Epidermal Nevus Syndrome With Azygos Anterior Cerebral Artery

Epidermal nevus syndrome is a neurocutaneous disorder characterized by the association of epidermal nevi with central nervous system or skeletal abnormalities. Central nervous system abnormalities include hemimegalencephaly, hydrocephalus, various migration disorders, intraspinal lipomas, and enlarg...

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Bibliographic Details
Published in:Pediatric neurology 2008-10, Vol.39 (4), p.283-285
Main Authors: Kara, Bülent, MD, İnan, Nagehan, MD, Bayramgürler, Dilek, MD, Altıntaş, Özgül, MD, Akbulut, Aynur, MD
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - diagnostic imaging
Abnormalities, Multiple - pathology
Anterior Cerebral Artery - abnormalities
Biological and medical sciences
Central Nervous System Vascular Malformations - pathology
Child, Preschool
Female
Humans
Medical sciences
Neurology
Nevus, Pigmented - pathology
Pediatrics
Skin Neoplasms - pathology
Syndrome
Tomography, X-Ray Computed - methods
Vascular diseases and vascular malformations of the nervous system
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Summary:Epidermal nevus syndrome is a neurocutaneous disorder characterized by the association of epidermal nevi with central nervous system or skeletal abnormalities. Central nervous system abnormalities include hemimegalencephaly, hydrocephalus, various migration disorders, intraspinal lipomas, and enlarged spinal roots. Rarely, vascular anomalies cause neurologic signs. We report on a 30-month-old girl with epidermal nevus syndrome who also exhibited vascular malformations of the azygos anterior cerebral artery, a cortical aneurysm, and right internal carotid artery hypoplasia. This rare vascular abnormality was not previously reported in patients with epidermal nevus syndrome.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2008.06.016