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Washington University Experience | VASCULAR | Hypoxia-Ischemia, fetal-neonatal | White Matter | 12A0 Case 12 History
Case 12 History ---- The patient was born on 5/7 to an 18-year-old G1P0, now P1, who was unaware of her pregnancy and presented to an outside hospital with concern for back pain. She was treated with Flexeril and Vicoprofen and discharged but returned several hours later in active labor, at which time, a fetal ultrasound showed a fetus thought be EGA of 33 weeks. The fetal work-up and ultrasound showed bradycardia, ascites, bilateral hydrocele, small bilateral pleural effusions and suspected cardiomegaly. Maternal serologies were reported as RPR non-reactive, HIV negative, rubella immune, hepatitis B serum antigen negative, and GBS pending. Mother denied use of alcohol. The child had a vaginal delivery with forceps and vacuum assist, a birth weight of 2540 grams (91-96th percentile), head circumference of 33 cm (91-95th percentile) and length of 45 cm (51-75th percentile). The patient was noted to have meconium aspiration syndrome with hydrops (Coombs negative). Apgars were 1, 1, and 3. Eventually high-frequency oscillator ventilation, intubation and nitric oxide were required along with surfactant x 3 and also required pressors for support. He was pancytopenic and transfused with multiple blood products, including platelets. An echocardiogram was consistent with pulmonary hypertension. He was hypoglycemic, and had elevated liver enzymes. An initial head ultrasound on 5/7/2012 showed caput succedaneum but no evidence of acute intracranial bleed. Blood cultures drawn at the outside institution showed no growth. HSV PCR was negative. Pleural fluid and tracheal aspirate also showed no growth but prophylactic ampicillin and cefotaxime were started. Placental gram stain collected on 5/7/2012 at the outside hospital showed gram-positive cocci and gram-negative bacilli; follow-up placental culture identified these organisms as Viridans Streptococcus species and Bacteroides fragilis group. Acute chorioamnionitis and acute funisitis were identified. He was transferred to SLCH to be evaluated for persistent hypoxemia and possible ECMO placement, but after arrival there was question of seizure activity and he did not qualify for ECMO placement. He was subsequently diagnosed with hypoxic ischemic encephalopathy with status epilepticus. Ultimately care was redirected. Per the clinical abstract provided at the time of postmortem examination, there was "abnormal background activity on EEG and significantly abnormal neurologic exam." Occasional eye blinking, reflex present with painful stimuli, but no purposeful movements were noted. No gag reflex was present. Pupils were reactive, but the infant was noted to have minimal respiratory effort. ---- Autopsy findings included extensive subacute hypoxic/ischemic damage, including diffuse white matter gliosis ("periventricular leukomalacia") with petechial hemorrhage, hippocampal neuron loss and astrocytosis, hippocampal CA1, CA2 and subiculum, cerebral cortical damage ranging from selective neuronal necrosis to laminar necrosis/infarction, particularly occipital cortex, pontosubicular necrosis, neuron loss, basal ganglia and thalamus with neuronal mineralization ("status marmoratus"). There is a spectrum of injury ranging from focal necrotic lesions (periventricular leukomalacia) to diffuse white matter astrogliosis (diffuse white matter gliosis) which is dependent on the gestational age or period of time of insult as well as the presence of infection/inflammation concurrently. This patient had a history of placental infection as well as extended hypoxemia even after birth.