Features

Sarcoidosis, a granulomatous inflammatory disorder of unknown etiology, can affect almost every organ, including the eye. Ocular involvement can be the presenting manifestation of the disease, requiring astute ophthalmic diagnosis and careful management. Definitive diagnosis requires noncaseating granulomas on biopsy from involved tissue, but this may be difficult to attain, and other clinical and laboratory findings can support the diagnosis.

Ocular sarcoidosis can involve any eye structure, including the orbit, adnexa, intraocular structures, and the optic nerve. A large percentage of patients develop bilateral, chronic granulomatous uveitis. The prevalence of ocular sarcoidosis is unknown as diagnostic criteria differ. Racial predilection varies; according to some, African Americans are more likely to develop ocular manifestations, while others show a high incidence in Japanese patients. A few studies show no difference between genders, while others reveal a female predominance. Ocular sarcoidosis can present at any age, even in young children. Typically, it is bimodal, with peak incidence at the second and third decades or the fifth and sixth decades. Recently, reports have described the development of systemic as well as ocular sarcoidosis in conjunction with granulomatous inflammation of tattoos. The cause of sarcoidosis remains unknown.

65.1.1 Common Symptoms

Symptoms typically reflect the tissue impacted. Inflammation of the lacrimal gland, eyelids, or conjunctiva results in eye irritation and dryness. In cases of intraocular inflammation, photophobia, pain, and decreased vision occur. With orbital inflammation, diplopia, proptosis, and pain develop. Neurological involvement may result in cranial nerve palsies and meningitis.

65.1.2 Exam Findings

Eyelids, Lacrimal System, Orbit, Conjunctiva, and Sclera

Focal infiltration of granulomas leads to papules or larger nodules, which can mimic tumors. Sarcoid can infiltrate the entire eyelid, creating a pseudocellulitis. The lacrimal gland is the most common orbital structure involved and can enlarge to a palpable mass. Orbital inflammation can involve fat and muscles, leading to proptosis, diplopia, and vision loss. Granulomas may involve the palpebral conjunctiva and chronic inflammation can cause cicatricial changes. If conjunctival granulomas are present, these lesions may be a good option for confirmatory biopsy. Scleral involvement is infrequent but can develop any scleritis.

Intraocular Inflammation

Anterior chamber inflammation is the most common presentation of sarcoid uveitis; however, hypopyon is rare. Patients can develop mutton-fat keratic precipitates (KP) or granulomas of the iris or trabecular meshwork (TM). Intermediate uveitis includes vitreous opacities, snowballs, pars plana exudates, or cystoid macular edema (CME). In posterior uveitis, retinal and choroidal lesions can be small (Dalen-Fuchs-like nodules) or large (granulomas), which may simulate choroidal tumors (▶ Fig. 65.1). Small choroidal lesions may resemble multifocal choroiditis or birdshot chorioretinopathy, with retinal pigment epithelium atrophy. An exudative retinal detachment may overlay large choroidal granulomas (▶ Fig. 65.2). Rarely, the optic disc or peripheral retina become neovascularized, potentially leading to intraretinal or vitreous hemorrhage. Perivascular sheathing may occur. Infrequently, severe cases develop periphlebitis, where yellow–white exudates or “candle wax drippings” develop along retinal veins. Rarely, retinal vein occlusions have been reported.

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