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Obstruction of major cerebral vessels in early childhood without neurological signs

From the Departments of Pediatrics and Radiology, Washington University School of Medicine, and St. Louis Children's Hospital, St. Louis, Mo.

SUMMARYA 7-year-old girl, who was born with a cavernous hemangioma of the right side of the face involving the right orbit, was investigated because of complaints of repeated, severe, right frontal headaches. Angiography revealed the right internal carotid artery to be absent, and polytome laminograms of the petrous portion of the temporal bone also revealed the right internal carotid canal to be absent. The left internal carotid artery and both vertebral arteries were obstructed shortly after entry into the cranial cavity. The external carotid arteries supplied the brain via transdural anastomoses and bilateral rete mirabile caroticum.

This child is the first patient not of Japanese ancestry to be described with intracranial occlusion of the major transcervical vessels. She has no neurological signs apart from loss of vision in the right eye which can be attributed to her hemangioma, functions at a superior intellectual level, and has had a nonprogressive course.

The absence of the right internal carotid artery and its canal suggests to us that this type of occlusive process can occur early in intrauterine life.

Dr. Prensky's address is 500 South Kingshighway, St. Louis, Mo. 63110.

Supported in part by United States Public Health Service grants Ncs. NS-5633, 5 T01 NB 05522, and 5 P01 MS 06833 and by grants from the Allen P. and Josephine B. Green Foundation, Mexico, Mo.

Submitted for publication Feb. 12, 1970; accepted March 2, 1970.

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