Three-dimensional organization of the hepatic microvasculature in hereditary hemorrhagic telangiectasia

Arch Pathol Lab Med. 2001 Sep;125(9):1219-23. doi: 10.5858/2001-125-1219-TDOOTH.


Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant systemic fibrovascular dysplasia. Although hepatic vascular shunts are often observed in HHT, the responsible pathological mechanism is unknown. This issue was addressed by performing a 3-dimensional reconstruction study of the hepatic microvasculature of an HHT-involved liver in a 79-year-old woman. Clinical observation revealed high-output congestive heart failure and hepatic encephalopathy due to arteriovenous and portovenous shunts, respectively. Angiography revealed tortuous dilation of hepatic arterial branches and intrahepatic arteriovenous shunts. The 3-dimensional analysis of the autopsy liver revealed focal sinusoidal ectasia, arteriovenous shunts through abnormal direct communications between arterioles and ectatic sinusoids, and portovenous shunts due to frequent and large communications between portal veins and ectatic sinusoids. Type 1 HHT was suggested by the lack of endoglin immunoreactivity in the liver. The 3-dimensional reconstruction study of hepatic microvasculature was successful in identifying the pathological changes responsible for the intrahepatic shunts in HHT.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma / complications
  • Adenocarcinoma / drug therapy
  • Adenocarcinoma / pathology
  • Adenocarcinoma / radiotherapy
  • Aged
  • Aged, 80 and over
  • Angiography
  • Antimetabolites, Antineoplastic / therapeutic use
  • Autopsy
  • Biopsy, Needle
  • Female
  • Floxuridine / therapeutic use
  • Heart Failure / complications
  • Heart Failure / pathology
  • Hepatic Artery / diagnostic imaging
  • Hepatic Artery / pathology
  • Hepatic Encephalopathy / complications
  • Hepatic Encephalopathy / pathology*
  • Humans
  • Image Processing, Computer-Assisted
  • Liver / blood supply*
  • Liver / pathology*
  • Liver Neoplasms / complications
  • Liver Neoplasms / drug therapy
  • Liver Neoplasms / pathology
  • Liver Neoplasms / radiotherapy
  • Microcirculation / pathology*
  • Telangiectasia, Hereditary Hemorrhagic / complications
  • Telangiectasia, Hereditary Hemorrhagic / pathology*


  • Antimetabolites, Antineoplastic
  • Floxuridine
  • doxifluridine