Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study
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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 H; Reutter, Heiko.
in: UROLOGY, Jahrgang 123, 01.2019, S. 210-219.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study
AU - Ebert, Anne-Karoline
AU - Zwink, Nadine
AU - Jenetzky, Ekkehart
AU - Stein, Raimund
AU - Boemers, Thomas M
AU - Lacher, Martin
AU - Fortmann, Caroline
AU - Obermayr, Florian
AU - Fisch, Margit
AU - Mortazawi, Kiarasch
AU - Schmiedeke, Eberhard
AU - Eisenschmidt, Volker
AU - Schäfer, Mattias
AU - Hirsch, Karin
AU - Rösch, Wolfgang H
AU - Reutter, Heiko
N1 - Copyright © 2018 Elsevier Ltd. All rights reserved.
PY - 2019/1
Y1 - 2019/1
N2 - 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.
AB - 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.
KW - Journal Article
U2 - 10.1016/j.urology.2018.05.039
DO - 10.1016/j.urology.2018.05.039
M3 - SCORING: Journal article
C2 - 30076940
VL - 123
SP - 210
EP - 219
JO - UROLOGY
JF - UROLOGY
SN - 0090-4295
ER -