["Epidemiology, diagnosis, treatment and follow-up of the bladder exstrophy-epispadias complex"].

The development of the new S3 guideline "Epidemiology, diagnosis, treatment and follow-up of the bladder exstrophy-epispadias complex" was funded by the German Innovation Fund of the Federal Joint Committee (G-BA). Despite the relatively low level of evidence of the identified literature, a systematic approach and consistent evaluation of the literature enabled the formulation of a large number of evidence-based recommendations across a variety of topics. In addition, a patient guideline is under development in order to disseminate the guideline recommendations and to enhance self-management and understanding among patients and their relatives.

Human primary muscle stem cells regenerate injured urethral sphincter in athymic rats.

Background: The aim of the study was to demonstrate the efficacy of human muscle stem cells (MuSCs) isolated using innovative technology in restoring internal urinary sphincter function in a preclinical animal model.

Methods: Colonies of pure human MuSCs were obtained from muscle biopsy specimens. Athymic rats were subjected to internal urethral sphincter damage by electrocauterization.

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

The prevalence of rare diseases is very important for health care research. According to the European Surveillance of Congenital Anomalies (EUROCAT) registers, the live prevalence for exstrophy and/or epispadias (grades 1-3) is reported with 1:23,255 (95% CI: 1:26,316; 1:20,000). A Europe-wide prevalence evaluation based on reports from excellence centers estimates a prevalence for exstrophies of 1:32,200 and for isolated epispadias of 1:96,800 in 2010.

[Health care reality of selected pediatric urologic surgeries in Germany from 2006 to 2019].

Background: The conservative and surgical treatment of children is a fundamental pillar of the urological specialist training program and represents one of the core competencies within urological healthcare. The loss of this expertise has been a reoccurring topic within urologic occupational policy. The aim of this study is to analyse actual case numbers and to compare the distribution and dynamics of pediatric urologic surgeries between the specialist departments of urology and pediatric surgery in Germany.

Improving Drinking and Micturition Habits in Preschool Children: An Educational Project.

Introduction: Children worldwide often do not drink enough. However, sufficient fluids are essential for physical and cognitive health. A regular and adequate supply of fluids also supports bladder maturation in the context of acquiring urinary continence.

Guideline-adherence in the treatment of symptomatic urolithiasis in children and adolescents in southwestern Germany.

Background: Approximately 1% of urolithiasis cases in Germany affect children. Interdisciplinary groups have agreed on national and international guidelines for children to recommend appropriate treatment pathways. The aim of this retrospective and preliminary study is to analyze whether adherence to current guidelines for pediatric stone disease in southwestern Germany is feasible.

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

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. 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). The majority of included individuals were males (67%).

[Bilateral reflux: contra "intravesical techniques"].

In reflux surgery, decisions on surgical procedures can be made on an individual basis today. Almost all open surgical implantation techniques provide very good, sustainable results, and parents prefer less invasive techniques. Therefore, if intravesical techniques are used to correct bilateral reflux, it should be considered and discussed with the parents that these procedures are associated with increased invasiveness in terms of longer operation times, longer catheter dwell times, a longer inpatient stay and more complications such as macrohaematuria and bladder spams compared with extravesical techniques.

Classic bladder exstrophy and adenocarcinoma of the bladder: Methylome analysis provide no evidence for underlying disease-mechanisms of this association.

The bladder exstrophy-epispadias complex (BEEC) represents the severe end of uro-rectal malformation spectrum involving aberrant embryonic morphogenesis of the cloacal membrane and the urorectal septum. The most common form of BEEC is isolated classic bladder exstrophy (CBE). Long-term complications in CBE are malignancies of the bladder with 95% of them being adenocarcinomas.

Needs Assessment in Care of Adults With Anorectal Malformations and Exstrophy-Epispadias Complex in Germany.

Medical needs of adults with anorectal malformations (ARM) and the exstrophy-epispadias complex (EEC) are not fully understood. Therefore, the aim of the study was to evaluate how affected individuals get along with the current national medical care and what their medical or social long-term requirements are. Between 11/2014-07/2016 all adult members (≥18 years, ARM = 113, EEC = 126) of the German self-help organizations SoMA e.

Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study.

Objective: To further investigate associated anomalies in exstrophy-epispadias complex (EEC) patients congenital uro-rectal malformations network (CURE-Net) database was systematically screened. In literature the EEC comprises a spectrum of anomalies, mainly occurring "isolated" without additional congenital defects. Nevertheless, previous epidemiological studies indicated a higher association with renal, anorectal, and lower neurotubular anomalies, which may originate from the same developmental morphogenetic fields.

Sexual Function and Quality of Life in Adult Male Individuals with Exstrophy-Epispadias Complex-a Survey of the German CURE-Network.

