Pediatr Pulmonol
. 2023 Jun 28.
doi: 10.1002/ppul.26565. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/37378468/
Pathological findings in congenital diaphragmatic hernia on necropsy studies: A single-center case series
Chu-Yi Meng 1, Ji-Zhen Zou 2, Ying Wang 1, Yan-Dong Wei 1, Jing-Na Li 1, Chao Liu 1, Zhong Feng 3, Ling-Ling Cai 2, Ping Xiao 2, Li-Shang Ma 1 3
Affiliations expand
- PMID: 37378468
- DOI: 10.1002/ppul.26565
Abstract
Introduction: Congenital 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.
Methods: We retrospectively reviewed the postmortem findings and corresponding clinical characteristics in eight CDH cases from 2017 to July 2022.
Results: The 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.
Conclusions: Patients 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.
Keywords: congenital diaphragmatic hernia; postmortem examination; pulmonary hypertension; pulmonary hypoplasia.
© 2023 Wiley Periodicals LLC.
