Multifocal Choroiditis and Panuveitis and Punctate Inner Choroidopathy


Summary

Multifocal choroiditis and panuveitis (MCP) and punctuate inner choroidopathy (PIC) are idiopathic inflammatory disorders that primarily affect the outer retina and subretinal pigment epithelium (RPE) bilaterally ( ). The two conditions exhibit many similarities and are considered within a spectrum of the same disorder, with PIC being a subtype of MCP. Younger myopic females are typically affected. MCP exhibits vitreous inflammation during active disease, whereas PIC typically lacks intraocular inflammation. Active lesions appear as yellowish or gray circular subretinal irregularities. Inactive lesions appear as multifocal “punched-out” atrophic areas with pigmented borders located throughout the fundus that can resemble those seen in presumed ocular histoplasmosis syndrome.

Secondary choroidal neovascularization (CNV) formation is the most frequent cause of vision loss. The distinction between inflammatory lesion and CNV is critical but can be difficult, even with fluorescein angiography. OCT, particularly OCT angiography, is extremely helpful in differentiating between these two pathologic sequelae ( Figs. 18.1.5.1–18.1.5.7 ). Treatment includes systemic immunosuppression, localized treatment to inflammatory lesions (steroid), and CNV (anti–vascular endothelial growth factor [anti-VEGF]). Treatment response, best monitored by OCT, can help confirm the type of underlying active lesion.

Fig. 18.1.5.1, (A) Multifocal choroiditis and panuveitis with active choroidal neovascularization (CNV). There are two separate elevated lesions located beneath the RPE. The nasal lesion (white arrow) is ill-defined, with loss of the RPE layer and an abnormal subretinal hyperreflective signal extending into the outer retinal layers. This area corresponds to a CNV with associated leakage on fluorescein angiography. The temporal lesion (yellow arrow) is a well-defined nodular sub-RPE elevation with medium reflectivity. This area did not have any definitive corresponding leakage on fluorescein angiography, suggesting no CNV. Subretinal fluid is also present (between white and yellow arrows). (B) After treatment with anti–vascular endothelial growth factor therapy, the temporal lesion regressed completely and the nasal lesion organized into a well-defined hyperreflective dome-shaped elevation at the level of the RPE. RPE , Retinal pigment epithelium.

Fig. 18.1.5.2, At 3 months after initial anti–vascular endothelial growth factor therapy, the patient had recurrence active choroidal neovascularization with an appearance similar to the initial occurrence (see Fig. 18.1.5.1 ). Note that the RPE is discontinuous and there is both intraretinal and subretinal fluid. RPE , Retinal pigment epithelium.

Fig. 18.1.5.3, Multifocal choroiditis and panuveitis with active choroidal neovascularization. OCT angiography slabs show two distinct areas of choroidal neovascularization (circles) located within the deep retinal slab. Corresponding OCT B-scan is shown with segmentation lines that demarcate the OCT angiography slabs (bottom) .

Fig. 18.1.5.4, At 6 months after initial presentation, despite anti–vascular endothelial growth factor treatment, a subfoveal fibrotic pigment epithelial detachment developed, with visual acuity measuring counting fingers.

Fig. 18.1.5.5, Wide-field fundus image of multifocal choroiditis and panuveitis illustrating numerous atrophic and pigmented round lesions distributed throughout the fundus.

Fig. 18.1.5.6, Baseline OCT in a patient with inactive multifocal choroiditis.

Fig. 18.1.5.7, (A) Color photograph of an active macular lesion (circle) in multifocal choroiditis and panuveitis. (B) Fluorescein angiogram reveals hyperfluorescence of the lesion resulting from staining but no definitive leakage, which was inconclusive regarding the presence of CNV. (C) Structural OCT B-scan shows an ill-defined, moderately reflective lesion at the level of the RPE with obscuration of the outer retinal layers. (D) OCT angiogram slabs confirm the presence of CNV in this location (circle) . A corresponding OCT B-scan is shown with segmentation lines that demarcate the OCT angiography slab (bottom) . CNV , Choroidal neovascularization; RPE , retinal pigment epithelium.

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