Treatment Strategies and Outcome of the Exstrophy–Epispadias Complex in Germany: Data From the German CURE-Net

Abstract

Introduction: To evaluate the impact of reconstructive strategies and post-operative management on short-and long-term surgical outcome and complications of classical bladder exstrophy (CBE) patients' comprehensive data of the multicenter German-wide Network for Congenital Uro-Rectal malformations (CURE-Net) were analyzed.Methods: Descriptive analyses were performed between 34 prospectively collected CBE patients born since 2009, median 3 months old [interquartile range (IQR), 2-4 months], and 113 cross-sectional patients, median 12 years old (IQR, 6-21 years).Results: The majority of included individuals were males (67%). Sixty-eight percent of the prospectively observed and 53% of the cross-sectional patients were reconstructed using a staged approach (p = 0.17). Although prospectively observed patients were operated on at a younger age, the post-operative management did not significantly change in the years before and after 2009. Solely, in prospectively observed patients, peridural catheters were used significantly more often (p = 0.017). Blood transfusions were significantly more frequent in males (p = 0.002). Only half of all CBE individuals underwent inguinal hernia repair. Cross-sectional patients after single-stage reconstructions showed more direct post-operative complications such as upper urinary tract dilatations (p = 0.0021) or urinary tract infections (p = 0.023), but not more frequent renal function impairment compared to patients after the staged Ebert et al.Outcome of Exstrophy Reconstrution Techniques approach (p = 0.42). Continence outcomes were not significantly different between the concepts (p = 0.51). Self-reported continence data showed that the majority of the included CBE patients was intermittent or continuous incontinent. Furthermore, subsequent consecutive augmentations and catheterizable stomata did not significantly differ between the two operative approaches. Urinary diversions were only reported after the staged concept. Conclusions:In this German multicenter study, a trend toward the staged concept was observed. While single-stage approaches tended to have initially more complications such as renal dilatation or urinary tract infections, additional surgery such as augmentations and stomata appeared to be similar after staged and single-stage reconstructions in the long term.

Myogenic Cell Transplantation in Genetic and Acquired Diseases of Skeletal Muscle

“…Seventy-five percentage of patients are lifelong dependent on catheterization or other non-physiologic urinary drainage ( Maruf et al, 2020 ). Complications include recurrent infections, urinary stones, dilatation of the upper urinary tract, urinary retention, renal impairment, metabolic complications, increased risk of cancer, revision surgery, hospitalization, as well as wide-ranging limitations in daily life and severe psychological distress ( Mouriquand et al, 2003 ; Surer et al, 2003 ; Ebert et al, 2020 ).…”

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).…”