Introduction

The evaluation of hepatic dysfunction requires careful consideration of both intrinsic and extrinsic etiologies. Hepatic venous outflow tract obstruction (HVOTO) presents a characteristic histologic injury pattern, including peri-central vein sinusoidal dilation, central vein fibrosis, and peri-central vein hemorrhage. Rarely, this is due to extrinsic compression of the IVC. We present two cases of HVOTO secondary to dilated thoracic digestive organs compressing the thoracic IVC.

Case 1: A 17-year-old patient with MMIHS, megaesophagus, and liver-small bowel transplant presented with elevated transaminases. Liver biopsy showed congestive hepatopathy without rejection. CT revealed HVOTO due to compression of the thoracic IVC from dilation of the esophagus with retained food. (Figure 1)

Case 2: A 3-year-old with intestinal failure associated liver disease was evaluated for liver-small bowel transplant. She had a history of esophageal atresia status post gastric pull-up and volvulus leading to intestinal failure. Her liver evaluation showed hepatomegaly, early bridging fibrosis, and sinusoidal fibrosis. Imaging revealed no vascular obstructions, but an empty stomach within the thoracic cavity near the posterior heart. The final diagnosis was intermittent extrinsic compression of the thoracic IVC due to dynamic stomach enlargement with food. (Figure 1)

Discussion

These cases highlight the importance of considering the patient's medical and anatomic history in the evaluation of liver dysfunction. When patients have abnormally large thoracic digestive organs, these can lead to compression of the IVC. An empty lumen on imaging may not immediately reveal how a compliant organ may dilate with food leading to intermittent compression of the IVC.