Features

Multifocal choroiditis and panuveitis (MCP) was first reported in 1973 in two patients who had a chorioretinopathy that resembled the lesions of presumed ocular histoplasmosis syndrome (POHS). In contrast to POHS, however, these patients had associated bilateral anterior chamber and vitreous inflammation. Although classically a panuveitis, MCP is categorized as a white dot syndrome due to its characteristic fundoscopic appearance. It is a rare, chronic, recurrent inflammatory eye disease that has a predilection for healthy individuals, especially young, myopic, women. Though idiopathic, it is presumed to be autoimmune in nature; however, there is no agreed upon etiology. The disease has been reported to appear in a broad age range from 6 to 69 years of age, with the most common occurrence between the third and fifth decades of life. Though MCP is bilateral, it may be asymmetric and is characterized by recurrent bouts of clinically evident inflammation around the sites of previous inflammation. The recurrences may occur unilaterally, bilaterally, separately, or simultaneously.

64.1.1 Common Symptoms

Clinically, most patients present subacutely and may complain of symptoms of posterior uveitis, including decreased central visual acuity (VA), floaters, photopsias, and visual field defects. Anterior segment symptoms such as photophobia may also occur. Initial presenting VA is highly variable from 20/20 to light perception. Vision loss is typically attributed to inflammation or choroidal neovascularization (CNV).

64.1.2 Exam Findings

During the active stage, examination discloses anterior chamber and vitreous inflammation, which is an important distinguishing feature from POHS. The anterior uveitis associated with MCP is nongranulomatous, may include posterior synechiae, and can range from mild to moderate in severity. The vitritis has a comparable degree of inflammation and can be asymmetric. The classic fundoscopic findings of active lesions are fluffy, yellow–gray chorioretinal lesions with pigmented borders that occur at the level of the retinal pigment epithelium (RPE) and choriocapillaris (▶ Fig. 64.1). These lesions range from 50 to 350 µm in diameter, although some may be larger, and can be distributed in the posterior, midperipheral, or peripheral retina. They may be found singly, arranged in clusters, or in linear streaks. Active disease may be associated with subretinal fluid, optic nerve hyperemia and edema, cystoid macular edema (CME), and macular and peripapillary CNV. Eventually, the older inactive lesions evolve into round, punched-out, atrophic, yellow–white areas with varying degrees of pigmentation. This, in turn, can lead to peripapillary scarring and extensive subretinal fibrosis. The peripapillary scarring resembles that seen in POHS. The recurrent bouts of inflammation make patients more susceptible to cataract formation, CME, and epiretinal membrane.

64.2 Key Diagnostic Tests and Findings

64.2.1 Optical Coherence Tomography

Active lesions appear as deposits of drusen-like homogeneous material and exhibit RPE elevation. These acute lesions demonstrate moderate reflectivity and are located in the sub-RPE and subretinal spaces (▶ Fig. 64.2). With more widespread involvement, it may incorporate the ellipsoid zone and extend beyond the zone of RPE elevation. In some cases, older lesions demonstrate scarring and loss of tissue, while others resolve without any evidence of anatomical alterations. During the acute phase, the choroid does not appear to be consistently affected by the disease process on optical coherence tomography (OCT). However, newer choroidal imaging techniques, such as enhanced depth imaging OCT and swept-source OCT, may demonstrate a subtle increase in choroidal thickness in the areas of activity.

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