Pancreatic involvement in hereditary hemorrhagic telangiectasia: assessment with multidetector helical CT
Alexis Lacout1, Jean-Pierre Pelage, Gilles Lesur
1Department of Radiology, Pluridisciplinary HHT Center, Hôpital Ambroise Paré, Assistance Publique-Hôpitaux de Paris, Université Paris Ile-de-France Ouest, 9, avenue Charles de Gaulle, 92104 Boulogne CEDEX, France.
Purpose:
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.
