnosis of bladder exstrophy (BE) is usually made by prenatal ultrasound screening or by inspection after birth
Including data of subsequent series [8, 9], there is no advice for histologically or immunohistochemically detectable premalignant changes after the interim of 6–8 weeks and in comparison to early bladder closure neither fibrosis nor more severe inflammation seems to be more frequent after that time
Until surgery the bladder template is covered with topical ointment compresses against inflammation and alteration of the mucosa [1]
Summary
Today the diagnosis of bladder exstrophy (BE) is usually made by prenatal ultrasound screening or by inspection after birth. Rösch et al characterized the histology of polyps and mucosal biopsies excised during primary delayed surgery [4] and compared their findings with previous data concerning biopsies obtained during early closure in the neonate. Including data of subsequent series [8, 9], there is no advice for histologically or immunohistochemically detectable premalignant changes after the interim of 6–8 weeks and in comparison to early bladder closure neither fibrosis nor more severe inflammation seems to be more frequent after that time. - In the neonate glomerular filtration rate (GFR) is low and doubles in the first 2 weeks and doubles again in the following 2–3 weeks This immature situation of renal function in the newborn period means a high risk for long-term kidney function. The time between birth and initial repair is useful to the parents to get psychological support if desired and to prepare themselves for the procedure and the lengthy recovery period following
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