Right congenital diaphragmatic hernia associated with hepatic pulmonary fusion

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

“…When a CDH is present on the right side, it may be associated with hepatic-pulmonary fusion (HPF), a very rare deformity. Only a few cases of this anomaly have been reported, affecting about 3 in 1000 patients [ 3 ]. In HPF, there is fibrous vascular fusion between the liver and lungs [ 4 ], which makes the treatment of this type of CDH very difficult and important.…”

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

“…Hepatopulmonary fusion is a rare and highly mortal malformation exclusively seen in right sided defects, characterized by the fibrovascular fusion of the liver and lung, affecting 3 in every 1000 patients [ 5 ]. Although its pathophysiology has not been fully understood, two theories are accepted: the appearance of a diaphragmatic defect during organ development and a preceding fusion causing a diaphragmatic hernia [ 5 ].…”

“…Hepatopulmonary fusion is a rare and highly mortal malformation exclusively seen in right sided defects, characterized by the fibrovascular fusion of the liver and lung, affecting 3 in every 1000 patients [ 5 ]. Although its pathophysiology has not been fully understood, two theories are accepted: the appearance of a diaphragmatic defect during organ development and a preceding fusion causing a diaphragmatic hernia [ 5 ]. A case report of a patient intraoperatively diagnosed with hepatopulmonary fusion in a right congenital diaphragmatic hernia managed with a two-stage surgical approach in an academic hospital is presented; 50 cases had been reported worldwide as of 2020, most of them with fatal outcomes [ 3 ].…”