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

Ebert AK, Zwink N, Jenetzky E, Stein R, Boemers TM, Lacher M, Fortmann C, Obermayr F, Fisch M, Mortazawi K, Schmiedeke E, Eisenschmidt V, Schäfer M, Hirsch K, Rösch WH, Reutter H (2019)

Publication Type: Journal article

Publication year: 2019

Journal

Urology Elsevier

Book Volume: 123

Pages Range: 210-220

DOI: 10.1016/j.urology.2018.05.039

Abstract

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. Materials and Methods: Seventy-three prospectively (born since 2009) and 162 cross-sectional recruited EEC patients (born 1948-2008) were analyzed. Associated anomalies were derived from patient's medical data as well as from a physical examination during a physician's interview, classified according to the international statistical classification of diseases and related health problems and grouped with the London Dysmorphology Database. Descriptive statistical analyses were performed. Results: Majority of participants were male (68%) and expressed the classical bladder exstrophy phenotype (71%). Exstrophy variants occurred significantly more often in newborns (21%, P <.0001). Anomalies such as inguinal hernias, skeleton, and joint anomalies were equally present in both groups (P =.65 and P =.67). Heart defects were seen more often in newborns (6%) than in the cross-sectional group (1%; P =.033) and the general German population (1%). In total, 59% of the prospective and 48% of the cross-sectional patients had associated anomalies outside the spectrum (P =.16). Conclusion: Phenomenological multicenter data confirmed the dimension of associated anomalies inside and outside the EEC spectrum. The detected anomalies are either important in preparing for the primary reconstruction or later in long-term follow-up. Associated anomalies of EEC should be spotlighted during routine check-up in all EEC patients.

Involved external institutions

Universitätsklinikum Ulm DE Germany (DE) Medizinische Hochschule Hannover (MHH) / Hannover Medical School DE Germany (DE) Universität Mannheim DE Germany (DE) Johannes Gutenberg-Universität Mainz (JGU) DE Germany (DE) Universitätsklinikum Tübingen DE Germany (DE) Universitätsklinikum Hamburg-Eppendorf (UKE) DE Germany (DE) Universitätsklinikum Köln DE Germany (DE) Universität Leipzig DE Germany (DE) Universitätsklinikum Regensburg DE Germany (DE) Städtisches Klinikum Karlsruhe DE Germany (DE) Klinikverbund Bremen (Gesundheit Nord) DE Germany (DE) Universitätsklinikum Bonn DE Germany (DE)

How to cite

APA:

Ebert, A.K., Zwink, N., Jenetzky, E., Stein, R., Boemers, T.M., Lacher, M.,... Reutter, H. (2019). Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study. Urology, 123, 210-220. https://dx.doi.org/10.1016/j.urology.2018.05.039

MLA:

Ebert, Anne Karoline, et al. "Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study." Urology 123 (2019): 210-220.

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