Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant
vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous
malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary
hemorrhagic telangiectasia), although symptomatic liver disease is quite infrequent. In symptomatic cases, clinical
manifestations relate most commonly to the predominant type of vascular shunting present (arteriovenous, arterioportal, or
portovenous). Clinically, liver disease can manifest as a high-output cardiac failure, portal hypertension, or biliary disease.
Imaging plays an important role in diagnosis, characterization, and follow-up of liver involvement, with ultrasound, computed
tomography, magnetic resonance imaging, and angiography being useful in this context. We present a case of patient with
Osler-Weber-Rendu syndrome with portal hypertension without evidence of liver cirrhosis.
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