CYTOMEGALOVIRUS RETINITIS AND OPTIC NEURITIS IN A CHILD WITH SEVERE COMBINED IMMUNODEFICIENCY SYNDROME

Background: Although cytomegalovirus (CMV) infection of the retina and brain is common in patients with acquired immunodeficiency syndrome ( AIDS), it is exceedingly rare in patients with immunodeficiencies due to other causes. This is the first report on ocular and cerebral histo pathology of disseminated CMV in a child with severe combined immunode ficiency syndrome (SCID). Methods: The authors examined by routine his topathologic methods the eyes of a 2-year-old white boy with SCID and bilateral CMV retinitis who died after failure of a third attempt at a llogeneic bone marrow transplantation (BMT). Results: Cytomegalovirus inclusions were found in the necrotic retinal remnants, in the hyperpl astic and scarred retinal pigment epithelium, and bilaterally in the o ptic nerves. There were infiltrates of macrophages in response to the infection or the infused silicon, but no lymphoid infiltrates. Cytomeg alovirus inclusions also were found in brain tissue. Conclusion: The h istologic features resembled those of CMV retinitis and optic neuritis in AIDS.