Congenital diaphragmatic hernia and associated omphalocele: a study from the CDHSG registry.

BACKGROUND

Congenital Diaphragmatic Hernia (CDH) associated with Omphalocele is a rare condition, and only a few case reports are available in the literature. Both conditions are associated with some degree of pulmonary hypoplasia. We hypothesize that the combination of CDH with Omphalocele might be associated with poorer outcomes.

AIM: The aim of this study was to describe the incidence of this association and postnatal outcomes from the largest database available for CDH.

METHODS

Data from the multicenter, multinational database on infants with CDH (CDHSG Registry) born from 2007 to 2018 was analyzed.

RESULTS

A total of 5730 entries were made into the registry during the study period. The incidence of Omphalocele associated with CDH was 0.63% (36 out of 5730). When comparing posterolateral Bochdalek hernias with Omphalocele (CDH + O) to CDH without Omphalocele (CDH-), CDH + O were born at significantly younger gestational ages. They were sicker directly after birth with significantly lower APGARs at all time points, but received ECMO significantly less often. The distribution of left vs right side or the defect size did not differ but CDH + O required patch in a significantly larger extent. CDH + O had surgical repair significantly later and had significantly higher rates of non-repairs and significantly lower survival rates. The morbidity was significantly higher with longer hospital stays and higher requirements for O2 at 30 DOL.

DISCUSSION: CDH associated with Omphalocele is a rare but more severe condition with higher mortality and morbidity rates. Newborns with these combined conditions can be difficult to stabilize or might pose complicated management problems due to pulmonary hypertension and/or pulmonary hypoplasia.

TYPE OF STUDY: Prognosis Study.

LEVEL OF EVIDENCE: Level I.