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Washington University Experience | VASCULAR | Hypoxia-Ischemia, fetal-neonatal | White Matter | 18A0 Case 18 History
Case 18 History ---- The patient was an 11-day old infant girl born at an estimated gestational age of 38 and 4/7 weeks to a G1P1 mother on 9/10. The pregnancy was complicated by gestational diabetes (well controlled) and chronic hypertension. Prenatal testing was unremarkable up until 1 week prior to delivery when there was concern for low amniotic fluid. The patient was delivered by vertex vaginal delivery using vacuum extraction and thin meconium stained amniotic fluid was noted. A nuchal cord was wrapped around the neck, then the body, then the neck again. Apgar scores were 5 (1 min) and 8 (5 min). There was no immediate respiratory effort at delivery and the heart rate remained > 100. The patient was intubated with an endotracheal tube and thin meconium-stained mucus was suctioned from the posterior oropharynx. The ET tube was removed and no cry was noted. She then received bag-mask ventilation for approximately 15 to 30 seconds. Following birth the patient had tachypnea, and had episodes of gagging/choking on secretions. The patient was started on multiple antibiotics due to concern for sepsis and her persistent tachypnea. She was also noted to have frequent episodes of bradycardia and oxygen desaturation with occasional episodes of apnea. Many of these episodes required stimulation and lasted 10-20 seconds and produced cyanosis. She had 22 episodes from 9/12 to 9/13. The patient was given phenobarbital and phenytoin with some clinical improvement; however, the patient continued to have random multi-focal jerking and some posturing. Cranial ultrasound performed on 9/13 showed increased echogenicity in the frontal and parietal white matter. The corpus callosum appeared decreased in size. MRI was performed and showed bilateral watershed infarcts in the frontal and parietal lobes. There were also areas of infarction in the occipital lobes, right greater than left and in the periventricular white matter adjacent to the temporal horns of both right and left lateral ventricles. EEG showed frequent seizure activities from various areas of the brain, but were predominantly from the right posterior region. There was also a small intradural subdural hematoma. In addition, the patient was hyponatremic (Na=120-122 mmoL/L) on 9/13. Her seizure activities did not improve despite anti-seizure medications. She was transferred to SLCH where she was intubated. EEG performed at SLCH on 9/14 showed nonconvulsive status epilepticus with the presence of 16 electrographic seizures. MRI of the brain (9/17) showed bilateral border zone diffusion abnormality consistent with ischemia, a thin and underdeveloped corpus callosum, a small amount of blood along the right tentorium and left posterior fossa, and an underdeveloped right sylvian fissure with a focal area of prominent subarachnoid space, but no definitive cortical dysplasia. Multiple ancillary genetic and molecular studies were performed, all were negative; karyotyping. Subsequent cytogenetics were of a normal female (i.e. 46, XX). Despite the use of multiple anti-seizure medications and additional treatments she continued to have subclinical seizure events. The family decided to redirect care and the child passed on 9/21. ---- An autopsy showed an immature brain (390 g) with severe hypoxic-ischemic injury involving cortex, white matter, and deep grey nuclei composed of white matter with multiple microinfarcts and marked gliosis (periventricular leukomalacia), areas of polymicrogyria and multiple CNS infarcts, with thinned corpus callosum. The majority of the hypoxic changes show evidence of organization and likely occurred prior to birth.
