Case 2: Asymmetrical Frontal Bossing and Refractory Seizures in a Newborn

A male infant is born at 37 6/7 weeks' gestation by repeat cesarean delivery to a 33-year-old gravida 2, para 0 mother who had a previous fetal demise at 37 weeks' gestation (etiology unknown; no autopsy performed). For this current pregnancy the mother had regular prenatal care. Gestational diabetes was treated with dietary interventions. The mother is deaf due to childhood meningitis. She has a history of genital herpes simplex virus (HSV), with no active lesions at the time of delivery while receiving suppressive valacyclovir. The delivery is complicated by a nuchal cord and difficult extraction. The infant requires brief positive pressure ventilation. Apgar scores of 8 and 9 at 1 and 5 minutes, respectively, are given. The infant weighs 3,735 g and is noted to have macrocephaly, with a head circumference of 15.8 in (40 cm). His length is 19.7 in (50 cm) and plots at 52%. He has a large open anterior fontanelle and asymmetrical frontal bossing, with prominence of the right forehead. The remainder of his newborn examination findings are normal. At 2 hours after birth he develops intermittent oxygen desaturations associated with arching of his back followed by right-sided shaking. His blood sugar level is 78 mg/dL (4.3 mmol/L). He is placed on 1-L nasal cannula oxygen and is loaded with 20 mg/kg of phenobarbital for suspected seizures. A blood culture is performed, and he is started on ampicillin, gentamicin, and acyclovir. A normal saline fluid bolus is given, and maintenance fluids are initiated at 80 mL/kg per day. The infant is then transferred to a facility that provides a higher level of care. On arrival at our facility he is noted to be hypotonic and requires nasal cannula oxygen at 2 L. The results of his complete blood cell count and comprehensive metabolic panel are …