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Washington University Experience | VASCULAR | Hypoxia-Ischemia, fetal-neonatal | Cerebellum | 2A0 Case 2 History
Case 2 History ---- The patient was a 3 year 8 month-old, former 34 week gestation girl with trisomy 21, complete atrioventricular canal, and mitral valve insufficiency. At 1 year of age, a prosthetic mitral valve was placed, complicated by complete heart block requiring pacemaker placement. She later had elective surgery for mitral valve replacement and correction of her subaortic stenosis. Postoperatively, she had fever to 108 ºF with CK levels of 30,000, consistent with malignant hyperthermia, and multiple seizures. MRI of her brain at the outside hospital revealed thalamic and basal ganglia strokes. Treated with heparin followed by Coumadin, she developed a right occipital hemorrhage complicated by development of respiratory failure requiring tracheostomy in September 2009 and, later, a G-tube. She developed MRSA endocarditis. Since the surgery, she stopped having purposeful movements, developed choreic movements in her arms, cortical blindness, and leg weakness suspected to be linezolid neuropathy. In late October she developed a fever and hypotension consistent with septic shock, and was treated with dopamine, epinephrine, and antibiotics. She developed seizures, and she again required mechanical ventilation. The family decided to withdraw support, and she expired. ---- Autopsy showed Down syndrome, foreshortening of frontal lobes with flat occipital lobes, and thin superior temporal gyrus with remote hypoxic-ischemic damage consisting of cerebral atrophy with hydrocephalus ex vacuo, patchy periventricular leukomalacia, laminar cortical necrosis and sclerotic cerebellar cortical atrophy.