Abstract

CASAS, Jorge et al. An unusual presentation of liver involvement in hereditary hemorrhagic telangiectasia biliary disease. Rev. gastroenterol. Perú [online]. 2009, vol.29, n.3, pp.266-271. ISSN 1022-5129.

Hereditary Hemorrhagic Telangiectasia is a relatively common, under-recognized autosomal dominant disorder characterized by angiodysplastic lesions that affect multiple organs, like liver. This is a case of a 76 years old woman with history of pain in the lower third of right hemithorax, presence of tiny telangiectases in fingers, lips and tongue, in addition to pain on palpation of right hypochondrium; the initial abdominal ultrasound assessment showed biliary intrahepatic dilation. Patient meets The Diagnostic Criteria of Curacao for Hereditary Hemorrhagic Telangiectasia. The presence of liver arteriovenous malformations was confirmed by DOPPLER-ECHO and CT scan.

Keywords : Hereditary-Hemorrhagic-Telangiectasia; Biliary Disease; CT scan; Doppler-ECHO; Arteriovenous Malformations.

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