Association of Pulmonary Hypoplasia and Pulmonary Hypertension with Giant Omphalocele: A Systematic Review

Abstract

Objective: To evaluate the prevalence of PH/PPHN and mortality in newborn infants with GO. Study Design: A systematic review according to PRISMA guidelines using MEDLINE/PubMed, Google Scholar, and Scopus platforms. The search strategy combined indexed and in-process citations up to May 22nd, 2023. The terms used were related to GO, PH, and PPHN. Results: Out of 651 articles, 23 met inclusion criteria, comprising 14 observational studies and 9 case reports. Observational studies revealed a mean PH/PPHN prevalence of 33.5% with a mortality rate averaging 25.4%. Studies reporting reduced pulmonary function highlight the critical impact of this condition on overall morbidity and mortality rates. Case reports highlighted PH/PPHN in 77.8% of neonates, among whom 55.5% succumbed, with 90.0% of fatalities attributed to PH/PPHN and 40.0% involving preterm births. The association of congenital abnormalities and preterm birth contributed significantly to the increase in the mortality rate. The use of ECMO did not improve the outcome. Conclusions: Prevalences of PH/PPHN and mortality are high in neonates with GO. Other factors also contribute to the mortality of these newborns, including associated congenital abnormalities and preterm birth. Early detection of PPHN and proper multidisciplinary treatment are crucial for improving outcomes in neonates with GO.