Official websites use. Share sensitive information only on official, secure websites. Vitor Pereira, M. The disease can involve a wide variety of tissues derived from all three embryonic layers. NF-1 vasculopathy has been described primarily in peripheral arteries, but arteries supplying the CNS may also be involved.
Of those, extracranial vertebral involvement is the commonest and most important. A series of four patients with NF-1 and vascular disease of the vertebral artery is described with a review of the pathophysiology, vascular phenotypes, their management and the pertinent literature.
Key words: vertebral aneurysms, neurofibromatosis type 1, vertebro-vertebral fistula, endovascular treatment.
Neurofibromatosis type 1 NF-1 is a common genetic disorder with a prevalence of about one patient in 3 - NF-1 primarily affects tissues derived from the neural crest but can also involve non neural crest-derived tissues including bone, brain and blood vessels 1 , 2. NF-1 can go along with three patterns of vascular lesions: arterial stenosis or occlusion, dysplastic changes with aneurysm formation and ruptured arteries causing arteriovenous fistulae. Of these three, stenosis of the renal artery is the most frequent 4 , but occlusion of the cerebral arteries can also occur and may result in "moyamoyalike" disease 1 , 5 , 6.
Aneurysmal formation and fistulae in the head and neck region typically affect the vertebral arteries rather than the carotid arteries, these parachordal or vertebra-vertebral fistulas being the consequence of an arterial rupture into a vein 7 - 9.
