BASICS

DESCRIPTION

Neuroretinitis is a unilateral, or more rarely, bilateral inflammation of the optic nerve and retina with macular exudate formation and associated visual loss. Originally termed “Leber’s idiopathic stellate Neuroretinitis,” it is now known to have infectious etiologies. Although the most common association in the US is with cat scratch disease, various other causes need to be completely investigated as multiple infectious and inflammatory etiologies exist (1)[A].

EPIDEMIOLOGY

Incidence

• 6 per 100,000 secondary to cat scratch disease

• Cat exposure is seen in 90% of patients who are serology positive for Bartonella henselae.

Prevalence

• Exact prevalence remains unknown

• Affects all ages

• No sex predilection

• Right and left eyes equally affected

RISK FACTORS

• May follow a recent upper respiratory viral illness in up to 50% of cases

• Inquire about any recent exposure to cats

• Important to take a sexually transmitted disease history

GENERAL PREVENTION

As disease is idiopathic or secondary to infectious etiology, no general prevention is known.

PATHOPHYSIOLOGY

• Optic nerve edema

• Macular edema with star formation

ETIOLOGY

• Infectious or immune mediated

• May be viral in up to 50% of patients

COMMONLY ASSOCIATED CONDITIONS

Bartonella henselae infection

DIAGNOSIS

HISTORY

• Blurred vision. Visual acuity can range from 20/20 to light perception.

• Usually painless

• Flu-like symptoms

• Regional lymphadenopathy

• Ocular erythema

• Decreased visual field

PHYSICAL EXAM

• Optic disc edema (if bilateral, may be papilledema and neuroimaging and LP may be needed to rule out compressive lesion and/or elevated intracranial pressure).

• Macular star formation (usually starts to appear 2–4 weeks after visual symptoms)

• Regional lymphadenopathy

• Afferent pupillary defect

• Visual field defect (most common is cecocentral, however, arcuate and altitudinal defects can be seen)

• Acquired dyschromatopsia

• Small chorioretinal lesions

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• MRI brain and orbits with and without gadolinium

• Bartonella henselae titer

• CBC

• ESR

• ANA

• ACE

• RPR

• Lyme titer

• HIV

• Toxoplasmosis

• Toxocariasis

• Hepatitis B & C antibodies

• Brucellosis

• Epstein Barr titers

• TB skin test

• Leptospirosis

Imaging

Initial approach

• Visual field with central or cecocentral defect

• Fluorescein angiogram shows leakage of disc vessels and late disc staining

• MRI brain and orbits to rule out a compressive lesion

Follow-up & special considerations

Macular exudates usually resolve within a few months but may be present for up to a year.

DIFFERENTIAL DIAGNOSIS

• Cat scratch disease

• Lyme disease

• Lupus

• Sarcoidosis

• Syphilis

• Toxoplasmosis

• Toxocariasis

• Hypertensive retinopathy

• Tuberculosis

• Pseudotumor Cerebri

• Leukemia

• HIV

TREATMENT

MEDICATION

First Line

• Continues to be controversial. Usually a benign self-limited condition. Medical therapies may shorten the recovery time.

• All immunocompromised individuals should be treated (A)[2].

• Treat accordingly if any serologic tests are positive.

Second Line

• Doxycycline (3)[A]

• Rifampin

• Azithromycin

• Ciprofloxacin

• Trimethoprim-sulfamethoxazole

ADDITIONAL TREATMENT

Issues for Referral

• Should be evaluated by an ophthalmologist

• Consider neuroophthalmologist and/or retinal specialist.

IN-PATIENT CONSIDERATIONS

Admission Criteria

Treated as an outpatient

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient should be seen every 2 weeks for the first 2 months to assess any change in visual function (visual acuity, visual field, color vision, and any change in optic nerve and/or retinal edema)

DIET

Regular diet

PATIENT EDUCATION

If patient is Bartonella positive, education regarding cat handling should be suggested.

PROGNOSIS

• Self-limited in immunocompetent individuals

• Good prognosis

COMPLICATIONS

• Permanent decrease in visual acuity and visual field

• Optic atrophy

• Metamorphopsia

• Dyschromatopsia

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