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 histopathology of disseminated CMV in a child with severe combined immunodeficiency syndrome (SCID).


METHODS
The authors examined by routine histopathologic 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 allogeneic bone marrow transplantation (BMT).


RESULTS
Cytomegalovirus inclusions were found in the necrotic retinal remnants, in the hyperplastic and scarred retinal pigment epithelium, and bilaterally in the optic nerves. There were infiltrates of macrophages in response to the infection or the infused silicon, but no lymphoid infiltrates. Cytomegalovirus inclusions also were found in brain tissue.


CONCLUSIONS
The histologic features resembled those of CMV retinitis and optic neuritis in AIDS.
Perren
B A
BA
Department of Ophthalmology, University of California, San Francisco 94143-0730, USA.
Raisanen
J
J
Good
W V
WV
Crawford
J B
JB
D016026:Bone Marrow Transplantation; D001921:Brain / Q000473:pathology; D002675:Child, Preschool; D017726:Cytomegalovirus Retinitis / Q000150:complications* / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009902:Optic Neuritis / Q000150:complications* / Q000473:pathology / Q000821:virology*; D012160:Retina / Q000473:pathology; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000601:surgery