Pancreatic arteriovenous malformation with portal hypertension

A 45-year-old man with recurrent episodes of hematemesis caused by extensive varices in the esophagus and stomach was admitted. He had a history of liver cirrhosis with hepatitis C virus infection. Computed tomography revealed a conglomeration of small strong nodular stains in the pancreatic head. Angiography revealed a racemose vascular network at the same site and early appearance of the portal venous system in the arterial phase. With a diagnosis of pancreatic arteriovenous malformation with portal hypertension, he underwent pylorus-preserving pancreaticoduodenectomy, preceded, 2 days earlier, by transcatheter arterial embolization of some of the feeding arteries. The varices observed preoperatively in the esophagus and stomach disappeared, and he has been well for 6 years after the operation. We reviewed 47 cases of pancreatic arteriovenous malformation previously reported in the English-language literature, with a focus on the clinical manifestations, treatment approaches, and etiological relationship with portal hypertension and liver cirrhosis.