Presence of a Hernia Sac Does Not Impact Lung Perfusion in CDH

Abstract

Purpose: There are variable long-term pulmonary outcomes of children with congenital diaphragmatic hernia (CDH). Diagnostic modalities evaluating pulmonary function can be limited by patient compliance. Pulmonary perfusion scintigraphy is a diagnostic tool to evaluate lung development in younger children. Presence of a hernia sac is known to correlate with less severe disease but little has been reported on any correlation with lung size and function. The purpose of this study was to evaluate the relationship between presence of a hernia sac, pulmonary perfusion and CDH defect size. Methods: Records of all children treated for CDH at our institution from January 2009 to December 2018 were reviewed. A subset of these children underwent lung perfusion scintigraphy. We hypothesized that the presence of a hernia sac would affect future perfusion studies. We assessed this with two linear regression models. Results: One hundred thirty-three children were treated for CDH over 10 years. Twenty-nine children (22%) underwent pulmonary scintigraphy, 9 of whom (31%) had a hernia sac. There was no correlation between hernia sac presence and improved perfusion (R2 = 0.005, p = 0.7). There was a correlation between area of the defect (cm2), and perfusion difference between the two lungs (R2 = 0.56, p < 0.05). When adjusting for presence of the hernia sac, the fit of the model was unchanged (R2 = 0.57, p < 0.05). Conclusions: As expected, there is a correlation between CDH defect size and lung perfusion. However, presence of a hernia sac did not impact lung function as determined by perfusion scintigraphy.