-Defined as inflammation of the anterior optic nerve and peripapillary retina
-It presents as a triad of vision loss, optic disc swelling, and macular exudates in the formation of a star
-It is a term given to the appearance but does not indicate a specific etiology
-It is broadly categorized as idiopathic, idiopathic-recurrent, and cat scratch-disease neuroretinitis (CSD-NR)
-Neuroretinitis may also be categorized based upon the etiology: infectious vs. non-infectious
-Idiopathic and idiopathic-recurrent neuroretinitis are usually non-infectious
-Usually unilateral
ETIOLOGY:
- Cat-scratch disease (by Bartonella henselae bacteria) : Most common
Other infectious causes:
-Lyme disease, Syphilis, TB, Salmonella, Varicella, Herpes simplex and zoster, Measles, Mumps, Rubella, Influenza, Hepatitis, EBV, Histoplasmosis, Toxoplasmosis, Toxocariasis, Leptospirosis
Inflammatory causes:
-Sarcoidosis, SLE, Behcet's, PAN, Takasayu's arteritis, VKH
PATHOPHYSIOLOGY:
-A study of disease progression in one patient with neuroretinitis used FFA and OCT to demonstrate the development of fluid spaces within the OPL in the peripapillary region
-The aqueous phase of the fluid then passes through the ELM to collect in the subretinal space
-The edema gradually resolves to leave lipid-rich exudates within OPL, which appear in a stellate formation due to the radial arrangement of fibers within this layer
-The mechanism of inflammation and vasculitis is unknown, but is generally thought to be due to either direct infection or an autoimmune process
-In cat-scratch disease- direct vascular invasion method is thought to predominate
HISTORY:
-Unilateral/bilateral loss of vision
-The pattern of visual field loss varies greatly but is generally central or ceco-central
-Any preceding flu-like prodrome, risk factors for Lyme disease (tick bite and erythema migrans), sexually transmitted infections, risk factors for TB
SIGNS:
-RAPD
-Disc swelling with/without flame hemorrhages at disc
-Retinal vascular occlusion
-SRF in peripapillary region
-Fluid from the optic disc tracks directly into the ONL-OPL layers of the retina and accumulates in the subretinal space giving rise to a focal macular detachment of the neurosensory retina
-With the resolution of the disc edema and retinal/subretinal fluid, lipid-rich exudates are left behind in a stellate formation- seen after around 3 weeks
EVALUATION:
-Lab tests - for syphilis and TB testing, Bartonella and Borrelia serology
-OCT - to identify SRF and intraretinal edema
-Findings of flattening of the foveal contour, fluid within the OPL, or SRF with early intraretinal exudates can suggest the diagnosis of neuroretinitis before the appearance of a macular star
-FFA - to determine the site of leakage, identify other less visible retinal and retinal vascular pathologies such as vasculitis, occlusion, and document diabetic or hypertensive retinopathy
-With neuroretinitis, leakage should begin in the optic disc and maybe segmental
-MRI - often normal. May show enhancement of optic disc and extend a few millimeters posteriorly within intraorbital portion of optic nerve
MANAGEMENT:
-To treat systemic cat-scratch disease, presenting with fever and lymphadenopathy, a prospective randomized clinical trial showed that a course of azithromycin more rapidly reduces lymph node volume than without treatment
-Antibiotics with steroids
-For patients with severe vision loss or moderate to severe systemic, systemic symptoms, treatment with doxycycline or azithromycin with rifampin for a duration of 4-6 weeks may provide benefit
-For recurrent cases of neuroretinitis, long-term immunosuppression with azithromycin
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