Hepatic Pulmonary Fusion: Two Cases with Diaphragmatic Hernia and One Case with Pentalogy of Cantrell

Abstract

Hepatic pulmonary fusion (HPF) is characterized by a fibrous connection between the liver and lung tissue. We present two cases of hepatic pulmonary fusion diagnosed with right diaphragmatic hernia and a third case with Pentalogy of Cantrell exhibiting complete agenesis of the diaphragm and finger-like projections of liver adhered to the right lung. It has been proposed that this anomaly is secondary to developmental failure of the mesoderm between days 14–18 after conception and is attributed to diaphragmatic maldevelopment. Understanding the molecular-genetic basis of diaphragmatic hernias may shed light on this unusual presentation and explain why other cases show no fusion.

Keywords :

• hepatic pulmonary fusion (HPF)
• congenital diaphragmatic hernia
• Pentalogy of Cantrell