Bilateral Acute Retinal Necrosis with Concurrent Retinopathy of Prematurity in Two Neonates

Acute retinal necrosis (ARN) is a syndrome characterized by one or more areas of necrotizing retinitis with adjacent occlusive retinal vasculitis involving both arteries and veins. Additional signs often include vitritis and optic neuropathy. 1 Since acute retinal necrosis is predominantly seen in the adult population, literature on neonatal cases is exceedingly rare. We present two cases of premature neonates who developed ARN with concurrent retinopathy of prematurity (ROP.) A 985-g male was born at 27 weeks gestational age via vaginal delivery secondary to preterm labor to a mother with an unremarkable past medical history with no evidence of herpetic infection. The neonate was in respiratory distress at birth with later radiographic support of respiratory distress syndrome. He was intubated and given surfactant at birth; he was extubated to continuous positive airway pressure (CPAP) at approximately 9 hours of life. The infant was evaluated in the neonatal intensive care unit (NICU) for ROP at 32 weeks gestational age. At that time, he was found to have peripheral cataracts and chorioretinitis bilaterally. He did not have ROP on initial evaluation. A TORCH workup (toxoplasmosis, ‘‘other’’ infections, rubella, cytomegalovirus, and herpes simplex virus) was initiated. Polymerase chain reaction (PCR) of his cerebral spinal fluid (CSF) was positive for herpes simplex virus (HSV) type 2, and negative for cytomegalovirus (CMV), toxoplasmosis, syphilis, human immunodeficiency virus (HIV), varicella zoster virus (VZV), and rubella. Despite treatment with intravenous acyclovir (20 mg/kg daily), chorioretinitis progressed to bilateral acute retinal necrosis and he developed stage 2 zone II ROP. No treatment for ROP was required, but laser demarcation of the necrotic retina was performed. Weekly follow-up exams were done while the patient was in the NICU until after the laser demarcation was done and retinal findings were stable. Intravenous acyclovir was increased to 20 mg/kg TID. After 3 weeks at this higher dosage, the ARN was stable and a repeat CSF PCR was negative for HSV type 2 and treatment was changed to oral acyclovir (20 mg/kg BID) on which he remains