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Washington University Experience | VASCULAR | Hypoxia-Ischemia, fetal-neonatal | White Matter | 3A0 Case 3 History

3A0 Case 3 History
Case 3 History ---- The patient was a 9-day-old female who was born with hypoplastic left heart syndrome with an intact atrial septum and obstructive pulmonary veins. Her medical care was complicated by late presentation for prenatal care as well as maternal gonorrhea infection that was treated within two months of delivery. At birth, the baby was cyanotic and in distress; she was quickly diagnosed with hypoplastic left heart syndrome with an intact atrial septum and was taken to the cardiac catheterization lab for balloon atrial septostomy. Following septostomy she continued to be hemodynamically unstable requiring dopamine and epinephrine infusions for circulatory support. She was subsequently noted to have a firm and distended abdomen which was concerning for necrotizing enterocolitis (NEC). On 3/23, she was noted to have a complex partial seizure involving the right upper extremity that lasted approximately one minute without specific intervention. On EEG testing she was noted to have several short, subclinical seizures and was treated with phenobarbital without return of seizure activity. She had a normal head ultrasound on 3/19 but repeat ultrasound on 3/24 suggested new areas of hemorrhage or infarct. A brain MRI was obtained on 3/25 which showed acute infarcts involving the frontal lobes bilaterally, right greater than left. There was also a small amount of hemorrhagic infarction noted in the right basal ganglia and the genu of the internal capsule. On the evening of 3/27 the family determined to redirect to comfort care and she expired on 3/27. ---- At autopsy the weight of the unfixed brain was 365 grams (normal= 382 + 53 g). She had subacute diffuse hypoxic ischemic white matter gliosis and bilateral neocortical ACA/MCA watershed infarcts.



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