Chorioretinal Scars in Congenital Cytomegalovirus Infection Following Prenatal Valacyclovir Therapy

BACKGROUND: Congenital cytomegalovirus infection is the most common congenital infection. Ocular abnormalities affect approximately 14% of symptomatic children, but fewer than 1% of asymptomatic children. Prenatal valacyclovir reduces vertical transmission, but ophthalmic outcomes in exposed infants are poorly described. METHODS: We describe 2 neonates with congenital cytomegalovirus confirmed by amniotic fluid and urine polymerase chain reaction following first-trimester maternal primary infection. Both mothers received high-dose valacyclovir (8 g daily) from 13 weeks' gestation. Infants underwent neonatal evaluation, including dilated fundus examination and multimodal retinal imaging. RESULTS: Maternal treatment continued to 36 weeks in 1 pregnancy and was discontinued at 22 weeks in the other, after amniocentesis. Both infants had macular chorioretinal scarring. Optical coherence tomography demonstrated focal ellipsoid zone disruption in a clinically normal fellow eye in 1 infant. Cranial ultrasonography demonstrated caudothalamic and temporal lobe cysts with lenticulostriate vasculopathy in 1 infant and isolated lenticulostriate vasculopathy in the other. Physical and neurologic examinations, head circumference, full blood count, liver function and auditory brainstem responses were normal in both. Both were classified as symptomatic with central nervous system involvement on the basis of ocular findings and therefore received valganciclovir for a planned 6 months. Retinal findings were stable at 3 months. CONCLUSIONS: Macular chorioretinal scarring was present in both infants despite otherwise limited clinical findings, and the extent of cranial imaging abnormality did not correspond to ocular involvement. Ophthalmologic examination determined disease classification and management. These observations do not permit conclusions regarding the effect of prenatal valacyclovir on ocular outcome.