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Neuroretinitis

para>If a bilateral presentation is found, the differential diagnosis should expand to include increased intracranial pressure or mass effect, although there have been case reports of bilateral presentation associated with cat-scratch disease (CSD).

DIAGNOSTIC TESTS & INTERPRETATION

Directed to suspected underlying etiology

Initial Tests (lab, imaging)

Evaluation might include the following:

  • CBC
  • Angiotensin-converting enzyme
  • Antinuclear antibody
  • Antidouble-stranded DNA
  • Complement (C3)
  • Erythrocyte sedimentation rate
  • Fluorescent treponemal antibody absorption
  • Serologies for viral, fungal, and bacterial etiologies including B. henselae
  • Enzyme-linked immunosorbent assay for T. canis
  • Tuberculin skin test
  • MRI of the head and orbits

Follow-Up Tests & Special Considerations

  • The stellate macular appearance may develop subsequently to the optic nerve involvement and should be monitored in the 1st weeks after initial presentation.
  • Rarely, a CSF evaluation may be indicated.

Diagnostic Procedures/Other

  • Visual field testing may show a central scotoma affecting vision in the central field or a cecocentral scotoma, which includes the blind spot and extends into the area of central fixation due to involvement of the papillomacular bundle.
  • Color vision testing
  • Fluorescein angiography
  • Optical coherence tomography
  • Diagnostic vitrectomy, when appropriate

TREATMENT

  • Treatment is directed at the underlying etiology.High-dose oral steroids are commonly used in idiopathic and recurrent neuroretinitis with occasional high-dose IV steroids used in recurrent neuroretinitis, although evidence for altering visual outcomes is lacking (3)[B].
  • Consider antibiotics for CSD while serologies are pending (4)[C].Adults: rifampin plus ciprofloxacin or azithromycinChildren: rifampin plus azithromycin or sulfamethoxazole-trimethoprim

MEDICATION

Direct to underlying etiology

ISSUES FOR REFERRAL

Refer for neurologic and ophthalmologic consultation upon initial suspicion.

ADDITIONAL THERAPIES

Laser treatment has been used for T. canis (5)[C].

SURGERY/OTHER PROCEDURES

Laser treatment can be directed at the invading organism in helminthic infections (5)[C].

INPATIENT CONSIDERATIONS

As necessary for underlying systemic/neurologic complications

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Systemic monitoring will depend on the symptoms and underlying etiology.
  • Ophthalmologic monitoring initially may be weekly to monitor for development of macular star and depending on underlying etiology and severity, followed by less-frequent monitoring until resolution of optic nerve and macular involvement is seen. Macular involvement may take longer to subside.

Patient Monitoring

Directed to underlying etiology

DIET

No specific diet

PROGNOSIS

  • Generally favorable outlook with typical reported recovery of vision to ≥20/40 within 2 months (6).
  • Initial visual acuity and lack of systemic symptoms appear to be the best predictors of good visual outcome in neuroretinitis caused by B. henselae (4).
  • Optic disc edema resolves >8 to 12 weeks (3).

COMPLICATIONS

  • Permanent vision loss
  • Retinal detachment
  • Optic atrophy
  • Retinal pigment epithelial defects

REFERENCES

11 Kitamei H, Suzuki Y, Takahashi M, et al. Retinal angiography and optical coherence tomography disclose focal optic disc vascular leakage and lipid-rich fluid accumulation within the retina in a patient with Leber idiopathic stellate neuroretinitis. J Neuroophthalmol. 2009;29(3):203 " 207.22 Ray S, Gragoudas E. Neuroretinitis. Int Ophthalmol Clin. 2001;41(1):83 " 102.33 Purvin V, Sundaram S, Kawasaki A. Neuroretinitis: review of the literature and new observations. J Neuroophthalmol. 2011;31(1):58 " 68.44 Chi SL, Stinnett S, Eggenberger E, et al. Clinical characteristics in 53 patients with cat scratch optic neuropathy. Ophthalmology. 2012;119(1):183 " 187.55 Narayan SK, Kaliaperumal S, Srinivasan R. Neuroretinitis, a great mimicker. Ann Indian Acad Neurol. 2008;11(2):109 " 113.66 Casson RJ, O 'Day J, Crompton JL. Leber 's stellate neuroretinitis: differential diagnosis and approach to management. Aust N Z J Ophthalmol. 1999;27(1):65 " 69.

ADDITIONAL READING

Spencer BRJr, Digre KB. Treatments for neuro-ophthalmologic conditions. Neurol Clin. 2010;28(4):1005 " 1035.

CODES

ICD10

  • H30.90 Unspecified chorioretinal inflammation, unspecified eye
  • H30.009 Unsp focal chorioretinal inflammation, unspecified eye
  • H30.899 Other chorioretinal inflammations, unspecified eye
  • H30.019 Focal chorioretinal inflammation, juxtapapillary, unsp eye
  • H35.069 Retinal vasculitis, unspecified eye
  • H30.92 Unspecified chorioretinal inflammation, left eye
  • H35.061 Retinal vasculitis, right eye
  • H35.063 Retinal vasculitis, bilateral
  • H30.91 Unspecified chorioretinal inflammation, right eye
  • H35.062 Retinal vasculitis, left eye
  • H30.001 Unspecified focal chorioretinal inflammation, right eye
  • H30.893 Other chorioretinal inflammations, bilateral
  • H30.93 Unspecified chorioretinal inflammation, bilateral
  • H30.002 Unspecified focal chorioretinal inflammation, left eye
  • H30.003 Unspecified focal chorioretinal inflammation, bilateral
  • H30.011 Focal chorioretinal inflammation, juxtapapillary, right eye
  • H30.012 Focal chorioretinal inflammation, juxtapapillary, left eye
  • H30.013 Focal chorioretinal inflammation, juxtapapillary, bilateral
  • H30.891 Other chorioretinal inflammations, right eye
  • H30.892 Other chorioretinal inflammations, left eye

ICD9

  • 363.05 Focal retinitis and retinochoroiditis, juxtapapillary
  • 363.00 Focal chorioretinitis, unspecified
  • 362.12 Exudative retinopathy
  • 362.18 Retinal vasculitis

SNOMED

  • neuroretinitis (disorder)
  • Focal retinitis (disorder)
  • Optic neuroretinitis
  • retinal vasculitis (disorder)
  • Juxtapapillary focal retinitis AND retinochoroiditis
  • Bartonella henselae neuroretinitis

CLINICAL PEARLS

  • For a patient with painless unilateral vision loss after a viral-like prodrome, consider neuroretinitis and refer immediately to an ophthalmologist for further evaluation including a complete eye exam with dilated funduscopy.
  • Many of the underlying etiologies can present with various eye manifestations in addition to neuroretinitis, including anterior uveitis, retinitis, chorioretinitis, posterior uveitis, optic neuritis, and endophthalmitis.