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ASSOCIATION BETWEEN EXSTROPHY-EPISPADIAS COMPLEX AND CONGENITAL ANOMALIES: A GERMAN MULTICENTER STUDY.

Urology 2018 August 2
OBJECTIVES: To further investigate associated anomalies in EEC patients CURE-Net database was systematically screened. In literature the exstrophy-epispadias complex (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.

PATIENTS AND METHODS: 73 prospectively (born since 2009) and 162 cross-sectional recruited EEC patients (born 1948-2008) were analysed. 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<0.0001). Anomalies such as inguinal hernias, skeleton and joint anomalies were equally present in both groups (p=0.65 and p=0.67). Heart defects were seen more often in newborns (6%) than in the cross-sectional group (1%; p=0.033) 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=0.16).

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.

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