Hepatopulmonary fusion: a rare and potentially lethal association with right congenital diaphragmatic hernia

Abstract

Hepatopulmonary fusion is an extremely rare accompaniment of right congenital diaphragmatic hernia. It is associated with abnormal systemic arterial supply and venous drainage of the right lung along with congenital heart disease. Children with this condition have a comparatively poor prognosis. We report a case of right congenital diaphragmatic hernia with hepatopulmonary fusion along with review of the literature with stress on diagnosis and management.

Hepatopulmonary fusion associated with right-sided congenital diaphragmatic hernia: management of this rare anomaly and a review of the literature

“…This is because the majority of reported cases were discovered intra-operatively. 17 Additionally, the only surrogate indicator mentioned in these previous reports was the absence of mediastinal shift, which was believed to be related to pulmonary hypoplasia. Furthermore, some cases of HPF were associated with mediastinal shift when the defect was large and there were intrathoracic herniated bowels.…”

“…Only 22 cases of HPF were identified (Table 1). [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] The embryological basis for development of HPF is not yet understood. One theory for this development is that failure in formation of the diaphragm during early gestation allows hepatic tissue to herniate into the chest during the 10th week of gestation.…”

High insertion of the right diaphragm complicated with congenital diaphragmatic hernia: A case report of rare thoracoscopic findings

“…Only six cases of high insertion of the diaphragm have been reported in the English literature, [1][2][3][4][5] including one stillborn case due to ascending amniotic infection (Table 1). All cases were right-sided, and the inserted parts and height varied respectively.…”

Muddle in the middle: A rare case of a hepatopulmonary fusion and lung sequestration in a neonate with a right‐sided congenital diaphragmatic hernia

“…CDH occur in 1 in 2500–3000 births, and right‐sided diaphragmatic hernias occur in only 14–25% of these neonates 2 . Right‐sided CDH are associated with poorer outcomes and higher mortality rates, especially if there are concurrent chromosomal or cardiac anomalies, or hypoplastic lung 2,3 . Hepatopulmonary fusion was first described in 1977 and by mid‐2019 there were less than 40 cases reported 4 .…”