Abstract

AUN, Romina et al. Spectrum of abdominal findings by multidetector computed tomography in hereditary hemorrhagic telangiectasia. Rev. argent. radiol. [online]. 2013, vol.77, n.2. ISSN 1852-9992.  http://dx.doi.org/10.7811/rarv77n2a09.

Learning objectives. To demonstrate the utility of multidetector computed tomography (MDCT) in conducting a specific study protocol for the diagnosis, characterization and differentiation of various abdominal vascular lesions found in hereditary hemorrhagic telangiectasia (HHT). Topic review. Hereditary hemorrhagic telangiectasia or Rendu-Osler-Weber syndrome is a multisystemic vascular disorder characterized by the development of angiodisplasic lesions, with direct communication between arteries and veins without a capillary network in between. It is transmitted as an autosomal dominant feature with a prevalence of 1-2 cases per 10,000 people. Clinically, it is characterized by the presence of mucocutaneous telangiectasias with recurrent epistaxis and gastrointestinal bleeding, as well as development of shunts, which depending on the affected organ, can cause hypoxemia, stroke, heart failure or brain abscess-es. Diagnosis is based on a combination of clinical history, physical examination and diagnostic methods. Imaging findings. The role of multidetector computed tomography in the diagnosis of hereditary hemorrhagic telangiectasia is becoming increasingly important, as it allows to obtain high resolution images with a spatial and temporal specific multiphase protocol. Such protocol consists in an early arterial phase, a late arterial phase within 20 seconds to the previous one and a venous phase 40 seconds after the first one. According to their behavior in the different phases, we can identify lesions such as telangiec-tasias, confluent vascular masses, hepatic perfusion disorders, arteriovenous, arterioportal or portal venous shunts and arterial aneurysms. Conclusion. Multidetector computed tomography, with its high spatial and temporal resolution and a specific multiphase protocol, allows recognition and characterization of typical lesions of this pathology in abdominal organs (main-ly the liver), contributing to the diagnosis of this disease.

Keywords : Hereditary hemorrhagic telangiectasia; Multidetector computed tomography; Rendu-Osler-Weber Rendu; Shunts; Telangiectasias.

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