Dissecting Hematoma of Intracranial Internal Carotid Artery in an 8-year-old Girl

Abstract

Background:An 8-year-old girl had a minor fall without head trauma and she collapsed the following day while playing. She was awake but mute with focal neurologic signs when admitted to hospital. Radiologic imaging studies showed a progressive left cerebral infarct with left hemisphere vascular narrowing and beading. She died on the third hospital day.Methods:Autopsy including exploration of neck vessels and neuropathological examination was performed. Postmortem studies included immunostaining for immunoglobulins and fixed complement.Results:Subtotal subintimal dissections of both proximal supraclinoid internal carotid arteries were found microscopically. On the left, the subintimal dissection extended into the major branches of the left internal carotid artery as dissecting hematomas with a major compromise of the arterial lumina. Specific IgM deposition at the dissection sites was found. A literature review shows that subintimal dissection of the intracranial internal carotid artery or its branches occurs rarely, it is often fatal, and it is present in patients with a mean age of 17.5 years in cases studied pathologically. Trauma and physical exertion are the most common associated factors.Conclusions:Among the causes of ischemic stroke in young individuals, dissecting hematomas of the intracranial portions of the internal carotid artery system rank low. Few reported cases have identifiable pre-existing pathology. The pathogenesis of dissecting hematomas in this region is reviewed and expanded with speculation regarding relevant developmental, anatomical, flow stress and possibly humoral factors that are involved in the disruption of the arterial elastica and subsequent development and extension of a subintimal hematoma resulting in luminal closure and often death.