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Pancreatic involvement in hereditary hemorrhagic telangiectasia: assessment with multidetector helical CT.
- Source :
-
Radiology [Radiology] 2010 Feb; Vol. 254 (2), pp. 479-84. - Publication Year :
- 2010
-
Abstract
- Purpose: To evaluate and describe pancreatic involvement by using multidetector computed tomography (CT) in patients with a diagnosis of hereditary hemorrhagic telangiectasia (HHT).<br />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.<br />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.<br />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.<br /> ((c) RSNA, 2010.)
- Subjects :
- Activin Receptors, Type II genetics
Adolescent
Adult
Aged
Contrast Media
Female
Humans
Iohexol
Male
Middle Aged
Mutation
Prospective Studies
Radiographic Image Interpretation, Computer-Assisted
Telangiectasia, Hereditary Hemorrhagic genetics
Arteriovenous Malformations diagnostic imaging
Pancreatic Diseases diagnostic imaging
Telangiectasia, Hereditary Hemorrhagic complications
Telangiectasia, Hereditary Hemorrhagic diagnostic imaging
Tomography, Spiral Computed methods
Subjects
Details
- Language :
- English
- ISSN :
- 1527-1315
- Volume :
- 254
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Radiology
- Publication Type :
- Academic Journal
- Accession number :
- 20093519
- Full Text :
- https://doi.org/10.1148/radiol.09090096