EXCEPTIONAL MULTIPLICITY OF CEREBRAL ARTERIOVENOUS-MALFORMATIONS ASSOCIATED WITH HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU SYNDROME)

PURPOSE: To describe the clinical and imaging features of seven patien ts with hereditary hemorrhagic telangiectasia and an exceptional numbe r of cerebral arteriovenous malformations (AVMs). METHODS: One hundred thirty-six patients from a dedicated hereditary hemorrhagic telangiec tasia clinic were screened systematically for cerebral AVMs by means o f MR imaging, Thirty-one were found to have abnormalities suggestive o f 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 cerebr al AVMs detected ranged from three to nine. At angiography, the AVMs v aried in size from 3 to 25 mm in maximal dimension and consisted of a poorly defined plexiform nidus that typically had a single arterial fe eding pedicle and a single draining vein, The two largest AVMs (20- an d 25-mm nidus, respectively) contained intranidal aneurysms. Treatment included embolization, surgical excision, or follow-up management. CO NCLUSIONS: Multiple cerebral AVMs are associated with hereditary hemor rhagic 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, ca theter angiography is necessary to establish the extent of central ner vous system involvement in this disorder.