Case 1: Term Infant with Intractable Seizures and Bilateral Hydronephrosis

Presentation

A male neonate is born via vaginal delivery at 37 1/7 weeks’ gestation to a healthy 30-year-old gravida 3, para 2 mother. The pregnancy had been complicated by polyhydramnios, severe fetal hydronephrosis, anti-Kell antibodies, and questionable fetal mega cisterna magna. Labor is induced secondary to worsening bilateral fetal hydronephrosis. The delivery is uncomplicated and Apgar scores are 9 and 9 at 1 and 5 minutes, respectively. Initial physical examination findings are significant only for left-sided club foot, and the infant is admitted to the well-newborn nursery. State newborn screening is performed 24 hours after birth, results of which are normal.

There is no significant familial history of congenital neurologic or urologic abnormalities, and both previous children of these parents are healthy. Amniocentesis performed during this pregnancy secondary to the bilateral fetal hydronephrosis had revealed a normal fetal microarray.

The infant is admitted to the NICU 3 days after birth secondary to hypernatremia (150 mEq/L [150 mmol/L]) and severe (grade 4) bilateral hydronephrosis confirmed with postnatal ultrasonography. While in the NICU, the neonate is noted to have episodes of lateral eye deviation, bilateral arm and leg twitching, and episodes of apnea. Electrolytes (excluding hypernatremia), glucose, and cranial ultrasonography findings are normal. A sepsis evaluation is completed and the infant is treated for 48 hours with ampicillin, cefotaxime, and acyclovir until blood, urine, and cerebrospinal fluid cultures return negative. Amplitude-integrated electroencephalography (EEG) findings are concerning for seizure activity and video EEG shows frequent multifocal epileptiform activity in both hemispheres indicative of abnormal cortical hyperexcitability, lowering the threshold for seizures. Following consultation with pediatric neurology he receives a loading dose of levetiracetam and maintenance therapy is initiated. Brain magnetic resonance imaging (MRI) reveals trace right occipital subarachnoid hemorrhage and trace infratentorial posterior fossa subdural hematoma thought to be secondary to birth trauma and …