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Washington University Experience | VASCULAR | Sickle cell anemia | 12A0 Case 12 History
Case 12 History ---- The patient was a 3 1/2 year old African-American girl with sickle-beta thalassemia (red cell size is reduced, symptoms are usually not as severe as HgB-SS) and ulcerative colitis, who presented to an outside ER on 3/16 with seizures. That morning, she complained of right ear pain and right occipital headache. She vomited and then developed episodic facial and arm twitching. She was intubated due to decreasing level of consciousness and respiratory acidosis. CT scan of the head revealed a hypodense area in the left occipital lobe with concomitant acute hemorrhage and areas of infarction in the right tectum and pons. She was transferred to SLCH where repeat head CT showed extension of the occipital infarct and uncal herniation. The clinical impression was basilar artery thrombosis secondary to hemoglobinopathy. At 2220, she suffered a cardiopulmonary arrest and expired. ---- At autopsy, the brain weighed 1200g. Hemorrhagic infarction with subarachnoid hemorrhage involved the right occipital lobe, cerebellum, midbrain and pons. The pattern of injury, including the prominent edema, vascular engorgement, subarachnoid or petechial parenchymal hemorrhage and the occipital and brainstem, and the presence of microthrombi in multiple vessels suggest the existence of diffuse sludging or microthrombosis. Alternatively or in addition, there may have been thrombosis of several larger channels, possibly veins draining the occipital lobes, brainstem and cerebellum.
