Aicardi-like chorioretinitis and maldevelopment of the corpus callosum in congenital lymphocytic choriomeningitis virus

Lymphocytic choriomeningitis virus (LCMV) is a rodent-borne arenavirus that can cause congenital infections similar to cytomegalovirus (CMV) or toxoplasmosis in liveborn infants. Congenital LCMV infection causes hydrocephalus, chorioretinitis, and neurodevelopmental abnormalities. For this reason, it should be added to the list of congenital infections subsumed currently under the TORCH acronym (toxoplasmosis, rubella, cytomegalovirus, and herpes simplex). Only 49 cases of congenital LCMV infections have been described worldwide since 1955, but a lack of recognition of the disorder may account for the low prevalence. The chorioretinopathy of LCMV is noteworthy for lacunar features, which may resemble the chorioretinal lacunae of Aicardi syndrome. LCMV has been distinguishable from Aicardi syndrome by 2 findings: (1) liveborn infants with LCMV may be boys or girls, whereas infants with Aicardi syndrome are girls (it is lethal in boys) and (2) infants with LCMV are reported to have a normal corpus callosum, whereas infants with Aicardi syndrome have callosal agenesis. We report here a boy with congenital LCMV infection, lacunar chorioretinopathy, and callosal agenesis. Our findings indicate that screening for congenital LCMV infection is advisable in infants with the features of Aicardi syndrome.