Life-threatening gastrointestinal bleeding due to arterio-venous malformation around the pancreatic head is a rare clinical entity. Herein we report a 60-year-old male patient with uncontrolled bleeding from varices of the duodenum due to pancreatic arterio-venous malformation, who was treated by emergency surgery. Upper GI endoscopy revealed bleeding from juxta-ampullary duodenal varices. While contrast-enhanced CT demonstrated a hypervascular mosaic lesion in the region of the pancreatic head, subsequent selective angiography confirmed the presence of the malformation. The patient underwent selective transcatheter arterial embolization, but despite successful embolization of the gastroduodenal artery, bleeding continued and the patient became hemodynamically unstable, at which point he was referred to our department. He underwent an emergency pancreatoduodenectomy with exteriorization of pancreatic juice. During surgery the superior mesenteric vein was very thin (3mm) and was misdiagnosed as a right colic vein, and subsequently divided in this situation. After accomplishing pancreatoduodenectomy, the superior mesenteric vein was reconstructed. During reconstruction of the vein, small bowel congestion became apparent and a temporary porto-systemic bypass was created. Reconstruction was performed by the standard modified Child procedure except for exteriorization of the pancreatic juice. The patient made an uneventful postoperative recovery. Pancreatojejunostomy (2nd stage) was carried out three months after the emergency surgery. Emergency pancreatoduodenectomy is a definitive life-saving procedure when other forms of management such as transcatheter arterial embolization have been exhausted. In this rare situation, the superior mesenteric vein is extremely thin and therefore care should be taken not to damage it.

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