Ileo-caecal arterio-venous malformation associated with extrahepatic portal hypertension: a case report

This paper is a case report describing a boy with Down syndrome and a novel combination of multiple vascular anomalies: extrahepatic portal hypertension, an arterio-venous malformation (AVM) at the ileo-caecal junction, and caval/iliac vein anomalies and developing anal bleeding. We considered that the ileo-caecal AVM would be one of the causes of the repeated hematochezia. The patient underwent ileo-caecal resection with the AVM, and anastomosis of the left external iliac vein and the jejunal branch vein because of the stenosis of the superior mesenteric vein (Clatworthy mesocaval shunt). Intraoperative portal pressure measurement at the site of the right colic vein showed a moderate pressure reduction (42.5-31.5 cm H2O). On the fourth month after operation, gastrointestinal fiberscopy showed no existence of esophageal varices. One year after operation, the patient was doing well without bleeding.