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Spectrum and prevalence of vasculopathy in pediatric neurofibromatosis type 1.

Kaas B, Huisman TA, Tekes A, Bergner A, Blakeley JO, Jordan LC
J Child Neurol. 2013 28 (5): 561-9

PMID: 22832780 · PMCID: PMC3496801 · DOI:10.1177/0883073812448531

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.

MeSH Terms (22)

Angiography Aorta, Abdominal Aortic Diseases Arterial Occlusive Diseases Cerebral Angiography Child Child, Preschool Comorbidity Cross-Sectional Studies Disease Progression Female Humans Hypertension Image Enhancement Ischemic Attack, Transient Magnetic Resonance Angiography Male Moyamoya Disease Neurofibromatosis 1 Optic Nerve Glioma Stroke Tomography, X-Ray Computed

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