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Washington University Experience | VASCULAR | Hypoxia-Ischemia, fetal-neonatal | Status Marmoratus | 1A0 Case 1 History
Case 1 History ---- The patient was born at estimated gestational age (EGA) of 26 weeks to a 19 year old mother with a prior history of birth to a 24 week EGA infant (which had necrotizing enterocolitis (NEC) and grade 4 IVH before death on day of life (DOL) 8). The patient's mother had prenatal care and denied alcohol and substance abuse during the pregnancy. Her pregnancy was complicated by preterm labor with vaginal bleeding. C-section delivery of a 728 gram baby resulted in Apgars were 4, 5, 5. The child’s ICU course was remarkable for severe respiratory distress syndrome with subsequent bronchopulmonary dysplasia (BPD), NEC with perforated bowel, s/p partial bowel resection, conjugated hyperbilirubinemia, thrombocytopenia, Klebsiella sepsis (CSF negative). Hypotensive episodes in the first two weeks of life necessitating administration of pressors with subsequent borderline high BPs. A head ultrasound done on DOL 3 showed a large IVH maximal in the left frontal horn, extending to adjacent left frontal lobe; there was also blood in both ventricles with asymmetric ventricular dilatation (R >L) followed by partial interval resorption. Repeat ultrasound on 6/30 (first at SLCH) showed a 1 cm L intraparenchymal hemorrhage, 1 cm subependymal bleed, and a small IVH. Followup ultrasounds on 8/21, 9/18, and 10/11 showed resolving IVH and development of porencephalic cyst without hydrocephalus. On 10/10, he had a seizure and was treated with Phenobarbital. Interictal EEG showed asymmetric slowing (L>R) and bilateral temporoparietal sharp waves (L>R). He remained seizure-free until about 2 weeks before his death when his course deteriorated again. About two weeks prior to death, he became febrile, more somnolent, had several seizures (Rx with higher doses of Phenobarbital), and developed signs of progressive liver disease. There was concern about the possibility of sepsis vs. meningitis vs. GI infection superimposed on portal hypertension. CSF couldn’t be obtained and he was empirically covered. In the last few days, he had increasing spells of bradycardia and desaturations. The family decided on redirection for comfort. ---- The weight of the unfixed brain is 500 g (normal = 665 ± 77 g) at autopsy which showed diffuse chronic hypoxic/ischemic injury manifest as microencephaly, status marmoratus of the thalamus, central lobular sclerosis of the cerebellum, diffuse white matter gliosis, hepatic encephalopathy, and a remote periventricular hemorrhage with periventricular cyst formation
