A Morgagni hernia with an absent ductus venosus: An unusual case causing unusual consequences

• There may be an association between an absent ductus venosus and Morgagni (M) hernia.
• Extrahepatic drainage of the umbilical vein alters the presentation of M hernia.
• Primary laparoscopic repair of M hernia with ductus venosus absence is feasible.

A Morgagni hernia is a rare form of congenital diaphragmatic hernia (CDH), comprising only 3–5% of all CDH cases. Agenesis of the ductus venosus with direct umbilical vein blood flow to the heart is a relatively uncommon finding that is often fatal in utero. We present a case of a 2-month-old infant with Morgagni hernia and absence of the ductus venosus. These combined defects led to neovascularization of the liver, severe pulmonary hypertension and right heart failure. In this report, we describe a Morgagni hernia that's presentation resembled that of a Bochdalek hernia likely because of concomitant absence of the ductus venosus causing severe pulmonary hypertension.