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Pathological Findings in Congenital Diaphragmatic Hernia on necropsy studies: A Single-Center Case Series
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  • Chu-Yi Meng,
  • Ji-Zhen Zou,
  • Ying Wang,
  • Yan-Dong Wei,
  • Jing-Na Li,
  • Chao Liu,
  • Zhong Feng,
  • Ling-Ling Cai,
  • Ping Xiao,
  • Li-Shang Ma
Chu-Yi Meng
Capital Institute of Pediatrics

Corresponding Author:[email protected]

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Ji-Zhen Zou
Capital Institute of Pediatrics
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Ying Wang
Capital Institute of Pediatrics
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Yan-Dong Wei
Capital Institute of Pediatrics
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Jing-Na Li
Capital Institute of Pediatrics
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Chao Liu
Capital Institute of Pediatrics
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Zhong Feng
Capital Institute of Pediatrics
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Ling-Ling Cai
Capital Institute of Pediatrics
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Ping Xiao
Capital Institute of Pediatrics
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Li-Shang Ma
Capital Institute of Pediatrics
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Abstract

Introduction: Congenital diaphragmatic hernia (CDH) is known with high mortality rates and significant pulmonary morbidities. The objective of this study was to describe the histopathological findings of necropsy and clinical manifestations in CDH patients to find the clinicopathological correlations. Methods: We reviewed the postmortem findings with associated clinical characteristics retrospectively in 8 CDH cases from 2017 to2022 July. Results: Of the eight cases, one was bilateral congenital eventration diagnosed by autopsy. Severe pulmonary hypertension with a right to left shunting of large patent ductus arteriosus (PDA) obtained from echocardiogram were most common. And the average time of survive was 46 (8-624) h. According to the autopsy reports, the major pathological lung changes were diffuse alveolar damages (congestion and hemorrhage) and hyaline membrane formation. Notably, although the lung volume was significantly reduced, pulmonary structural dysplasia was not observed at all, presented normal lung development (50%); bilateral (25%) or ipsilateral (25%). Lobulated deformities were accompanied in three (37.5%) cases. All patients exhibited large PDA and a patent foramen ovale with increased right ventricle (RV) volume, and the myocardial fibers were slightly congested and swollen. Pulmonary vessels showed mild to moderate arterial media thickening. Lung hypoplasia and diffuse lung damages reduced gas exchange, meanwhile the PDA and PH caused RV failure, contributed a clinical picture of organ dysfunction, which lead to death. Conclusions: Pulmonary structures have certain heterogeneity in CDH. The arteries’ pathological changes are not consistent with clinical diagnosis. And the adverse outcome may should be due to the cardiopulmonary vicious cycle.
02 Nov 2022Submitted to Pediatric Pulmonology
02 Nov 2022Submission Checks Completed
02 Nov 2022Assigned to Editor
02 Nov 2022Review(s) Completed, Editorial Evaluation Pending
07 Nov 2022Reviewer(s) Assigned
09 Dec 2022Editorial Decision: Revise Major
11 Apr 20231st Revision Received
11 Apr 2023Submission Checks Completed
11 Apr 2023Assigned to Editor
11 Apr 2023Review(s) Completed, Editorial Evaluation Pending
11 Apr 2023Reviewer(s) Assigned
24 Apr 2023Editorial Decision: Revise Minor
22 May 20232nd Revision Received
23 May 2023Submission Checks Completed
23 May 2023Assigned to Editor
23 May 2023Review(s) Completed, Editorial Evaluation Pending
23 May 2023Reviewer(s) Assigned
10 Jun 2023Editorial Decision: Accept