Cytomegalovirus (CMV), a member of the herpesvirus family, is a ubiquitous virus that can cause a wide range of infection, from asymptomatic acquired infection in immunocompetent individuals to severe infections in newborn infants and immunocompromised patients. Over 80% of adults in developed countries have antibodies to the virus.
Congenital infection with CMV is the most common congenital infection in humans; it occurs in approximately 1% of infants. Clinically apparent disease is present in 10%–15% of infected neonates, and 20%–30% of these cases are fatal. Transmission to the fetus or newborn can occur transplacentally, from contact with an infected birth canal during delivery, or from ingestion of infected breast milk or maternal secretions. Congenital CMV disease is characterized by fever, jaundice, hematologic abnormalities, deafness, microcephaly, and periventricular calcifications.
Ophthalmic manifestations of congenital CMV infection occur primarily in infants with systemic symptoms and include retinochoroiditis (Fig 28-27), optic nerve anomalies, microphthalmia, cataract, and uveitis. The retinochoroiditis usually presents with bilateral focal involvement consisting of areas of RPE atrophy and whitish opacities mixed with retinal hemorrhages. The retinitis can be progressive, or it may present as a quiescent CMV chorioretinal scar that is difficult to differentiate from the scar seen in toxoplasmosis. CMV retinitis can be acquired in children who are immunocompromised (most frequently by infection with HIV or AIDS or following organ transplantation or chemotherapy). The retinitis is a diffuse retinal necrosis with areas of retinal thickening and whitening, hemorrhages, and venous sheathing. Vitritis may also be present.
Diagnosis is based on the clinical presentation in acquired disease and is supplemented by serologic testing for antibodies to CMV in congenital infection. In infected infants, the virus can be recovered from bodily secretions.
Infants with severe systemic or sight-threatening disease are usually treated with ganciclovir. Medications that are available for treatment of older immunocompromised children include ganciclovir, valganciclovir, foscarnet, cidofovir, and fomivirsen.
Figure 28-27 Active cytomegalovirus retinochoroiditis in a premature infant, right eye.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.