TUBERCULOSIS

Ocular tuberculosis is rare, affecting 1% to 2% of patients with active disease. However, since 1984 reported cases of tuberculosis have been increasing, making this entity important to consider in the differential diagnosis of uveitis.

Symptoms

The clinical manifestations are varied, but patients may present with blurred vision, floaters, and occasionally redness, pain, and photophobia.

Clinical Features

Tuberculosis can involve both the anterior and posterior segments of the eye as well as the ocular adnexae and orbit. The most common ocular manifestations include phlyctenular keratoconjunctivitis, interstitial keratitis, granulomatous iridocyclitis, scleritis, choroidal granulomas, posterior uveitis, and retinal vasculitis.

Ancillary Testing

Fluorescein angiography and ultrasonography can be used to evaluate choroidal granulomas. On angiography, the lesions may be hyperor hypofluorescent early; they stain late. B-scan ultrasonography typically shows an elevated mass with an absent scleral echo. A-scan ultrasonography shows low internal reflectivity. Fluorescein angiography will show staining of vessels in cases of retinal vasculitis.

Pathology/Pathogenesis

Mycobacterium tuberculosis is an acid-fast-staining obligate anaerobe with worldwide prevalence. Infection begins with inhalation of airborne organisms from patients with pulmonary tuberculosis. The majority of patients do not develop active disease, but the disease remains in a state of dormancy. Some patients develop rampant hematogenous spread resulting in miliary tuberculosis. In general, intraocular disease evolves from hematogenous spread (eg, choroidal tubercules). Surface ocular infection results from direct inoculation (eg, conjunctival nodule) or delayed hypersensitivity (eg, phlyctenule). Histopathologic examination of direct infection reveals caseating granulomas with Langerhans’ giant cells and acid-fast bacilli.

Treatment/Prognosis

A patient with suggestive ocular findings and a positive purified protein derivative (PPD) test should be treated if there is other evidence of tuberculosis. The Centers for Disease Control and Prevention recommend that all newly diagnosed patients receive a four-drug regimen

of isoniazid, rifampin, pyrazinamide, and either streptomycin or ethambutol for 6 to 9 months. Treatment should be coordinated with a specialist. Systemic corticosteroids should not be used in persons with ocular tuberculosis. Topical corticosteroids and cycloplegics may be used adjunctively.

Systemic Evaluation

Skin testing (PPD) is used to establish exposure to M tuberculosis. Because a positive skin test without active systemic disease is usually insufficient to make a presumptive diagnosis of intraocular tuberculosis, a full systemic evaluation should follow. This evaluation typically includes chest x-ray; sputum analysis; biopsy of liver, lymph nodes, or bone marrow; stool for acid-fast smear; and cultures of blood, urine, cerebrospinal fluid, or pleural fluid. It is important to rule out human immunodeficiency virus infection.

Fundus photograph montage of a patient with acquired immunodeficiency syndrome complicated by miliary tuberculosis. He had diffuse choroidal granulomas in each eye.

Higher-magnification photograph of the same patient confirms the subretinal location of the choroidal granulomas.

Vogt-Koyanagi-Harada (VKH) syndrome is a systemic inflammatory syndrome affecting primarily heavily pigmented individuals between the ages of 20 and 50 years. Asians, Spanish Americans, blacks, and Native Americans are most often affected.

Symptoms

Decreased vision may be associated with meningeal and ear symptoms, including neck stiffness, headache, tinnitus, and vertigo. Visual loss is usually bilateral but may be asymmetric. Iridocyclitis may cause photophobia, ciliary flush, and mild eye pain. Dysacusis, tinnitus, and hearing loss affect approximately 75% of patients with this disorder. Dermatologic involvement may include alopecia, poliosis, and vitiligo.

Clinical Features

Bilateral iridocyclitis with a granulomatous anterior chamber reaction is found with the initial onset of disease. Vitritis and exudative choroiditis lead to a decline in vision. Choroidal thickening is associated with overlying neurosensory retinal detachment. There are often multilobed serous detachments, which may coalesce into broad areas of serous retinal detachment. The optic nerve is hyperemic, and vitreous inflammatory cells are present.

Late in the course of disease, there may be optic disc neovascularization, choroidal neovascularization with hemorrhagic macular detachment, and depigmentation and clumping of the retinal pigment epithelium (RPE).

Ancillary Testing

Fluorescein angiography shows patchy delayed choroidal filling followed by pinpoint areas of hyperfluorescence at the level of the RPE. These pinpoint areas may leak into areas of serous retinal detachment. B-scan ultrasonography demonstrates thickening of the choroid, posterior serous retinal detachment, which may settle inferiorly, vitreous opacities, and posterior scleral thickening.

Lumbar puncture may show a cellular pleocytosis in the early stages of the disease.

Pathology/Pathogenesis

Although the pathogenesis is not completely understood, VKH syndrome is considered to be a cell-mediated autoimmune disease against melanocytes. A T-cell immune response may be directed against an antigenic component of the melanocyte, possibly tyrosinase or a tyrosinase-related protein.

Treatment/Prognosis

Corticosteroids are the first line of treatment to decrease the inflammatory disease. Systemic therapy is necessary, and the duration of treatment is often prolonged to prevent recurrence of disease. Visual outcomes with treatment are favorable. The retinal detachment usually resolves rapidly, with a slower resolution of anterior segment inflammation.

Immunosuppressive medications including cyclosporine and chlorambucil have been used when response to corticosteroids is inadequate.

Systemic Evaluation

Neurologic evaluation for associated meningeal disease is indicated. Neurologic symptoms may lead to changes in personality and even psychosis.

This patient with Vogt-Koyanagi-Harada syndrome had panuveitis with iridocyclitis, vitritis, and exudative choroiditis. Note the multilobular nature of the serous retinal detachments.

This patient with Vogt-Koyanagi-Harada syndrome has a serous neurosensory retinal detachment that is most notable superior to the optic disc.

This fundus photograph of the same eye demonstrates turbid subretinal fluid and retinal pigment epithelial alterations.

Fluorescein angiography reveals hyperfluorescence resulting from leakage in the region of the neurosensory retinal detachment.

SELECTED REFERENCES

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Acute Retinal Necrosis

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Behçet’s Disease

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Cytomegalovirus Retinitis

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Diffuse Unilateral Subacute Neuroretinitis

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Endophthalmitis

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Human Immunodeficiency Virus Retinopathy

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Intermediate Uveitis

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Multifocal Choroiditis and Panuveitis

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Multiple Evanescent White Dot Syndrome

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Neuroretinitis

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Posterior Scleritis

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Presumed Ocular Histoplasmosis Syndrome

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Sarcoidosis

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Serpiginous Choroiditis

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Syphilis

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Systemic Lupus Erythematosus

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Toxocariasis

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Toxoplasmosis

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Tuberculosis

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Vogt-Koyanagi-Harada Syndrome

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