Pathological findings in congenital diaphragmatic hernia on necropsy studies: A single‐center case series

Abstract

IntroductionCongenital diaphragmatic hernia (CDH) is associated with high mortality rates and significant pulmonary morbidities. The objective of this study was to delineate the histopathological features observed in necropsies of CDH patients and correlate these with their clinical manifestations.MethodsWe retrospectively reviewed the postmortem findings and corresponding clinical characteristics in eight CDH cases from 2017 to July 2022.ResultsThe median survival time was 46 (8–624) hours. Autopsy reports showed that diffuse alveolar damage (congestion and hemorrhage) and hyaline membrane formation were the primary pathological lung changes observed. Notably, despite significant reduction in lung volume, the lung development appeared normal in 50% of the cases, while lobulated deformities were present in three (37.5%) cases. All patients displayed a large patent ductus arteriosus (PDA) and a patent foramen ovale, resulting in increased right ventricle (RV) volume, and myocardial fibers appeared slightly congested and swollen. The pulmonary vessels indicated thickening of the arterial media and adventitia. Lung hypoplasia and diffuse lung damage resulted in impaired gas exchange, while PDA and pulmonary hypertension led to RV failure, subsequent organ dysfunction and ultimately death.ConclusionsPatients with CDH typically succumb to cardiopulmonary failure, a condition driven by a complex interplay of pathophysiological factors. This complexity accounts for the unpredictable response to currently available vasodilators and ventilation therapies.

Diagnosis, management and long term cardiovascular outcomes of phenotypic profiles in pulmonary hypertension associated with congenital diaphragmatic hernia

“…The anatomical abnormality of the diaphragm and subsequent herniation of abdominal contents during critical stages of lung development produces a mass effect that affects the growth of lung parenchyma, bronchial/bronchiolar branching, and the development of the pulmonary vasculature ( 3 ). On postmortem examination, ipsilateral lung has been found to be grossly hypoplastic ( 9 ). There is reduced bronchial and bronchiolar arborization, fewer alveoli, acinar hypoplasia, thick alveolar walls and increased interstitial tissue resulting in a reduced gas-exchange area ( 10 ).…”

“…There is reduced bronchial and bronchiolar arborization, fewer alveoli, acinar hypoplasia, thick alveolar walls and increased interstitial tissue resulting in a reduced gas-exchange area ( 10 ). The abnormalities of pulmonary vasculature in CDH have been well described and include both structural and functional changes ( 3 , 9 , 10 ). Pulmonary arteries are decreased per unit lung volume.…”

“…Pulmonary arteries are decreased per unit lung volume. There is reduced pulmonary vascular branching accompanied by thickening of muscular media and adventitia (9)(10)(11). All these changes lead to a decreased anatomical vascular area for gas exchange.…”