Document Type

Article

Publication Date

1-1-2020

Abstract

INTRODUCTION: Hepatopulmonary fusion is a very rare finding associated with right-sided congenital diaphragmatic hernia. With less than 50 reported cases, management and outcomes of hepatopulmonary fusion are poorly understood. This report highlights that clinical presentation is not a reliable indicator of outcomes in this rare disease.

CONCLUSIONS: Many patients with hepatopulmonary fusion and only mild symptoms die postoperatively from severe pulmonary hypertension and progressive respiratory failure. Preoperative clinical status is not indicative of postoperative outcomes, and literature suggests that patients who require less support preoperatively have high mortality rates. The availability of ECMO for postoperative complications may be necessary in patients requiring repair of hepatopulmonary fusion.

Publication Title

Case Rep Pediatr

ISSN

2090-6803

Volume

2020

First Page

8851341

Last Page

8851341

DOI

10.1155/2020/8851341

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