Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) or Rendu- Osler Weber syndrome

Background and study aims : Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) is usually asymptomatic and does not require treatment. However, when present, clinical mani- festations can cause considerable morbidity and mortality. Current expertise in the variable clinical manifestations and recommenda- tions for diagnostic approach and management of hepatic involve- ment in HHT are outlined. Methods and materials : A review of current literature was performed using the MEDLINE search string: "Hereditary hemorrhagic telangiectasia [ALL] OR Rendu-Osler-Weber [ALL] AND (liver OR hepatic [ALL])". Results : Due to the lack of therapeutic consequence, systematic screening for hepatic involvement in asymptomatic patients with HHT is currently not recommended. In symptomatic patients, di- agnostic tools include non-invasive techniques such as abdominal color Doppler ultrasound, CT and/or MRI. In any case, liver biop- sy should be avoided in patients with suspected HHT because of the high bleeding risk.