Internal carotid artery occlusion in a child with sickle cell disease: Case report and immunohistochemical study

Purpose: The purpose of this report is to decribe the clinical and pathologic features of a patient with acute thrombosis of both internal carotid arteries leading to death. Methods: This is a case report of special interest because of extensive brain vessel pathologic examination. Results: The analysis of this case showed that the brain had suffered massive infarction and cerebral edema, The internal carotid arteries (ICAs) were occluded by acute thrombus. The arterial wall of the left ICA, studied at its distal segment, showed a small amount of intimal hyperplasia which did not cause encroachment on the lumen, Immunohistochemical stains indicated that this lesion was formed by proliferative vascular smooth muscle rather than incremental thrombus formation. Conclusion: Acute thrombus formation can occur in the large cerebral arteries of children with sickle cell disease in the presence of only minimal intimal hyperplasia. The intimal hyperplasia which forms the sickle related vasculopathy seen on angiography or detected by Transcranial Doppler may be more related to stimulation of smooth muscle cells than dysregulation of thromboregulation at the endothelial surface. Implications for preventive treatment are discussed.