Significant Liver Atrophy Due to Vascular Compromise Associated With Adult Congenital Diaphragmatic Hernia

Diaphragmatic hernia in adults is mostly post-traumatic in origin, and rarely congenital. In both situations, the right side is less commonly involved due to the protection offered by the liver and earlier closure of the right pleuroperitoneal canal. A congenital diaphragmatic hernia may present in adulthood with multi-visceral contents, of which the liver is an extremely rare content, mentioned only in a few previous reports. A herniated liver may mimic a pulmonary tumor and may be completely atrophic due to sustained compression of the venous outflow. Careful operative planning is essential to identify and reduce the liver, along with other contents. We are reporting two adults with a congenital diaphragmatic hernia, with multi-visceral contents and an atrophied liver. The first patient was a 28-year-old man with a remote history of trauma found to have a large right diaphragmatic hernia on imaging. The right liver was completely atrophied due to right hepatic venous compression, while the left liver underwent massive hypertrophy and rotation of the left portal axis. Exploratory laparotomy and reduction of contents, along with mesh repair, were accomplished with satisfactory results. The second patient was a 26-year-old man with Down’s syndrome detected to have multiple bowel loops in the right thorax on imaging. At laparoscopy, a Larrey’s type of Morgagni hernia with a right paramedian defect was found. The left liver was atrophied into a leaf-like appendage due to possible portal obliteration and was dissected away from the diaphragm edge. Appropriate mesh repair was completed by a minimally invasive technique.