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Washington University Experience | VASCULAR | Sickle cell anemia | 4A0 Case 4 History
Case 4 History ---- The patient was an 18 year old right handed African-American female with sickle cell anemia (diagnosed at 6 months of age), who presented for her final hospital admission on 1/15 with a 24 hour history of vomiting. The patient had multiple hospital admissions during her course for complications of sickle cell anemia, including several for sickle crisis which typically involved abdominal and sometimes joint pain. One admission at the age of 3 1/2 was for meningitis for which she was treated with a 10 day course of Penicillin and Chloramphenicol without the isolation of any organism. At the age of 9, the patient was again admitted with a history of headache and nausea and vomiting over the space of 24 hours. Left hemiparesis and left central facial weakness developed and brain scan showed decreased activity in the distribution of the right middle cerebral artery (MCA). At 14 she presented with aphasia, right 6th nerve palsy, mild right central facial weakness and a new right hemiparesis. Angiography demonstrated occlusion of the right internal carotid 1.5 cm. above the bifurcation and retrograde filling of the right MCA by the right PCA and the right ACA by a hypertrophied right PCA also via the left anterior communicating artery. Also there was occlusion and stenosis distal to the two opercular branches of the left MCA and non-filling of the left ACA from the internal carotid were identified. There were hypertrophied vertebral arteries noted bilaterally with a normal posterior fossa. The patient had multiple subsequent admissions for transfusion therapy for sickle cell anemia and continued to demonstrate spastic quadriplegia, complex dysphasia and pseudobulbar affect. Her present and final admission was precipitated by 24 hours of nausea, vomiting, and mild dehydration. It was the plan of her physicians to slowly rehydrate her using IV and PO intake to do so and after approximately 36 hours of hospitalization, this plan was being carried out with good effect. However, at that time, the patient was noted to begin hyperventilating and she was transferred to Intensive Care with the provisional diagnosis of pulmonary embolus. Her course after transfer was one of rapid deterioration and she expired at the age of 18. ---- At autopsy the brain weighed 1120g. The cerebral hemispheres showed an area of remote infarction involving the right frontal lobe. The anterior half of the circle of Willis was asymmetrical. The supraclinoid portion of the right internal carotid artery and the first portion of the MCA were stenotic and atrophic (1-2 mm in diameter) which extended to the first bifurcation of the MCA. The right ACA contained patches of whitish coloration in the segment between the MCA and the anterior communicating artery. On the left, the internal carotid and MCA, as well as anterior cerebral also were abnormal, the anterior particularly atrophic. Examination of coronal sections of the cerebral hemispheres discloses confluent, small cystic infarcts chiefly involving the white matter of the centrum semiovale, right > left, internal capsule and in both basal ganglia. Posteriorly the infarcts in the white matter are evident as grey, slightly sunken, partially translucent subcortical lesions. The corpus callosum anteriorly was markedly thinned. In both the right and left hemispheres, the frontal and parietal cortex overlying the areas of white matter infarction was intact and of normal thickness and coloration except at the level of the mamillary bodies where a portion of the right middle cortex and inferior frontal gyri is infarcted. No focal lesions were found in the brainstem, cerebellum and spinal cord. The patient also had multiple system defects with multiple infarcts of the lungs, liver and spleen. There was also diffuse bronchopneumonia, aspirated material and pulmonary infarct but no pulmonary emboli.
