Dank der heute hervorragenden Versorgung komplexer urogenitaler Fehlbildungen in der Neonatalzeit und Kindheit benötigen zunehmend mehr Patient*innen im Erwachsenenalter eine kompetente Langzeitversorgung. Idealerweise übernimmt die Urologie die Versorgung dieser Menschen, die in der Regel einen lebenslangen urologischen Nachsorgebedarf haben. Nur wenn adäquates Wissen vorhanden ist und das auch so bleibt, können diese Patient*innen mit komplexen und seltenen Krankheitsbildern, auch in Zukunft im Erwachsenenleben leitliniengerecht versorgt werden. Zudem ist die Versorgung dieser Patienten aufwendig und muss adäquat vergütet werden. Eine gute urologische Grundausbildung als Fundament sowie eine spezialisierte kinder- und jugendurologische Zusatzweiterbildung können helfen kompetente Experten auszubilden.

INTRODUCTION:As the exstrophy-epispadias complex (EEC) has not only physical, but also complex psychological consequences, further research is required to understand these in greater detail. This study aimed to test hypotheses concerning the association between shame, disclosure, friendship quality and life satisfaction among individuals with EEC, in order to identify specific areas for potential patient support. SAMPLE AND METHODS:During the pre-registered study, data were collected online and anonymously via an Instagram account and four support groups in Germany and Switzerland. This cross-sectional survey incorporated the "Chronic Illness-related Shame Scale", four subscales of the "McGill Friendship Questionnaire", and the "Short Scale to Measure General Life Satisfaction", with minor modifications. The disclosure scale was self-created and tested using exploratory factor analysis. The reliability of the scales ranged from acceptable to very good. For hypothesis testing, Pearson correlation and linear regressions were performed (α = 0.05). To examine sex differences, t-tests were conducted. RESULTS:106 adults (47 men, 58 women, 1 divers or no answer) completed the questionnaire. On average, shame was reported in the lower to mid-range of the scale, while disclosure, friendship quality, and life satisfaction were reported at moderate to high levels. In line with the hypotheses, the analyses revealed significant negative correlations between EEC-related shame and social support and intimacy within the best friendship, life satisfaction, and the propensity to disclose EEC with approach goals, thus indicating an association between lower levels of shame and more positive social factors. Additionally, in affected friendships, a positive correlation between disclosure with approach goals and positive feelings, social support, and reliable alliance was found. Contrary to expectations, men reported a marginally insignificant tendency to experience more EEC-related shame than women. Women scored higher than men in terms of friendship quality and disclosure. DISCUSSION:In EEC, the level of shame and disclosure appears to be important in friendships. Furthermore, shame seems to play an important role in life satisfaction. Men, in particular, may be vulnerable in relation to most of these constructs. The results are largely consistent with previous research. Limitations of this study are especially the predominance of support groups and the underrepresentation of men. The findings suggest the need for practical support. CONCLUSION:The study emphasized the importance of psychosocial factors in EEC. Future research should continue to focus on psychosocial aspects of EEC, with a particular emphasis on health services research.

Thanks to the excellent care of complex urogenital malformations during the neonatal period and childhood available today, an increasing number of patients require competent long-term care in adulthood. Ideally, urology takes responsibility for the transition of these individuals, who have a lifelong need for urological follow-up care. Only if adequate knowledge is available and maintained will these patients receive appropriate care throughout adult life. This care is demanding and must be reimbursed appropriately. Solid basic training in urology, as well as specialized additional training in paediatric and adolescent urology, can help to train competent experts.

