Late-onset inferior vena cava obstruction in a shunted patient--a unique cause of rebleeding in children with portal hypertension.

Background: Rebleeding in the presence of an adequate patent portosystemic shunt in a patient with portal hypertension (PHT) is uncommon. Inferior vena cava (IVC) obstruction as the cause of rebleeding in this situation has not been reported in the literature.

Methods: Records from a pediatric tertiary care center were reviewed over a 15-year period. Portosystemic shunt procedures for bleeding esophageal varices were done in 39 children. Patients who, after a shunt surgery for PHT, developed a rebleed because of IVC obstruction in the presence of a patent shunt were identified.

Results And Conclusions: Late IVC obstruction in the presence of a patent shunt was identified in 2 patients. The etiology included adhesions, caudate lobe hypertrophy, and macronodular cirrhosis. Diagnosis was by angiography, and treatment included angioplasty and liver transplantation. Awareness of this condition helps direct treatment appropriately in the clinical scenario of a rebleed in a shunted patient with PHT.