Ebert, Anne-Karoline and Zwink, Nadine and Jenetzky, Ekkehart and Stein, Raimund and Boemers, Thomas M. and Lacher, Martin and Fortmann, Caroline and Obermayr, Florian and Fisch, Margit and Mortazawi, Kiarasch and Schmiedeke, Eberhard and Eisenschmidt, Volker and Schaefer, Mattias and Hirsch, Karin and Roesch, Wolfgang H. and Reutter, Heiko (2019) Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study. UROLOGY, 123. pp. 210-219. ISSN 0090-4295, 1527-9995
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OBJECTIVE To further investigate associated anomalies in exstrophy-epispadias complex (EEC) patients con- genital 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. (C) 2018 Elsevier Inc.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | BLADDER EXSTROPHY; DIAGNOSIS; VARIANTS; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Kinder- und Jugendmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 28 Apr 2020 06:12 |
| Last Modified: | 28 Apr 2020 06:12 |
| URI: | https://pred.uni-regensburg.de/id/eprint/27939 |
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