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Pancreatic involvement in hereditary hemorrhagic telangiectasia: assessment with multidetector helical CT Lacout A; Pelage JP; Lesur G; Chinet T; Beauchet A; Roume J; Lacombe PRadiology 2010[Feb]; 254 (2): 479-84PURPOSE: To evaluate and describe pancreatic involvement by using multidetector computed tomography (CT) in patients with a diagnosis of hereditary hemorrhagic telangiectasia (HHT). MATERIALS AND METHODS: Institutional review board approval was obtained, and all patients provided informed consent. Across 12 months, all consecutive adult patients with a confirmed diagnosis of HHT referred to our pluridisciplinary HHT center for evaluation were enrolled prospectively in the study and underwent contrast material-enhanced multidetector CT of the abdomen. Pancreatic telangiectases and arteriovenous fistulas were noted, and their characteristics were described. Genetic mutation was also investigated. RESULTS: Thirty-five patients (19 women, 16 men; mean age, 48.4 years) were included. All patients were asymptomatic. A genetic mutation was identified in 28 (80%) patients, including endoglin in 16 (57%), activin type-II-like receptor kinase 1 (ALK1) in 11 (39%), and SMAD4 in one (4%). Eleven (31%) patients exhibited pancreatic involvement. Fifty-four percent of patients with ALK1 mutation had pancreatic involvement. Twenty-three pancreatic telangiectases were identified during the arterial phase in nine patients. Seven pancreatic arteriovenous malformations (AVMs) were identified in four patients. CONCLUSION: Pancreatic involvement commonly is found in patients with HHT (31% in our study), mainly in patients with ALK1 mutation; pancreatic telangiectases or AVMs are only diagnosed duringthe arterial phase at multidetector CT.|Activin Receptors, Type II/genetics[MESH]|Adolescent[MESH]|Adult[MESH]|Aged[MESH]|Arteriovenous Malformations/*diagnostic imaging[MESH]|Contrast Media[MESH]|Female[MESH]|Humans[MESH]|Iohexol[MESH]|Male[MESH]|Middle Aged[MESH]|Mutation[MESH]|Pancreatic Diseases/*diagnostic imaging[MESH]|Prospective Studies[MESH]|Radiographic Image Interpretation, Computer-Assisted[MESH]|Telangiectasia, Hereditary Hemorrhagic/*complications/*diagnostic imaging/genetics[MESH]|Tomography, Spiral Computed/*methods[MESH] |
