Hydrocephalus and macrocephaly: New manifestations of neonatal lupus erythematosus

Objective. Neurologic involvement has been reported to occur in infants with neonatal lupus erythematosus (NLE), although the significance of these findings is unknown. The purpose of this study was to determine if hydrocephalus/ macrocephaly is a manifestation of NLE. Methods. Infants from the Hospital for Sick Children in Toronto, Canada whose mothers had anti-Ro antibodies were followed prospectively. A total of 87 infants were seen in the study period (1999-2004). The maternal autoantibody status of all infants was documented, and all infants underwent full clinical examination at each visit. Results. Of the 87 infants, 47 had NLE. Five of the 47 infants with NLE and 2 of the healthy infants had hydrocephalus, resulting in a prevalence of 8.0% (95% confidence interval 4.0-15.0%) in the entire cohort. This is significantly higher than the prevalence in the general population of 0.048-0.081%. Head circumference measurements of infants in the Toronto cohort were largest between 12-24 months of age (Z score difference = 0.71, P = 0.008). Conclusion. We suggest that hydrocephalus and macrocephaly are manifestations of NLE and that infants born to mothers with anti-Ro antibodies should be carefully monitored for hydrocephalus as part of their routine physical examination.