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 azithromycin
- Children: 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.