Published online August 24, 2009
PEDIATRICS Vol. 124 No. 3 September 2009, pp. e476-e483 (doi:10.1542/peds.2009-0152)
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Submit a response
Right arrow View responses
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrowRequest Permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Google Scholar
Right arrow Articles by Rea, D.
Right arrow Articles by Askalan, R.
PubMed
Right arrow PubMed Citation
Right arrow Articles by Rea, D.
Right arrow Articles by Askalan, R.
Related Collections
Right arrow Neurology & Psychiatry
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Facebook   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

ARTICLE

Cerebral Arteriopathy in Children With Neurofibromatosis Type 1

David Rea, MDa, John F. Brandsema, MDb, Derek Armstrong, MDa, Patricia C. Parkin, MDc, Gabrielle deVeber, MD, MHScb, Daune MacGregor, MDb, William J. Logan, MDb and Rand Askalan, MD, PhDb

a Division of Pediatric Neuroimaging, Department of Diagnostic Imaging, and Divisions of
b Neurology
c Pediatric Medicine, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada

OBJECTIVE: Cerebrovascular abnormalities are serious but underrecognized complications of neurofibromatosis type 1 (NF1). The aim of this study was to investigate the prevalence, clinical presentation, imaging findings, and prognosis of cerebral arteriopathies in childhood NF1.

METHODS: Patients followed at the NF1 clinic at the Hospital for Sick Children, Toronto, Ontario, Canada, between 1990 and 2007 were studied. Patients with confirmed NF1 diagnosis and neuroimaging results were included. All neuroimaging studies were reviewed for the presence of arteriopathy by 2 study pediatric neuroradiologists blinded to clinical information. Clinical records of children with cerebral arteriopathy were reviewed.

RESULTS: Among 419 children with confirmed NF1, 266 (63%) received neuroimaging. Among children with neuroimaging results, 17 had cerebral arteriopathy (minimum prevalence rate of 6%). Among the 35 patients who received magnetic resonance angiography (MRA), arteriopathy was more common in patients with NF1 with optic gliomas (11 of 21) compared with those without optic glioma (4 of 14). Forty-seven percent of children developed focal deficits months to years after the diagnosis of the arteriopathy. Follow-up at a mean of 7 years after diagnosis of arteriopathy showed that 35% (6 of 17) had progressive arteriopathy requiring revascularization surgery. Seven patients received aspirin for primary stroke prevention. On retrospective review of imaging studies, a mean delay of 51 months to clinical radiographic reporting of these findings was observed.

CONCLUSIONS: The prevalence of cerebral arteriopathy in children with NF1 in this study was at least 6% and was associated with young age and optic glioma. Arteriopathy causes stroke with resultant neurologic deficits. Medical and/or surgical interventions may prevent these complications. Therefore, the addition of vascular imaging (MRA/conventional angiography) to brain imaging studies for early detection of arteriopathy should be considered for children with NF1, particularly young patients with optic glioma.

Key Words: neurofibromatosis • cerebral arteriopathy • childhood stroke

Abbreviations: FASI—focal areas of abnormal signal intensity • FLAIR—fluid-attenuated inversion recovery • MRA—magnetic resonance angiography • NF1—neurofibromatosis type 1 • CVR—cerebrovascular reactivity

Accepted Apr 10, 2009.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Facebook Facebook   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


eLetters:

Read all eLetters

Cerebral Arteriopathy in Children With Neurofibromatosis Type 1
Fernando Maria de Benedictis, et al.
Pediatrics Online, 21 Sep 2009 [Full text]