Congenital diaphragmatic hernia: 25 years of shared knowledge; what about survival?

Lakshminrusimha Satyan, Vali Payam
2019 Jornal de Pediatria  
Congenital diaphragmatic hernia (CDH) remains one of the most dreaded congenital anomalies in the neonatal intensive care units (NICUs). Herniation of the abdominal organs through a malformed and defective diaphragm during fetal development can lead to nearlethal pulmonary hypoplasia and cardiac deformation. Severe persistent pulmonary hypertension of the newborn (PPHN), hypoxic respiratory failure, and cardiac dysfunction often complicate the medical course of newborns with CDH. Recognizing
more » ... interdependence of the pulmonary and cardiovascular systems, especially in the context of abnormal physiology, is of paramount importance when providing medical management to these patients. The incidence of CDH is estimated at approximately one case per 3,000 live births. A reliable estimation, however, is complicated by termination of pregnancy, inaccurate data collection, and unreported cases of newborns who die of the disease without a known prenatal diagnosis (referred to as "hidden mortality"). 1 The condition is uncommon enough so that the majority of regional NICUs manage less than a dozen cases of CDH annually, which also explains the ongoing challenges in conducting prospective randomized studies. In 1995, the CDH Study Group (CDHSG) registry was established with the purpose to unite tertiary referral centers across four continents to compile data on live born infants with CDH, and to assess therapies and outcomes. 2 Short of randomized controlled trials, registries such as the CDHSG and observational studies such as the study by Carmo et al. 3 in this issue of Jornal de Pediatria provide an important source of knowledge. There were 129 newborns with a prenatal diagnosis of CDH delivered at a large NICU in Rio de Janeiro, Brazil during the years 2005-2015. The reported newborn characteristics (i.e., gestational age at birth, sex, side of the CDH, liver herniation, associated incidence of congenital anomalies, etc.) are similar to other published reports on CDH. 3] [4] [5] [6] This large discrepancy in mortality can partly be explained by the lack of ECMO. Given that 30.3% of CDH patients reported to the registry between 2007 and 2013 were placed on ECMO 4 and nearly 51% of This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
doi:10.1016/j.jped.2019.10.002 pmid:31629706 pmcid:PMC7162701 fatcat:kg7lobs3bbantfhd3ywqy3fuby