Right-Sided Congenital Diaphragmatic Hernia Caused by Hepatopulmonary Fusion

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. Case Presentation. A term neonate admitted for tachypnea and complete opacification of the right hemithorax was diagnosed with right-sided congenital diaphragmatic hernia. Preoperative respiratory support was minimal, and the only symptom exhibited was tachypnea. During surgical repair, fusion of the lung and liver were noted, consistent with a diagnosis of hepatopulmonary fusion. Postoperatively, the patient’s pulmonary hypertension worsened and required extracorporeal membrane oxygenation. 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.

Congenital Hepatopulmonary Fusion

“…Corrective surgery for patients with HPF, regardless of clinical presentation, should be performed at a center prepared to offer ECMO postoperatively. 35 In this review, the outcome after partial and complete separation between the liver and lung differed between the clinical cases reported in the literature and those reported by the CDH Study Group. Abbreviations: AD, autosomal dominant; AR, autosomal recessive; CDH, congenital diaphragmatic hernia; CNS, central nervous system; VSD, ventricular septal defect.…”

A rare case of hepatopulmonary fusion associated with a right congenital diaphragmatic hernia: Case report and review of the literature

“…Congenital diaphragmatic hernia (CDH) is considered rare, with an estimated incidence of 2.4–4.2 per 10,000 births in the world. This anomaly is characterized by the presence of a defect in the diaphragm during embryonic development, which results in the penetration of abdominal contents into the thoracic cavity and their pressure on the thoracic elements [ 1 ]. The majority of CDHs occur on the left side, accounting for about 80 % [ 2 ].…”

Survival after a two-stage surgical approach in hepatopulmonary fusion: A case report

“…Congenital diaphragmatic hernias are a rare malformation with an incidence of 2.4–4.1 per every 10,000 births worldwide. It is characterized by the appearance of a diaphragmatic defect during development that allows herniation of abdominal organs to the thoracic cavity [ 3 ]. In terms of laterality, 80 % of cases are left sided, 15 % are right sided, and 5 % are present bilaterally [ 4 ].…”