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Washington University Experience | VASCULAR | Sickle cell anemia | 2A0 Case 2 History
Case 2 History ---- The decedent was a 15 year old African-American female with a past medical history of Hgb-SS disease and chronic transfusions admitted to the SLCH on 06/19 for vaso-occlusive crisis. One week after admission, on 06/25, she developed mental status changes accompanied by elevated ammonia levels, which did not respond to biochemical interventions (Ammonul). She further developed hemodynamic instability, requiring epinephrine infusion, and progressive acidosis and was subsequently transferred to the pediatric ICU. At that point, CT and MRI scans showed no hemorrhage or global edema. She remained in the pediatric ICU for liver failure, severe encephalopathy with medically refractory cerebral edema, and intracranial hypertension. Head CT on 6/27 showed a large intraparenchymal hemorrhage associated with a right frontal approach ventriculostomy catheter, with a small amount of hemorrhage extending into the right Sylvian fissure. In the 24 hours preceding her death, she developed worsening pulmonary compliance, hypoxemia and hemodynamic instability despite escalation of epinephrine and norepinephrine and multiple fluid boluses. She arrested and the family asked to stop chest compressions. She had a weak pulse after compressions were held, and ultimately became more bradycardic and hypotensive. Her vasoactive infusions were stopped and she ultimately progressed to asystole and died surrounded by family on 7/21. ---- At autopsy the weight of the unfixed brain was 1360g. Multiple subacute infarcts involved the cerebral hemispheres, basal ganglia, thalamus, brainstem and cerebellum most with accompanying intravascular plugs of sickled red cells. Microscopically, acute and subacute infarcts were identified in almost all sections examined. In addition, a large, right frontal hemorrhage was found at the site of ventriculostomy placement. Diffuse cerebral edema was also present. The white matter composing the cerebellar arbor vitae appears preferentially involved and pale with sickle-cell occlusive micro-vasculopathy which may reflect the relative hypo-vascularity of white matter in comparison to gray.
