Spectrum and Prevalence of Vasculopathy in Pediatric Neurofibromatosis Type 1

To describe the spectrum and associated clinical features of peripheral and cerebral vasculopathy in pediatric patients with neurofibromatosis type 1, children seen at a single center from 2000 to 2010 with appropriate imaging studies were identified. Scans were assessed for vascular disease by 2 pe...

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Bibliographic Details
Published in:Journal of child neurology 2013-05, Vol.28 (5), p.561-569
Main Authors: Kaas, Bonnie, Huisman, Thierry A. G. M., Tekes, Aylin, Bergner, Amanda, Blakeley, Jaishri O., Jordan, Lori C.
Format: Article
Language:English
Subjects:
Angiography
Aorta, Abdominal - abnormalities
Aortic Diseases - diagnosis
Aortic Diseases - epidemiology
Arterial Occlusive Diseases - diagnosis
Arterial Occlusive Diseases - epidemiology
Cerebral Angiography
Child
Child, Preschool
Comorbidity
Cross-Sectional Studies
Disease Progression
Female
Humans
Hypertension - diagnosis
Hypertension - epidemiology
Image Enhancement
Ischemic Attack, Transient - diagnosis
Ischemic Attack, Transient - epidemiology
Magnetic Resonance Angiography
Male
Moyamoya Disease - diagnosis
Moyamoya Disease - epidemiology
Neurofibromatosis 1 - diagnosis
Neurofibromatosis 1 - epidemiology
Optic Nerve Glioma - diagnosis
Optic Nerve Glioma - epidemiology
Stroke - diagnosis
Stroke - epidemiology
Tomography, X-Ray Computed
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Summary:To describe the spectrum and associated clinical features of peripheral and cerebral vasculopathy in pediatric patients with neurofibromatosis type 1, children seen at a single center from 2000 to 2010 with appropriate imaging studies were identified. Scans were assessed for vascular disease by 2 pediatric neuroradiologists. Of 181 children, 80 had pertinent imaging studies: 77 had brain imaging, 6 had peripheral imaging, and 3 had both. Vasculopathy was identified in 14/80 children (18%, minimum prevalence of 14/181; 8%). Of those with vascular abnormalities, 2/14 had peripheral vasculopathy (1% minimum prevalence) and 12/14 had cerebrovascular abnormalities (7% minimum prevalence). No associations were found between vasculopathy and common clinical features of neurofibromatosis type 1, including optic pathway glioma, plexiform neurofibroma, skeletal abnormalities, attention-deficit hyperactivity disorder (ADHD), or suspected learning disability. Both peripheral and cerebral vasculopathy are important complications of pediatric neurofibromatosis type 1 and should be considered in the management of this complex disease.
ISSN:0883-0738
1708-8283
DOI:10.1177/0883073812448531