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

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.

journal_name

Urology

journal_title

Urology

authors

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

doi

10.1016/j.urology.2018.05.039

subject

Has Abstract

pub_date

2019-01-01 00:00:00

pages

210-220

eissn

0090-4295

issn

1527-9995

pii

S0090-4295(18)30595-8

journal_volume

123

pub_type

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