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American Journal of Neuroradiology 20:1154-1157 (6 1999)
© 1999 American Society of Neuroradiology

ARTICLE

Dysgenesis of the Internal Carotid ArteryAssociated with Transsphenoidal Encephalocele:A Neural Crest Syndrome?

Jerry Blustajna, Irène Netchinea, Daniel Frédya, Pierre Bakouchea, Jean Daniel Piekarskia and Jean François Meder,a

a From the Department of Neuroradiology (J.B., D.F., J.F.M.), Centre Hospitalier Sainte-Anne, the Department of Radiology (J.B., J.D.P.), Fondation Ophtalmologique A. de Rothschild, the Department of Pediatric Endocrinology (I.N.), Hôpital Necker Enfants-Malades, and the Department of Neurology (P.B.), Fondation Ophtalmologique A. de Rothschild, Paris, France.

Summary: We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of the craniofacial arteries, the forebrain, and the face. Data suggest that the link between these rare malformations is abnormal neural crest development.




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Copyright © 2008 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X