Case 112: Hereditary Hemorrhagic Telangiectasia (HHT), M. Osler

Diagnostic Categories:

Clinical Presentation:

49 year old woman on the waiting list for liver transplant for end stage liver disease.

CT: Advanced stage, with bilomas

Imaging Findings:

Hereditary telengiectasia. CT shows innumerable hypervascular foci representing arterio-venous malformations replacing a large volume of parenchyma, causing ischemia to liver parenchyma and bile ducts. Cystic spaces are biliary lakes (bilomas) due to ischemia. Note also multiple capsular vascular shunts, including shunts in the abdominal and thoracic wall. Diagnosis was confirmed upon hepatectomy following orthotopic liver transplantation.