Pancreatic arteriovenous malformations (AVM), while extremely rare, are frequently complicated by gastrointestinal bleeding. The elimination of pancreatic AVM is difficult once portal hypertension has developed. We describe herein a patient with congenital AVM of the pancreatic head presenting with recurrent episodes of melena, in whom pylorus-preserving pancreatoduodenectomy provided a means of definitive management. We also review the literature and focus on the diagnostic and therapeutic approaches. Angiography is always necessary to facilitate tactics of treatment, even if diagnosis has been established by non-invasive imaging modalities. To obtain complete regression, total extirpation of the affected organ, or at least the involved portion, should be performed before this disease leads to the lethal complications of gastrointestinal bleeding and portal hypertension. Transcatheter arterial embolization is the only alternative treatment for the control of hemorrhage.
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