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

Ebert, Anne‐Karoline; Zwink, Nadine; Jenetzky, Ekkehart; Stein, Raimund; Boemers, Thomas M.; Lacher, Martin; Fortmann, Caroline; Obermayr, Florian; Fisch, Margit; Mortazawi, Kiarasch; Schmiedeke, Eberhard; Eisenschmidt, Volker; Schäfer, Mattias; Hirsch, Karin; Rösch, Wolfgang; Reutter, Heiko

doi:10.1016/j.urology.2018.05.039

Cited by 8 publications

(8 citation statements)

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“…2 A German multicenter study by Ebert et al investigated associated anomalies in 73 prospectively (born since 2009) and 162 cross-sectionally (born between 1948 and 2008) evaluated patients with BEEC. 5 Most of these patients had BE, 46 (63%) in the first group and 121 (75%) in the second group. The study showed an association between CHD and BE because CHD was diagnosed in 6.5% of the 46 patients in the prospective group and in 0.8% of the cross-sectional group.…”

Section: Discussionmentioning

confidence: 94%

Congenital Heart Defects in Patients with Classic Bladder Exstrophy: A Hitherto Neglected Association?

Promm¹,

Gerling

Schepp

et al. 2021

Eur J Pediatr Surg

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Introduction Classic bladder exstrophy (BE) is regarded as an isolated malformation without any further anomalies, but some studies have indicated a higher incidence of cardiac anomalies. This cross-sectional study is planned to evaluate the prevalence of congenital heart defects (CHDs) and the clinical relevance for patients with BE admitted for primary closure. Materials and Methods Patients were prospectively recruited between March 2012 and January 2019. Patients' profiles including demographic data, results of transthoracic echocardiography (TTE), as well as essential peri- and postoperative data were assessed. Results Thirty-nine (25 boys and 14 girls) patients with BE (median age 61 days) underwent delayed primary bladder closure. Thirty-seven (24 boys and 13 girls) patients had received TTE 1 day before surgery. CHD was detected in 7 (18.9%) out of the 39 patients, but no clinical differences between patients with and without CHD were observed peri- or postoperatively. Discussion and Conclusion This prospective systematic evaluation shows an even higher rate of CHD in patients with BE than assumed previously. Although peri- and postoperative outcome did not differ between patients with and without CHD, we consider TTE an important additional method for ensuring a safe peri- and postoperative courses and a short- and long-term care for patients with CHD.

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Section: Discussionmentioning

confidence: 94%

Congenital Heart Defects in Patients with Classic Bladder Exstrophy: A Hitherto Neglected Association?

Promm¹,

Gerling

Schepp

et al. 2021

Eur J Pediatr Surg

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“…In the independent patient registry CURE-Net only a minority of participants had epispadias (14% in both newborns and in the older age cases) compared to exstrophy ( 8 ). The majority had the most severe epispadias grade 3 (80% of the newborn and 78% of older age epispadias cases), whereas only a minority of patients had epispadias grade 1 (10% of newborn/13% of older epispadias patients) and epispadias grade 2 (10% of newborn/9% of older age epispadias patients) ( 8 ). This report is at odds with the dominant observation that the less severe phenotypes occur most frequently.…”

Section: Discussionmentioning

confidence: 99%

“…Epidemiological studies reported a male-to-female ratio of 2.2:1 for epispadias and a 1.8:1 male predominance for classical bladder exstrophy ( 13 ) as well as of 1.4:1 for epispadias and 2.8:1 for exstrophy ( 14 ). In CURE-Net, the majority of participants were male (68%), with a male-to-female ratio of 2.2:1 and 2.1:1 in the newborn and older age patient groups, respectively, ( 8 ). Taken together, a male predominance in EEC is consistently reported, even in the DIMDI data over several age groups.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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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.

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“…In case of delayed management, only a few diagnostic measures are required preoperatively. Besides the ultrasound of the upper urinary tracts and the hips an echocardiography is recommended, recent studies indicate that there is an increased risk of associated congenital heart failures in BE patients (20). Further diagnostics like MRI or computer tomography are not necessary.…”

Section: Preoperative Managementmentioning

confidence: 99%

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

Promm¹,

Roêsch²

2019

Front. Pediatr.

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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.

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

Cited by 8 publications

References 13 publications

Congenital Heart Defects in Patients with Classic Bladder Exstrophy: A Hitherto Neglected Association?

Congenital Heart Defects in Patients with Classic Bladder Exstrophy: A Hitherto Neglected Association?

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

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

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