title: Spectrum and prevalence of vasculopathy in pediatric neurofibromatosis type 1.
abstract: 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.
authors: Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors: Bonnie
authors: B
authors: Kaas
authors: Thierry A G M
authors: TA
authors: Huisman
authors: Aylin
authors: A
authors: Tekes
authors: Amanda
authors: A
authors: Bergner
authors: Jaishri O
authors: JO
authors: Blakeley
authors: Lori C
authors: LC
authors: Jordan
mesh_terms: D000792:Angiography; D001012:Aorta, Abdominal / Q000002:abnormalities*; D001018:Aortic Diseases / Q000175:diagnosis* / Q000453:epidemiology*; D001157:Arterial Occlusive Diseases / Q000175:diagnosis* / Q000453:epidemiology*; D002533:Cerebral Angiography; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D006973:Hypertension / Q000175:diagnosis* / Q000453:epidemiology*; D007089:Image Enhancement; D002546:Ischemic Attack, Transient / Q000175:diagnosis / Q000453:epidemiology; D018810:Magnetic Resonance Angiography; D008297:Male; D009072:Moyamoya Disease / Q000175:diagnosis* / Q000453:epidemiology*; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000453:epidemiology*; D020339:Optic Nerve Glioma / Q000175:diagnosis / Q000453:epidemiology; D020521:Stroke / Q000175:diagnosis / Q000453:epidemiology; D014057:Tomography, X-Ray Computed