Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: A neural crest syndrome?

We describe two original cases of internal carotid artery dysgenesis associ ated with a malformative spectrum, which includes transsphenoidal encephalo cele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic ne ural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior sku ll base, the walls of the craniofacial arteries, the forebrain, and the fac e. Data suggest that the link between these rare malformations is abnormal neural crest development.