LAUSR

Cytomegalovirus (CMV), like other members of the herpes virus family, has the unique property of chronic infection, latency, and potential reactivation. Overt CMV infection occurs following primary reactivation of the latent virus or by infection with a new strain. Following primary infection, this virus remains latent in the peripheral blood mononuclear cells and bone marrow but can compartmentalize in various organs and be unique from a strain in a differing site. Risk factors for initially contracting this disease include breast feeding, blood transfusions, and close contact with colonized children. Some studies have suggested increased risk with older maternal age and multiparity. CMV is the most frequent congenital infectious agent causing newborn malformation in developed countries despite the introduction of infectious disease screening in pregnant women and is also very frequent in underdeveloped countries. The prevalence of congenital CMV is reported variably 0.2% to 2%, second only to cerebral palsy in causes of congenital malformations. Additionally, it has been documented that infants with symptomatic CMV infection have a greater incidence of long-term neurological and neurodevelopmental complications than Down syndrome or fetal alcohol syndrome. Neurologic manifestations caused by congenital CMV infection include sensorineural deafness/hearing loss, mental retardation, microcephaly, seizure disorders, and cerebral palsy. Only 10% of infected infants have signs and symptoms at birth, but between 40% and 90% of symptomatic neonates go on to develop subtle neurological consequences later. For those asymptomatic at birth, between 7% and 20% have been found to develop at least mild disabilities. Signs specifically at birth are intrauterine growth restriction, prematurity, microcephaly, jaundice, petechie, hepatosplenomegaly, periventricular calcifications, chorioretinitis, pneumonitis, hepatitis, and sensorineural hearing loss. If discovered later in childhood, patients may present with pneumonia, hepatosplenomegaly, hepatitis, thrombocytopenia, sepsis-like syndrome, and atypical lymphocytosis. Asymptomatic congenital CMV infection normally occurs when a woman already has immunity to CMV. Even though normal at birth, the risk for growth restriction and sensorineural hearing loss is still high at 15%. Evidence has shown that preventative actions, early intervention for CMV-infected children with sensorineural hearing loss, new antiviral treatments, and availability of vaccines for pregnant women and neonates are effective. In 2015, the International Congenital Cytomegalovirus Recommendations Group convened as part of the Fifth International Congenital Cytomegalovirus Conference to assess the evidence and create guidelines to direct the diagnosis, prevention, and therapy of congenital CMV infection. This group addressed diagnosis of maternal/fetal infections, education, treatment guidelines for both infants and mothers, and preventive measure (see Table 1).