Cytomegalovirus retinitis and optic neuritis in a child with severe combined immunodeficiency syndrome

Barbara A. Perren, Jack Raisanen, William V. Good, J. Brooks Crawford

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

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. Conclusion: The histologic features resembled those of CMV retinitis and optic neuritis in AIDS.

Original languageEnglish (US)
Pages (from-to)117-121
Number of pages5
JournalRetina
Volume16
Issue number2
DOIs
StatePublished - 1996

Keywords

  • allogeneic bone marrow transplant
  • cytomegalovirus optic neuritis
  • cytomegalovirus retinitis
  • histologic examination
  • severe combined immunodeficiency syndrome

ASJC Scopus subject areas

  • Ophthalmology

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