Hereditary hemorrhagic telangiectasia - hepatic involvement

Discussion:

Hepatic vascular malformations suggest liver involvement of hereditary hemorrhagic telangiectasia (HHT), which is an autosomal dominant vascular disease involving multiple systems, whose main pathological change is the presence of abnormal arteriovenous communications.

Liver involvement in HHT is uncommon and almost always asymptomatic. Radiological findings are consequences of the arteriovenous malformations:

  • hepatic artery prominence
  • dilated hepatic vein with early venous drainage
  • arteriovenous shunts

In this case, there was no clinical evidence of telangiectasia. However, CT imaging nevertheless suggests hepatic HHT involvement.

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