INTRODUCTION:Distal hypospadias is often operated in early childhood on the assumption that adjusting penile appearance reduces disturbances in psychological development. However, data on the outcome of unoperated men is scarce, and therefore, this assumption has no evidence base. This study compares the psychological outcome of men with uncorrected distal hypospadias to age-matched men without a genital variation. METHODS:In a cross-sectional study, 39 men with uncorrected distal hypospadias (age range: 20-70 years; mean age: 49.05 years, SD: 13.34) and a control group of 39 age-matched, unaffected men (age-range: 20-70 years; mean age: 49.08 years, SD: 13.42) completed questionnaires about sexuality, urinary function, and coping with uncorrected hypospadias. In addition, they answered the Medical Outcomes Study Short Form 36-item questionnaire (SF-36) to measure their health-related quality of life (HRQoL), the International Index of Erectile Function (IIEF) to measure their erectile function (EF), and the Penile Perception Score (PPS) to measure their genital self-perception. RESULTS:Men with uncorrected hypospadias had a significantly lower PPS and were significantly more often bothered by their penile appearance than were controls. However, they reported the same HRQoL, a comparable frequency and quality of sexuality, and the same EF. They did not hide their penis in public toilets more often than controls. Furthermore, they did not feel ridiculed more often due to their penis, nor did they feel more embarrassed during sexual contact. Almost all men (n = 37; 94.8 %) were able to void in a standing position. CONCLUSION:Our study indicates that some men with uncorrected distal hypospadias adapt well to their penile condition regarding their sexuality, HRQoL and voiding function. This indicates that not all men with uncorrected hypospadias suffer a psychological burden. However, our study also showed that men with uncorrected hypospadias had a lower genital self-perception and were more often bothered by their penile appearance compared to men without hypospadias. Therefore, delaying surgery of distal hypospadias beyond early childhood, until the affected individuals can give informed consent, may be a valid option for some individuals, particularly those with mild forms of distal hypospadias. The decision for or against surgery in early childhood must be considered carefully, and families must be supported in making their individual choices. This study has important limitations: The small group of unoperated men who volunteered to participate may be a selection of men who cope particularly well with their condition and therefore results may not be representative.

Die Erstellung der neuen S3-Leitlinie „Epidemiologie, Diagnostik, Therapie und Nachsorge des Blasenekstrophie-Epispadie Komplexes“ wurde durch 2021 beantragte Forschungsgelder vom Innovationsfonds des Gemeinsamen Bundesausschusses (G-BA) ermöglicht. Wenngleich das Evidenzlevel der identifizierten Literatur vergleichsweise niedrig ist, konnten durch ein systematisches Vorgehen und eine konsequente Bewertung der Literatur eine Vielzahl von evidenzbasierten Empfehlungen zu vielen Themen formuliert werden. Zudem wird eine Patient*innenleitlinie erstellt, um die Leitlinienempfehlungen in die Breite zu bringen und das Selbstmanagement und Verständnis der Betroffenen und Zugehörigen zu fördern. Um die für Patient*innen wichtigen Themen in der Patient*innenleitlinie adäquat abzubilden, wurde im Vorfeld eine Bedarfsanalyse durchgeführt. Nach Abschluss der Arbeiten an der deutschen Leitlinie ist mit dem e‑UROGEN-Netzwerk eine Kooperation für eine englische Übersetzung geplant.

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. A needs analysis had been carried out in order to adequately assess the topics that are most important for patients and relatives. Upon completion of the German guideline, an English translation in cooperation with the e-UROGEN network is planned.

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. Five days after injury, 2 × 10 5 muscle stem cells or medium as control were injected into the area of sphincter damage ( n = 5 in each group). Peak bladder pressure and rise in pressure were chosen as outcome measures. To repeatedly obtain the necessary pressure values, telemetry sensors had been implanted into the rat bladders 10 days prior to injury. Results There was a highly significant improvement in the ability to build up peak pressure as well as a pressure rise in animals that had received muscle stem cells as compared to control ( p = 0.007) 3 weeks after the cells had been injected. Only minimal histologic evidence of scarring was observed in treated rats. Conclusion Primary human muscle stem cells obtained using innovative technology functionally restore internal urethral sphincter function after injury. Translation into use in clinical settings is foreseeable.

