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The Case
A 3-hour-old full-term female neonate presents with white pupils noted at the time of NICU admission.
Prenatal and Birth Histories
Born to a 34-year-old gravida 1, para 0 white woman
Pregnancy complicated by gestational hypertension, headaches, anxiety, and depression. Maternal medications included prenatal vitamins, fluoxetine, and sumatriptan
Prenatal maternal laboratory findings: Blood type B+, antibody negative, rubella immune, hepatitis B surface antigen negative, rapid plasma reagin nonreactive, HIV negative
Estimated gestational age: 37 6/7 weeks’ gestation
Vaginal delivery after the induction of labor because of maternal hypertension
Apgar score: 2, 7, and 8 at 1, 5, and 10 minutes, respectively
The infant was limp and cyanotic at delivery and required positive pressure ventilation for 5 minutes. No risk factors for sepsis were noted and there was no evidence of placental abruption. In addition, the fetal heart rate tracing was normal before delivery. The reasons for the perinatal depression at birth were unclear. Subsequently, the infant started breathing spontaneously and was transitioned to room air. Neurologic examination findings obtained at the outside facility were significant for wide-eyed appearance with staring and generalized low muscle tone.
Presentation
The infant was transferred to the NICU at 3 hours of age for therapeutic hypothermia evaluation. Initial physical examination findings were remarkable for the presence of leukocoria bilaterally. This was visualized easily with both ambient lighting in the patient room as well as direct examination with an ophthalmoscope (Fig 1). Red reflex was absent bilaterally.
Bilateral leukocoria.
The infant’s neurologic assessment findings at admission were consistent with moderate hypoxic-ischemic encephalopathy. Therapeutic whole-body cooling was initiated. An umbilical venous catheter was placed and continuous electroencephalogram obtained. The infant also received ampicillin and ceftazidime, intravenous fluids, and continuous fentanyl infusion.
Vital Signs (3 Hours of Age)
Heart rate: 115 beats/min
Respiratory rate: 40 breaths/min
Blood pressure: 80/68 mm Hg
Oxygen saturation: 100% (in room air)
Temperature: 97.7°F (36.5°C) …
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