Objective: To investigate sexual function and quality of life (QoL) in adult male individuals with exstrophy-epispadias complex (EEC). Data from the German Network for Congenital Urorectal Malformations (CURE-Net) were used.

Patients And Methods: Fifty-one male participants (≥18 years) recruited by CURE-Net between 2009 and 2012 were re-contacted per mail and asked to fill out 4 questionnaires including International Index of Erectile Function (IIEF-5), Cologne Assessment of Erectile Dysfunction (KEED), the Short-Form 36 (SF-36), and one self-designed questionnaire about their medical history, current health status, and sexual experience.

[Acute Flank Pain in Children and Adolescents].

Acute flank pain in children and adolescents is a clinically demanding symptom requiring profound diagnostic work-up. Thorough history taking, careful clinical examination as well as urine and laboratory tests in addition to ultrasound are required to guide towards the diagnosis. This article presents possible underlying conditions with specific diagnostic and therapeutic considerations.

Phimosis in a 10-yr-old Boy Without Urinary Infection-How to Inform Parents: Against Circumcision.

In a 10-yr-old boy with no abnormalities or symptoms other than nonretractability of the foreskin, the foreskin should be preserved. If treatment is needed, local corticoid application should be used as first-line therapy, as it gives excellent results in up to 90% of cases, before removing this sensitive part of the body.

Cowper's syringocele: A rare differential diagnosis of infravesical obstruction in boys and young adults.

Objective And Introduction: Cowper's syringocele is a cystic dilation of the bulbourethral gland duct, initially defined by Maizels et al. in 1983. Although obstructive and non-obstructive types of this rare anomaly are described, clinical symptoms are highly unspecific.

Evaluation of sexual function in females with exstrophy-epispadias-complex: A survey of the multicenter German CURE-Net.

Objective: Standardized knowledge about genital function in adult female individuals with exstrophy-epispadias complex (EEC) is scarce. The aim of this study was to investigate sexual function using the standardized Female Sexual Function Index (FSFI), and to assess the influence of bladder and vaginal reconstruction and the presence of incontinence on FSFI results.

Method: Sixty-one females (aged ≥18 years) recruited by the German multicenter network for congenital uro-rectal malformations (CURE-Net) were asked to complete the FSFI and a self-designed semi-structured questionnaire assessing comprehensive medical data, gynecological, and psychosocial items.

CNV analysis in 169 patients with bladder exstrophy-epispadias complex.

Background: The bladder exstrophy-epispadias complex (BEEC) represents the severe end of the congenital uro-rectal malformation spectrum. Initial studies have implicated rare copy number variations (CNVs), including recurrent duplications of chromosomal region 22q11.21, in BEEC etiology.

Morphological and Functional Hip Long-Term Results after Exstrophy Repair.

 Abnormalities of the bony pelvis in exstrophy-epispadias complex (EEC) and their possible relation to hip disease are well described. However, there is a lack of information about long-term orthopedic consequences and hip function in patients with EEC. Therefore, we investigated clinical and radiological results in an EEC patient cohort after long-term follow-up.

Sexual function in adult patients with classic bladder exstrophy: A multicenter study.

Background: The bladder exstrophy-epispadias complex (BEEC) comprises a spectrum of congenital anomalies that represents the severe end of urorectal malformations, and has a profound impact on continence as well as sexual and renal functions.

Objective: The relation between severity of BEEC and its associated functional impairments, on one hand, and the resulting restrictions in quality of life and potential psychopathology determine the patients' outcome. It is important for improving further outcome to identify BEEC-related sources of distress in the long term.

Classic bladder exstrophy: Frequent 22q11.21 duplications and definition of a 414 kb phenocritical region.

Background: Classic bladder exstrophy (CBE) is the most common form of the bladder exstrophy and epispadias complex. Previously, we and others have identified four patients with a duplication of 22q11.21 among a total of 96 unrelated CBE patients.

Delayed-onset ureteral obstruction after endoscopic dextranomer/hyaluronic acid copolymer (Deflux) injection for treatment of vesicoureteral reflux in children: a case series.

We report 4 patients with upper urinary tract (UUT) obstruction requiring ureteric reimplantation at 1, 7, 28, and 63 months after dextranomer/hyaluronic acid copolymer (Dx/HA) injection for vesicoureteric reflux. Histopathologic evaluation of ureteric segments revealed extensive foreign body formation in all cases. We conclude that UUT obstruction is a rare but serious complication after Dx/HA injection that can occur even years after surgery.

Isolated bladder exstrophy associated with a de novo 0.9 Mb microduplication on chromosome 19p13.12.

Background: The exstrophy-epispadias complex (BEEC) is a urogenital birth defect of varying severity. The causes of the BEEC are likely to be heterogeneous, with individual environmental or genetic risk factors still being largely unknown. In this study, we aimed to identify de novo causative copy number variations (CNVs) that contribute to the BEEC.