CASE REPORT
ِِA 25-year-old man was referred to an ophthalmology clinic due to vision loss in his left eye. He has no past medical or ocular history, and he is not taking any medications. Additionally, he is a non-smoker and drinks alcohol socially.
The patient reported waking up with vision loss in his left eye five days ago and sought medical attention at an urgent care center, where he was then referred to ophthalmology. He denies experiencing eye pain or pain with eye movements but has been feeling fatigued, and tired, and developed a low-grade fever over the past few days.
During the ophthalmological examination, his visual acuity was 20/20 in the right eye, and counting fingers in the left eye. Pupils were equal, but a left relative afferent pupillary defect (RAPD) was observed.
Color vision using Ishihara color plates was normal in the right eye (14/14) but impaired in the left eye (0/14). Ocular motility and alignment were found to be normal, and the slit lamp examination did not reveal any abnormalities. However, the left eye exhibited 1/2+ cells in the vitreous.
The neurological examination yielded normal results. Despite these findings, the patient’s examination is considered atypical for neuroretinitis due to the significant optic disc edema and subretinal fluid in the macula observed in the left eye.
ًWhat is Neuroretinitis?
Neuroretinitis is an inflammation of the neural retina and optic nerve. It was originally described by Leber in 1916 as a “stellate maculopathy,” but this definition was challenged by Don Gass in 1977, citing that disc edema precedes macular exudates.
Subsequently, Gass confirmed optic disc leakage by fluorescein angiography and suggested the term “neuroretinitis.” More recent retinal and optic nerve imaging has supported Gass’ description.
Risk factors for neuroretinitis relate to susceptibility to each particular causative agent. Immunocompromised patients from chronic disease, HIV/AIDS, medications, health care workers, recent immigrants, or those with recent travel to endemic areas are all high-risk populations.
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From Choroida — the team behind this siteNeuroretinitis MANAGEMENT
General treatment
The treatment of neuroretinitis is directed at the underlying etiology. Suspicion for infectious etiology, particularly tuberculosis, may merit consultation with an infectious disease specialist.
If the causative agent is found to be cat-scratch disease, various treatment methods are found in the literature, including no treatment, antibiotics only, antibiotics and steroids, and steroids only.
As a high degree of spontaneous visual recovery exists in cat-scratch disease neuroretinitis, definite conclusions cannot be made regarding antibiotic efficacy.
If an infectious etiology is suspected, an appropriate workup with broad-spectrum antibiotic treatment, while results are pending, is appropriate. Recommended antibiotics are ciprofloxacin or azithromycin for adults and azithromycin or sulfamethoxazole-trimethoprim for children.
For the idiopathic variety, high-dose oral corticosteroids have been administered. Antibiotics may be considered to cover cat-scratch disease while serologies are pending.
Medical follow up
If an infectious etiology is suspected but serologies are negative, retesting at six weeks for rising IgG titers is appropriate.
Neuroretinitis Complications
Complications stem from side effects from pharmacologic therapy or chronic visual loss from recurrence. Anti-tuberculous treatments are well-known for ocular complications, including retrobulbar neuritis with ethambutol and significant anterior uveitis, and hypopyon with rifabutin therapy.
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Two smartphone imaging tools built for everyday clinic use — one for the slit lamp, one for the fundus.
From Choroida — the team behind this siteREFERENCES
- Fouch B, Coventry S. A case of fatal disseminated Bartonella henselae infection (cat-scratch disease) with encephalitis. Arch Pathol Lab Med. 2007;131(10):1591-4.
- Ghauri R, Lee A. Optic Disk Edema With a Macular Star. Survey of Ophthalmology. 43(3): 270-4, 1998.
- Purvin VA, Chioran G. Recurrent neuroretinitis. Arch Ophthalmol. 1994;112:365-71.
- Purvin V, Sundaram S, Kawasaki A. Neuroretinitis: Review of the Literature and New Observations. J Neuro-Ophthalmology. 2011;31: 58-68.
- Vaphiades MS. Rocky Mountain Spotted Fever as a cause of macular star figure. Neuroophthalmol. 2003;23(4):276-8.
- Vaphiades MS, Wigton EH, Ameri H, Lee AG. Neuroretinitis with retrobulbar involvement. J Neuroophthalmol. 2011;31(1):12-5.
Test yourself
A few questions straight from this article.
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Neuroretinitis is best described as inflammation of which structures?
Neuroretinitis is an inflammatory process involving both the neural retina and the optic nerve, which is why disc swelling and macular changes appear together. -
Under what name did Leber first describe neuroretinitis in 1916?
Leber described the condition in 1916 as a stellate maculopathy; Don Gass later challenged that framing in 1977 by showing that disc oedema precedes the macular exudates. -
Which imaging finding led Gass to propose the term neuroretinitis?
Gass confirmed optic disc leakage on fluorescein angiography and, on that basis, suggested the term neuroretinitis; later retinal and optic nerve imaging supported his description. -
Which group is identified as a high-risk population for neuroretinitis?
Risk relates to susceptibility to the causative organism, so immunocompromised patients, those with HIV/AIDS, health care workers, recent immigrants and recent travellers to endemic areas are high-risk. -
Suspicion of which infection in neuroretinitis particularly merits infectious disease consultation?
Treatment of neuroretinitis targets the underlying cause, and suspected infectious aetiology, tuberculosis in particular, may merit consultation with an infectious disease specialist. -
Which antibiotics are recommended for adults with suspected infectious neuroretinitis?
When an infectious cause is suspected, broad-spectrum cover is started while results are pending; ciprofloxacin or azithromycin is recommended for adults. -
Which antibiotics are recommended for children with suspected infectious neuroretinitis?
For children with suspected infectious neuroretinitis the recommended agents are azithromycin or sulfamethoxazole-trimethoprim, rather than the adult fluoroquinolone options. -
How is idiopathic neuroretinitis managed?
High-dose oral corticosteroids have been used for the idiopathic variety, and antibiotics may be added to cover cat-scratch disease while serologies are still pending. -
In suspected infectious neuroretinitis with negative serology, when should retesting be performed?
If an infectious cause is suspected but the initial serologies are negative, repeating the tests at six weeks to look for rising IgG titres is appropriate. -
Which ocular complication of anti-tuberculous therapy is attributed to ethambutol?
Anti-tuberculous drugs carry ocular complications: ethambutol is associated with retrobulbar neuritis, while rifabutin therapy can cause significant anterior uveitis with hypopyon.
