symptoms and include retinochoroiditis (Fig 28-18), optic nerve anomalies, microphthalmos, 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/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.

Figure 28-18 Active cytomegalovirus retinochoroiditis in a premature infant, right eye.

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. The use of intraocular ganciclovir implants has not been extensively studied in children.

Herpes Simplex Virus

Herpes simplex virus (HSV) is a member of the herpesvirus family, which includes 2 types of simplex virus (HSV-1 and HSV-2), herpes zoster, Epstein-Barr virus, and CMV. Neonatal HSV infection occurs in 1 in 3000 to 20,000 births and is usually secondary to HSV-2. HSV-1 typically affects the eyes, skin, and mouth region and is transmitted by close personal contact. HSV-2 is typically associated with genital infection through venereal transmission.

Congenital HSV infection is usually acquired during passage through an infected birth canal. The neonatal infection is confined to the CNS, skin, oral cavity, and eyes in one-third of cases. It commonly manifests with vesicular skin lesions, ulcerative mouth sores, and keratoconjunctivitis. Disseminated disease occurs in two-thirds of cases and can involve the liver, adrenal glands, and lungs. Eye involvement in congenital infection can include conjunctivitis, keratitis, retinochoroiditis, and cataracts. Keratitis can be epithelial or stromal. Retinal involvement can be severe and may include massive exudates and retinal necrosis. Affected infants are treated with systemic acyclovir. The mortality rate from disseminated disease is significant, and survivors usually have permanent ocular and CNS impairment.