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 pat hologic features of a patient with acute thrombosis of both internal c arotid arteries leading to death. Methods: This is a case report of sp ecial interest because of extensive brain vessel pathologic examinatio n. Results: The analysis of this case showed that the brain had suffer ed massive infarction and cerebral edema, The internal carotid arterie s (ICAs) were occluded by acute thrombus. The arterial wall of the lef t ICA, studied at its distal segment, showed a small amount of intimal hyperplasia which did not cause encroachment on the lumen, Immunohist ochemical stains indicated that this lesion was formed by proliferativ e vascular smooth muscle rather than incremental thrombus formation. C onclusion: Acute thrombus formation can occur in the large cerebral ar teries of children with sickle cell disease in the presence of only mi nimal intimal hyperplasia. The intimal hyperplasia which forms the sic kle related vasculopathy seen on angiography or detected by Transcrani al Doppler may be more related to stimulation of smooth muscle cells t han dysregulation of thromboregulation at the endothelial surface. Imp lications for preventive treatment are discussed.