Guenther Karl Schneider MD, PhD, Presenter: Nothing to Disclose

Alexander Massmann MD, Abstract Co-Author: Nothing to Disclose

Peter Fries MD, Abstract Co-Author: Nothing to Disclose

Katrin Altmeyer, Abstract Co-Author: Nothing to Disclose

Roland Michael Seidel MD, Abstract Co-Author: Nothing to Disclose

Urban W. Geisthoff, Abstract Co-Author: Nothing to Disclose

To demonstrate imaging findings of liver pathologies in patients with hereditary hemorrhagic telangiectasia [HTT].

203 consecutive patients (87 men, mean age 47.4±17.6 years; 116 women, 46.1±15 years) with diagnosed HHT or first degree relatives were screened for cerebral, abdominal and pulmonary involvement of the disease by MRI. Of these patients 28 presented with arteriovenous malformations (AVMs) of the liver and were further investigated before and after the bolus injection of Gd-BOPTA at a dose of 0.05 mmol/kg BW. Both dynamic and steady state imaging of the liver was performed.

Two different findings in HHT patients with hepatic AVMs were noted. In one group imaging findings included a prominent celiac trunk/hepatic artery and multiple regenerative hepatic lesions leading to an overall increase of liver size. The second group only presented with hepatic AVMs demonstrated by a prominent celiac trunk/hepatic artery and tortuos intrahepatic vessels but no regenerative nodules. In this group liver size was normal or decreased and more often patients presented with RV-insufficiency.

Because of the specific vascular blood supply of the liver, two pathophysiologic mechanisms in HHT patients seem to result in the presence or absence of regenerating nodules and an increased rate of RV insufficiency: a. The origin of regenerative nodules in HHT Patients seems to result from arterio-portal shunts with local overgrowth of hepatic tissue due to increased arterial blood supply which would be consistent with the proposed mechanism of development of FNH. For this reason the appearance of hepatic nodules in HHT patients in the dynamic and hepatobiliary phase after Gd-BOPTA was similar to that discribed for FNH and NRH and should not be misinterpreted for malignant lesions. b. In direct arterio-venous shunts the blood-flow bypasses the liver without influencing liver tissue itself and thus no increase in liver size but an increased RV-volume load is noted.

Arterio-portal shunts in HHT patients may lead to multiple regenerative lesions. RV insufficiency seems to be more common in the abscence of these findings indicating arterio-venous shunting.

Schneider, G, Massmann, A, Fries, P, Altmeyer, K, Seidel, R, Geisthoff, U, MR Imaging of Hepatic Changes in Patients with Rendu-Osler-Weber Syndrome (Hereditary Hemorrhagic Telangiectasia/HHT).  Radiological Society of North America 2007 Scientific Assembly and Annual Meeting, November 25 - November 30, 2007 ,Chicago IL. http://archive.rsna.org/2007/5014127.html