Cytomegalovirus retinitis in immunosuppressed children.

PURPOSE: To describe the ocular and systemic features of children with cytomegalovirus retinitis and their disease outcomes.

METHODS: Review of all cases of cytomegalovirus retinitis diagnosed or treated at a tertiary care pediatric hospital during a 10-year period.

RESULTS: Nine immunocompromised children younger than 16 years were diagnosed as having cytomegalovirus retinitis. The underlying causes of immunocompromise were severe combined immunodeficiency syndrome (n = 2), severe combined immunodeficiency syndrome after bone marrow transplantation (n = 1), acquired immunodeficiency syndrome (AIDS) (n = 2), AIDS and previous bone marrow transplantation for leukemia (n = 1), immunosuppressive therapy after renal transplantation (n = 1), chemotherapy for leukemia (n = 1), and congenital cytomegalovirus infection (n = 1). Five children (56%) had symptomatic extraocular cytomegalovirus infection. Only two children reported visual symptoms with cytomegalovirus retinitis at initial examination. Cytomegalovirus retinitis was bilateral in eight children (89%) and involved the posterior pole in at least one eye of all nine children. Four children (44%) died within 10 months of being diagnosed with cytomegalovirus retinitis. The remaining five children were alive, with follow-up ranging from 14 to 70 months. Successful bone marrow transplantation in one child and discontinuation of immunosuppressive medications in two children improved systemic immune function and permitted discontinuation of anticytomegaloviral therapy.

CONCLUSION: Pediatric cytomegalovirus retinitis is often asymptomatic and bilateral and involves the posterior pole at initial examination. Recovery of systemic immune function may occur in some children. Evaluation of children at risk and prompt treatment of cytomegalo. virus retinitis are important to prevent long-term visual morbidity.