Complex pancreatic vascular anomalies in children.

BACKGROUND: Vascular anomalies are vascular tumors and congenital malformations that rarely involve the pancreas. Diagnosis and management of these lesions is complex.
METHODS: An institutional review board-approved retrospective database and record review from 1994 through 2004 at a quaternary referral center for vascular anomalies was conducted.
RESULTS: Of 5051 patients with a vascular anomaly, 6 had a lesion involving the pancreas. All patients were younger than 3 years. There were three tumors (two infantile hemangiomas and one Kaposiform hemangioendothelioma) and three malformations (two lymphatic and one venous). The referring diagnoses were correct for 4 patients. All anomalies were diagnosed with a combination of cross-sectional imaging and angiography. Five patients received medical therapy including two malformation patients who were initially treated with antiangiogenic drugs. Two patients with lymphatic malformations underwent operative intervention. One infantile hemangioma was treated with percutaneous biliary drainage for obstructive jaundice.
CONCLUSION: Complex pancreatic vascular anomalies are rare. Vascular tumors are generally amenable to treatment with antiangiogenic medications. Operative resection is reserved for refractory cases or complications. Symptomatic vascular malformations are treated exclusively by surgery. Evaluation and management of these lesions is complex and may benefit from coordinated interdisciplinary care.