Right congenital diaphragmatic hernia associated with fusion of the liver and the lung

Right Congenital Diaphragmatic Hernia Associated With Hepatic Pulmonary Fusion: A Case Report

“…Hepatic and pulmonary tissues fuse by a fibrous band and broncho-biliary fistula formation are the other reported findings associated with HPF. [2] Anomalous venous drainage from the right lung to the intrahepatic inferior vena cava was also reported. [1,5] Treatment of congenital diaphragmatic hernia with HPF is surgical repair, which may require partial hepatectomy and/or pneumonectomy.…”

“…[1][2][3] Most of these cases presented in the early neonatal period or infancy. There may be varying degrees of pulmonary hypoplasia associated with this condition.…”

“…Robertson et al, [2] used LigaSure device (Valley Lab, Boulder, Colo) in order to separate the lung from the liver, and in another case, attempted blunt dissection created a large air leak. [2,4] Slovis et al [4] described 2 cases in whom the defect was repaired without separation of the fused tissue.…”

“…Most patients usually die during the perioperative period due to various complications, including respiratory distress, right heart failure, persistent pulmonary hypertension, and thrombosis of inferior vena cava. [2,6] There are 8 deaths among 14 reported cases of HPF. [2,3] The poor prognosis is usually attributed to CDH related pulmonary complications and any further negative impact on prognosis by the presence of HPF is not proven as yet.…”

“…[2,6] There are 8 deaths among 14 reported cases of HPF. [2,3] The poor prognosis is usually attributed to CDH related pulmonary complications and any further negative impact on prognosis by the presence of HPF is not proven as yet. …”