Die Persistenz des Sinus urogenitalis mit unvollständiger Trennung von Urethra und Scheide tritt bei ätiologisch komplett unterschiedlichen Anomalien auf. Entsteht der Sinus durch Androgeneinfluss wie bei Varianten der Geschlechtsentwicklung, so entwickelt sich die weibliche Urethra normal und erreicht die Klitoris, ohne dass die Sphinkterfunktion beeinträchtigt ist. Kommt der persistierende Sinus im Rahmen einer nicht erfolgten Trennung von Rektum, Scheide und Harntrakt in der frühen Embryonalperiode zustande, so bestimmen neben der Kloakenpersistenz vor allem die neurogenen Komorbiditäten Verlauf und Outcome. Die isolierte Form der Sinuspersistenz ohne Zeichen einer Maskulinisierung und ohne anorektale Anomalie resultierend aus einer fehlenden Reabsorption des distalen Sinus ist sehr selten. Fehlbildungen des weiblichen Genitales sind meist durch Inspektion im Kindesalter erkennbar, bleiben aber in ihrer funktionellen Bedeutung klinisch oft inapparent. Mit Ausbleiben der Menarche bzw. im Rahmen der Abklärung eines unerfüllten Kinderwunsches wird die Diagnose gestellt. Selten kommt es durch Ausbildung einer Hämatometra zu einer akuten Abdominalsymptomatik. Ein vergleichsweise häufiges gynäkologisches Problem in der Kindheit stellen entzündliche Veränderungen wie die Vulvovaginitis dar und müssen bei dysurischen Beschwerden zur Harnwegsinfektion differenzialdiagnostisch abgegrenzt werden. Vulvovaginitiden im Kindesalter sind meist unspezifisch und können mit einfachen Hygienemaßnahmen sowie der Therapie evtl. zugrundeliegender Darm- und Blasenentleerungsstörung erfolgreich behandelt werden

Sowohl die der Hypospadie als auch die Epispadie sind Hemmungsfehlbildungen des Penis, die unterschiedlich stark ausgeprägt sein können. Die Hypospadie, bei der die Spaltung ventral, also an der Unterseite des Penis vorliegt, tritt recht häufig auf. Bei der Epispadie liegt die Spaltung dorsal; sie gehört zu den sehr seltenen Fehlbildungen. Dieser Beitrag soll einen Überblick über die große Bandbreite der Erscheinungsformen und die Behandlungsoptionen geben.

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. We investigated whether training preschool children and their caretakers improves drinking and micturition habits. Methods: This field study in a pre-post design was conducted in 6 kindergartens in the district of Garmisch-Partenkirchen from October 2018 to February 2019. An intervention group (IG) received a 3-day training on drinking and micturition habits and was compared to a control group (CG) without any training. Caretakers (IG + CG) were instructed about drinking and voiding management, too. Behavioral changes were identified by questionnaires. To analyze the long-term effect, group interviews were performed with the IG 3 months after training. The training was evaluated on different levels. Results: After training, the estimated total daily fluid intake in the IG (1,160 mL) significantly exceeded that of CG (830 mL) (p = 0.015). In the IG, fluid intake until 12:00 a.m. increased (p = 0.001), children took more time for voiding (p = 0.029), and urgency decreased (p = 0.008). Children (IG + CG) used leg support to enable pelvic floor relaxation more often both at home (p = 0.026) and in kindergarten (p = 0.047). Nocturnal enuresis was reduced by approximately 46% in the IG (p = 0.485). Group interviews in the IG showed a considerable learning effect. Conclusion: The present study could demonstrate an increased intake of fluids and significant changes in micturition behavior in the IG. So far, this is the first educational project in Germany addressing drinking and voiding management. Our results suggest that a training of preschool children and their caretakers is feasible and effective. Further nationwide research will be needed to confirm our results and assess the need for prevention in these areas.

Introduction: 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. However, the frequency of exstrophy [International Statistical Classification of Diseases and Related Health Problems revision 10 (ICD-10): Q64.1] and epispadias (ICD-10: Q64.0) treated in different age groups in Germany remains unclear.Material and Method: Public health insurance data from 71 million people (approximately 87% of the population) were provided by the German Institute for Medical Documentation and Information (DIMDI) in accordance to the German Social Insurance Code for this research purpose. DIMDI analyzed the data source for the ICD diagnoses exstrophy and epispadias between 2009 and 2011. As provided data were robust over the years, averaged data are mentioned. Detailed subgroup analysis of small numbers was forbidden due to privacy protection.Results: Annually, 126 persons of all ages with epispadias and 244 with exstrophy are treated as inpatients. In the observed population, 34 infants (<1 year of age) with epispadias and 19 with exstrophy (58% male) are treated as outpatients each year. This corresponds to an estimated live prevalence of 1:11,000 (95% CI: 1:14,700; 1:8,400) for EEC (exstrophy–epispadias complex), more specifically a prevalence of 1:17,142 for epispadias and of 1:30,675 for exstrophy. The male-to-female ratio for exstrophy is 1.4:1 for infants and 1.6:1 for all minors. In children and adolescents, 349 epispadias and 393 exstrophies (up to the age of 17) are treated annually, whereas adults with exstrophy and even more with epispadias make comparatively less use of medical care.Conclusion: With the help of DIMDI data, the live prevalence of bladder exstrophy and epispadias in Germany could be estimated. The prevalence of epispadias was higher than in previous reports, in which milder epispadias phenotypes (grade 1 or 2) may not have been included. These analyses might enlighten knowledge about nationwide incidence and treatment numbers of rare diseases such as the EEC.

