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The progressive outer retinal necrosis syndrome

Abstract

The progressive outer retinal necrosis (PORN) syndrome is a recently described clinical variant of necrotizing herpetic retinopathy in patients with the acquired immunodeficiency syndrome (AIDS). It is caused by varicellazoster virus infection of the retina. Its course and clinical features distinguish it from the acute retinal necrosis syndrome and CMV retinopathy. Early disease is characterized by multifocal deep retinal opacification. Lesions rapidly coalesce and progress to total retinal necrosis over a short period of time. Despite aggressive therapy with intravenous antivirial drugs, prognosis is poor; disease progression and/or recurrence is common, and the majority of patients develop no light perception vision. Total retinal detachments are common. Prophylaxis against retinal detachment using laser retinopexy has not been useful in most cases. PORN syndrome is an uncommon, but devastating complication of AIDS.

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References

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    Forster DJ, Dugel PU, Frangieh OT et al. Rapidly progressive outer retinal necrosis in the acquired immunodeficiency syndrome. Am J Ophthalmol 1990; 110: 341.

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    Margolis TP, Lowder CY, Holland GN et al. Varicella-zoster virus retinitis in patients with the acquired immunodeficiency syndrome. Am J Ophthalmol 1991; 112: 119.

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    Engstrom RE, Holland GN, Margolis TP, Muccioli C, Lindley JI, Belfort R, Holland SP, Johnston WH, Wolitz RA, Kreiger AE. The progressive outer retinal necrosis syndrome: A variant of necrotizing herpetic retinopathy in patients with AIDS. Ophthalmol, in press.

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Holland, G.N. The progressive outer retinal necrosis syndrome. Int Ophthalmol 18, 163–165 (1994). https://doi.org/10.1007/BF00915966

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Key words

  • acquired immunodeficiency syndrome
  • AIDS
  • progressive outer retinal necrosis syndrome
  • retinitis
  • varicella-zoster virus