Neuroretinitis

Description

• Neuroretinitis is an acute inflammatory condition of the retina defined by optic disc edema and stellate maculopathy.
  • A characteristic pattern of macular exudates surrounds the fovea.
  • Vitreous cells are common.
• Although many cases remain idiopathic, a broad list of infectious and autoimmune conditions has been associated.
• Various forms of neuroretinitis include the following:
  • Leber idiopathic stellate neuroretinitis
  • Diffuse unilateral subacute neuroretinitis
  • Idiopathic retinitis
  • Vasculitis
  • Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN)
  • Recurrent neuroretinitis

Epidemiology

• No gender predilection
• Ages have been reported from 8 to 55 years, with an average of 28 years.
• Epidemiologic data are limited for neuroretinitis.

Incidence
Unknown

Prevalence
Unknown

Etiology and Pathophysiology

• The underlying pathophysiology involves increased permeability of disc vasculature, but the exact etiology is not fully defined.
  • Autoimmune
  • Infectious
• Optic disc edema is thought to be from the leakage of vessels within the optic nerve as demonstrated by fluorescein angiography (1).
• Juxtapapillary leakage may contribute to focal detachment and exudates.
• Exudates within the outer plexiform layer form the characteristic stellate pattern (2). These exudates get reabsorbed by macrophages.
• Idiopathic (up to 50% of cases)
• Various bacterial, viral, protozoal, and parasitic causes have been described or suspected (2).
  • More commonly described are Bartonella henselae, Rickettsia typhi, Mycobacterium tuberculosis, Borrelia burgdorferi, Treponema pallidum, Leptospira spp., Toxoplasma gondii, HIV, herpes simplex virus, herpes zoster virus, Epstein-Barr virus, hepatitis B, hepatitis C, mumps, coxsackie B, Toxocara canis, Histoplasma capsulatum, Brucella spp.
• Autoimmune conditions include antiphospholipid syndrome and sarcoidosis.

Genetics
No known genetic component

Risk Factors

Varies by underlying etiology

General Prevention

Treat any identified underlying etiologies.

Commonly Associated Conditions

• Infectious and autoimmune disorders
• Variations include Leber idiopathic stellate neuroretinitis, diffuse unilateral subacute neuroretinitis (associated with nematode infection), idiopathic retinitis, vasculitis, IRVAN, and recurrent neuroretinitis.