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Everything you need to know about Computed Tomography (CT) & CT Scanning

Syndromes in CT: Hht Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Syndromes in CT ❯ HHT

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  • ”Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is an autosomal dominant inherited disorder with an estimated prevalence of 1–2 individuals per 10,000. It is characterized by vascular abnormalities consisting of dilated, disorganized venules, with direct connection to arterioles (a.k.a. arteriovenous malformations or telangiectasias). The disease affects multiple organs and vascular beds throughout the body, with the skin, mucous membranes, lungs, central nervous system, gastrointestinal tract and liver among the most common sites of involvement.”
    Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
    Christopher L. Welle et al.
    Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7
  • “Clinical diagnosis of HHT is made via the Curacao cri- teria. The four criteria are: (1) spontaneous and recurrent epistaxis, (2) characteristic mucocutaneous telangiectasias (lips, oral cavity face and fingers), (3) visceral (brain, lung, liver, bowel) arteriovenous malformations (AVM), and (4) diagnosis of HHT in a first degree relative utilizing these same Curacao criteria. Patients with ≥ 3 criteria are labeled as “definite HHT” while those with 2 of the 4 criteria are labeled as “possible” or “suspected” HHT
    Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
    Christopher L. Welle et al.
    Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7
  • “In patients with the clinical diagnosis of HHT, greater than half demonstrate an abdominal vascular abnormality, with the most commonly involved organ being the liver. These may be under recognized on routine or single phase contrast- enhanced CT of the abdomen. This supports the use of optimized multiphasic abdominal CT exams as an important tool for the evaluation and screening of patients with HHT.”
    Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
    Christopher L. Welle et al.
    Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7
  • “In patients with abdominal vascular abnormalities, the liver was the most commonly involved organ, with 180 out of 333 (54.1%) patients demonstrating at least one hepatic vascular abnormality (telangiectasia, confluent vascular mass, transient perfusion abnormalities, and hepatic shunts), with most (70.0%) demonstrating multiple hepatic vascular abnormalities. The other most common organs involved included the pancreas (18.0%), spleen (6.3%), and small bowel (4.5%).”
    Abdominal manifestations of hereditary hemorrhagic telangiectasia: a series of 333 patients over 15 years
    Christopher L. Welle et al.
    Abdominal Radiology https://doi.org/10.1007/s00261-019-01976-7
© 1999-2019 Elliot K. Fishman, MD, FACR. All rights reserved.