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Washington University Experience | VASCULAR | Hypoxia-Ischemia, fetal-neonatal | Status Marmoratus | 3A0 Case 3 History
Case 3 History ---- The patient was a three year old female born at 25 weeks EGA. Extreme prematurity resulted in neonatal ICU stay of 5 months. She developed severe bronchopulmonary dysplasia, hypothyroidism, and multiple infections. Head ultrasound performed on DOL 3 and 10 demonstrated multiple cystic areas, radiologically consistent with PVL in the left frontal parietal region which showed interval resolution and incorporation into the ventricle at 4-6 months.. She eventually was discharged to home at 3 months corrected age. An EEG performed at age three months was negative. At 1 1/2 years of age she was diagnosed with severe developmental delay with expressive language function, severe truncal and extremity hypotonicity, and profound deafness. An EEG at the age of 2 showed diffuse slowing with an MRI showing hypoplastic cerebellum and brainstem, abnormal deep white matter T2 signal consistent with delayed myelination, and a hypoplastic posterior corpus callosum. Her parents placed her in hospice care at home and was brought to the ER shortly thereafter and was pronounced dead. ---- At autopsy the weight of the unfixed brain was 1,010 g (normal = 1211 ± 98g ). Examination showed chronic diffuse hypoxic/ischemic injury characterized by microencephaly with diffuse white matter damage, multlifocal remote hemorrhagic infarcts of the atrophic cerebellum with atrophy of the olivary bodies and basis pontis.
