Hydrops and Congenital Diaphragmatic Hernia: Reported incidence and postnatal outcomes. Analysis of the CDHSG Registry

Abstract

Abstract Obejctive: Congenital Diaphragmatic Hernia (CDH) associated with hydrops is rare. The aim of this study was to describe the incidence of this combination of anomalies and the postnatal outcomes from a large database for CDH. Study design: Data from the multicenter, multinational database on infants with prenatally diagnosed CDH (CDHSG Registry) born from 2015 to 2021 were analyzed. Results: A total of 3985 patients were entered in the registry during the study period, 3156 were prenatally diagnosed and 88 were reported to have associated fluid in at least 1 compartment, representing 2.8 % of all prenatally diagnosed CDH cases in the registry. The overall survival to discharge for CDH patients with hydrops was 43%. The hydropic CDH group had lower birth weight and gestational age at birth, and increased incidence of right sided CDH (55%) and rate of non-repair (45%). However, the survival rate for hydropic infants with CDH undergoing surgical repair was 80%. Other associated anomalies were more common in hydropic CDH (50% vs 37%, p=0.001). Conclusion: Hydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right sided CDH. Survival rates are low, with higher rates of non-repair. However, early decision-making regarding goals of care and an aggressive surgical approach may result in survival rates comparable to non-hydropic cases.