Zusammenfassung Hintergrund Die konservative und chirurgische Behandlung von Kindern ist in der urologischen Facharztweiterbildung fest verankert und stellt eine Kernkompetenz der urologischen Versorgung dar. Berufspolitisch wird seit vielen Jahren ein zunehmender Verlust dieses Schwerpunkts befürchtet. Ziel dieser Studie ist es, reale Fallzahlen und eine mögliche Dynamik in der Verteilung kinderurologischer Eingriffe auf die Fachabteilungen für Urologie und Kinderchirurgie in Deutschland zu prüfen. Material und Methoden Als Index-Eingriffe definierten wir Operationen des Hodenhochstands, der Hypospadie und des vesikoureteralen Refluxes (VUR). Mithilfe der Software reimbursement.INFO (RI Innovation GmbH, Hürth) werteten wir die öffentlich zugänglichen Qualitätsberichtsdaten der deutschen Krankenhäuser von 2006 bis 2019 aus und wiesen sie den entsprechenden Fachrichtungen zu. Ergebnisse Die Orchidopexie erfolgt häufiger in der Urologie, wohingegen die Hypospadiekorrektur und die operative Therapie des VUR häufiger in der Kinderchirurgie durchgeführt werden. Anteilig zeigte sich für die Orchidopexie und die operative Refluxtherapie keine relevante Verschiebung zwischen urologischen und kinderchirurgischen Kliniken im Untersuchungszeitraum. Bei den Hypospadiekorrekturen nimmt der Anteil der Operationen in kinderchirurgischen Einheiten zu (p < 0,0001). In der Kinderchirurgie erfolgen 84–93 % der analysierten Eingriffe in High-volume-Abteilungen während dieser Anteil in der Urologie bei 56–73 % liegt. Insbesondere die operative Refluxtherapie in der Urologie erfolgt zu einem hohen Anteil als Gelegenheitseingriff (30 % „very low volume“). Schlussfolgerung Die Qualitätsberichtsdaten ermöglichen die Erfassung der Fallzahlen und die Analyse der Verteilung zwischen Urologie und Kinderchirurgie in Deutschland. Dabei ist für die Hypospadiekorrektur eine relevante Verschiebung in Richtung der Kinderchirurgie zu beobachten. Die Ursachen und möglichen berufspolitischen Konsequenzen dieser ersten Erhebung sind komplex und bedürfen weiterer Analysen.

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. Materials and methods We defined the surgical treatments of maldecensus testis, hypospadias, and vesicoureteral reflux (VUR) as index interventions. Using the tool reimbursement.INFO (RI Innovation GmbH, Hurth, Germany) we analysed publicly available quality report data of German hospitals between 2006 and 2019. Results While orchidopexy was more commonly performed in the field of urology, the correction of hypospadias and the surgical treatment of VUR showed higher case numbers in the field of pediatric surgery. Proportionally, there was no relevant shift between urologic and pediatric surgical clinics for orchidopexy and surgical VUR therapy during the study period. For hypospadias corrections, the proportion of surgeries performed in pediatric surgical units is increasing (p < 0.0001). In pediatric surgery 84-93% of the analyzed procedures are performed in high-volume units, while this proportion is 56-73% in urology. In particular, a high proportion of VUR therapy in urology is performed as an occasional procedure (30% very low volume). Conclusions The quality report data enable the compilation of case numbers and the analysis of the distribution between urology and pediatric surgery in Germany. Merely the correction of hypospadias has shown a relevant shift towards pediatric surgery. The causes and possible consequences for professional policy of this preliminary investigation are complex and require further analysis.

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 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.