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Abstract

Exceptional multiplicity of cerebral arteriovenous malformations associated with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).

C M Putman, J C Chaloupka, R K Fulbright, I A Awad, R I White and P B Fayad
American Journal of Neuroradiology October 1996, 17 (9) 1733-1742;
C M Putman
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J C Chaloupka
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R K Fulbright
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I A Awad
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R I White Jr
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P B Fayad
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Abstract

PURPOSE To describe the clinical and imaging features of seven patients with hereditary hemorrhagic telangiectasia and an exceptional number of cerebral arteriovenous malformations (AVMs).

METHODS One hundred thirty-six patients from a dedicated hereditary hemorrhagic telangiectasia clinic were screened systematically for cerebral AVMs by means of MR imaging. Thirty-one were found to have abnormalities suggestive of a vascular malformation. Eighteen of these 31 patients subsequently underwent diagnostic cerebral angiography.

RESULTS Of the 18 patients who had cerebral angiography, all were found to have at least one AVM and seven were found to have three or more AVMs. The number of cerebral AVMs detected ranged from three to nine. At angiography, the AVMs varied in size from 3 to 25 mm in maximal dimension and consisted of a poorly defined plexiform nidus that typically had a single arterial feeding pedicle and a single draining vein. The two largest AVMs (20- and 25-mm nidus, respectively) contained intranidal aneurysms. Treatment included embolization, surgical excision, or follow-up management.

CONCLUSIONS Multiple cerebral AVMs are associated with hereditary hemorrhagic telangiectasia and further highlight the uniqueness of central nervous system involvement by this systemic angiodysplasia. MR imaging can underestimate the number and size of cerebral AVMs; therefore, catheter angiography is necessary to establish the extent of central nervous system involvement in this disorder.

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American Journal of Neuroradiology
Vol. 17, Issue 9
1 Oct 1996
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Exceptional multiplicity of cerebral arteriovenous malformations associated with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).
C M Putman, J C Chaloupka, R K Fulbright, I A Awad, R I White, P B Fayad
American Journal of Neuroradiology Oct 1996, 17 (9) 1733-1742;

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Exceptional multiplicity of cerebral arteriovenous malformations associated with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).
C M Putman, J C Chaloupka, R K Fulbright, I A Awad, R I White, P B Fayad
American Journal of Neuroradiology Oct 1996, 17 (9) 1733-1742;
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  • Rare manifestations in a case of Osler-Weber-Rendu disease
  • Brain Arteriovenous Malformation Multiplicity Predicts the Diagnosis of Hereditary Hemorrhagic Telangiectasia: Quantitative Assessment
  • Intracranial Hemorrhage in Infants and Children With Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome)
  • Reporting Terminology for Brain Arteriovenous Malformation Clinical and Radiographic Features for Use in Clinical Trials
  • Cerebrovascular Manifestations in 321 Cases of Hereditary Hemorrhagic Telangiectasia
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