Hereditary haemorrhagic telangiectasia: hepatic involvement
Follow up CT scan for vesico-uterine fistula.
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Hepatic vascular malformations suggest liver involvement of HHT.
Arterial phase CT scans demonstrate heterogeneous enhancement of the liver, more evident in the right lobe; portal phase images show a normal liver appearance.
There is also prominence of the hepatic artery and its intrahepatic branches, as well as early filling of a dilated left hepatic vein.
Authors of this case are: Dr. Dario Giambelluca, Dr. Isidoro Cannela, Prof. Giuseppe Brancatelli.
Hereditary hemorrhagic telangiectasia (HHT) , or Osler-Weber-Rendu syndrome, 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.
- 1. Wu JS, Saluja S, Garcia-tsao G et-al. Liver involvement in hereditary hemorrhagic telangiectasia: CT and clinical findings do not correlate in symptomatic patients. AJR Am J Roentgenol. 2006;187 (4): W399-405. doi:10.2214/AJR.05.1068 -
- 2. Garcia-tsao G, Korzenik JR, Young L et-al. Liver disease in patients with hereditary hemorrhagic telangiectasia. N. Engl. J. Med. 2000;343 (13): 931-6. doi:10.1056/NEJM200009283431305