Elastic tissue dysplasia of the internal carotid artery

Elongation with kinking of the internal carotid artery is not an uncommon finding. Cerebral vascular insufficiency has been reported secondary to this abnormality. Although arteriosclerosis is a frequent cause in adults, the etiology in children has never been explained. Three children with significant kinking of the internal carotid artery are reported here. Each underwent surgical correction by excision and reconstruction. Histological review showed the elastic tissue to be disrupted, diminished in amount, and unevenly distributed. Retrospective examination of this elastic tissue dysplasia showed it to be localized, there being normal morphology both proximally and distal to the involved area. There was recurrence of elongation and kinking in one patient due to incomplete excision. Correction was accomplished after re-excision of the involved tissue with a vein graft interposition. From this study, it can be concluded that elongation and kinking of the internal carotid artery in children is secondary to elastic tissue dysplasia. It is imperative that the entire involved area be removed in order to prevent recurrence. The excised specimen should be studied histologically with elastic tissue stains. Such stains cannot be performed as frozen section. Should permanent studies show the margins of resection not to be beyond the dysplastic tissue, careful followup of these children is essential to recognize recurrence.