To evaluate the agreement between the Buscarini criteria on ultrasound (US) and multiphase contrast-enhanced multidetector-row computed tomography (MDCT) in the diagnosis and staging of liver involvement in hereditary hemorrhagic telangiectasia (HHT) (i.e., Rendu-Osler disease). To evaluate the role of variations in hepatic artery anatomy in the production of disagreement between these two imaging techniques.

This single center retrospective study included 62 patients with confirmed or suspected HHT. Each patient underwent Doppler US analyzed according to the Buscarini criteria and arterial and portal phase MDCT on the same day or one day apart. Liver involvement was classified as absent, slight, moderate or severe for each examination. Agreement was evaluated by the kappa and Bhapkar tests. Variations in hepatic arterial anatomy was analyzed by MDCT based on the Michel's classification and analysis of agreement and disagreement was performed using the Chi² test.

Significant disagreement was identified between the two tests in 29 patients with κ=0.376 and a Bhapkar critical probability of P=0.0053. Staging of liver involvement was significantly more severe with MDCT in case of disagreement. A variant hepatic artery anatomy was identified in 12 patients with agreement and 7 patients with disagreement, but the difference was not significant (Chi²=0.297).

The results of our study confirm the importance of associating contrast-enhanced MDCT with Doppler US in the evaluation of liver involvement in patients with HHT.