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

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.

Development of Late Continence in Bladder Exstrophy and Epispadias Patients

“…In the previous studies, there is a wide variation in continence results in CBE and epispadias patients depending on the definition of continence, age at evaluation and surgical treatment protocol 2 . Some studies suggest that more than 70% of the patients have achieved daytime urinary continence with normal voiding at a very young age 9,10 .…”

“…properly remain major problems for patients operated for classic bladder exstrophy (CBE) or epispadias. Reported urinary continence rates have been between 12% and 83% in the patients with volitional voiding 1,2 . In many patients, the continence is achieved only with the aid of bladder augmentation or clean intermittent catheterization (CIC) program [3][4][5] .…”

Bladder augmentation in exstrophy vesicae: Long‐term results of a single experienced center

“…Although significant effort has been made on surgical treatment, BE management remains a matter of debate in pediatric urology practice (Anand & Lotfollahzadeh, 2021a; Promm & Roesch, 2019). Various surgical procedures described so far can be divided into two main categories: reconstructive procedures and diversion procedures.…”

“…Nevertheless, many patients with BE still require BA with nine other conduits, as persistent urinary incontinence is a common complication, and reconstructive procedures might fail to acceptable dryness in these patients (Purves, 2011). Therefore, it has been recognized worldwide that the BA/CIC/Mitrofanoff regimen is decisive in achieving long‐term positive results regarding bladder capacity, kidney function, and continence rates, as well as thriving and gentle initial bladder closure (Dickson, 2014; Mathews & Gearhart, 2005; Promm & Roesch, 2019). In this study, we sought to demonstrate the long‐term results of the BA/CIC/Mitrofanoff regimen in BE through the experience of a single pediatric urology center.…”

A Prevalence Estimation of Exstrophy and Epispadias in Germany From Public Health Insurance Data

“…Today, the EEC is understood as a spectrum from an isolated genito-urethral defect affecting males and females (epispadias), to classical bladder exstrophy with an open bladder and pelvis in addition to the genital defect culminating in a multiorgan anomaly-the cloacal exstrophy. As it is shown in various recent long-term outcome studies, comparatively little substantial improvements in functional outcomes were achieved (1,2). Although a rare disease, EEC treatment in Germany is decentralized with possible profound long-term sequelae such as unfavorable outcome related to small hospital treatment numbers (3,4).…”