Congenital Cytomegalovirus Infection and Brain Clefting

Abstract Background Human cytomegalovirus, a major cause of permanent neurodevelopmental disability in children, frequently produces intracranial abnormalities, including calcifications and polymicrogyria, in infants with congenital cytomegalovirus infections. This report describes the features of cerebral cortical clefting, including schizencephaly, in children with congenital cytomegalovirus infection. Methods This is a retrospective review of the medical records of infants and children with congenital cytomegalovirus infection evaluated at Primary Children's Medical Center, Salt Lake City, Utah, between 1999 and 2008. Findings Twenty-five children with congenital cytomegalovirus infection were identified during this 10-year period; 23 (92%) had computed tomography and 17 (68%) had magnetic resonance imaging. Imaging was obtained at a median age of 6 months (mode 1 month or less). Of 15 children with confirmed congenital infections, 10 (66%) had polymicrogyria or abnormal gyral patterns, five (33%) had cleft cortical dysplasia, and two (13%) had schizencephaly. Of 10 children with suspected congenital cytomegalovirus infection, eight (80%) had polymicrogyria, two (20%) had cleft cortical dysplasia, and one (10%) had bilateral schizencephaly with calcifications. Seventeen of the 25 infants (68%) had intracranial calcifications. Interpretation These results indicate that clefting, either as cleft cortical dysplasia or schizencephaly, is an important feature of congenital cytomegalovirus infection.