PMC3381111_01
Evolution of the visual field defect during the first episode of choriocapillaritis diagnosed as multiple evanescent white dot syndrome (MEWDS) and the second episode characterized as multifocal choroiditis. The peripapillary defect at presentation (left column) corresponded to indocyanine green angiography (ICGA) hypofluorescence (figure 4) and resolved without treatment (second and third columns) after the first choriocapillaritis episode diagnosed as MEWDS. Sixteen months later, a similar visual field defect again corresponding to ICGA hypofluorescence (figure 7) developed during a second episode of choriocapillaritis (fourth column) and progressed over a period of 4 days (second column from the right) but resolved (right column) one month after a sub-Tenon's injection of triamcinolone in parallel with resolution of ICGA hypofluorescence.
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Visual field testing using the Octopus Perimeter  (Haag-Streit, Bern, Switzerland) showed a peripapillary scotoma OS (Fig. 1) and a normal visual field OD. Octopus  perimetry showed a peripapillary scotoma similar to the one seen during the prior presentation which increased 4 days later (Fig. 1; third and second columns from the right); the microperimetry score declined again to 338/560 as during the first episode. ICGA where only small hypofluorescent dots representing previous chorioretinal scars were still visible (Fig. 7e) and corresponded to POSL reconstitution (Fig. 8b) as well as visual field recovery (Fig. 1, right column). In parallel, microperimetry improved from 328/560 to 424/560.
Fundus images at presentation (2a), after 1 month (2b), and at 4 months (2c). Faint discolored peripapillary fundus lesions typical of multiple evanescent white dot syndrome (MEWDS) were seen at presentation (2a) and still present after one month (2b); corresponding to fluorescein angiography (FA) hyperfluorescent areas and hyperautofluorescent areas. At 4 months (2c), these lesions take the aspect of chorioretinal scars while FA and hyperautofluorescent lesions are no longer present.
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Fundus examination showed pale discolored dots around the optic disc and along the superior temporal arcade OS (Fig. 2a) which corresponded to faintly hyperfluorescent areas on FA (Fig. 3a). Four months later, fundus examination showed scars in areas where lesions had been present, a finding not typical of MEWDS (Fig. 2c).
Fluorescein angiography at presentation (3a), after 1 month (3b), and at 4 months (3c). Patchy hyperfluorescent areas were seen at presentation around the optic disc and along the temporal superior arcade (3a) and were still present at 1 month (3b), but resolved after 4 months (3c).
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These scars were also seen on FA frames in the form of late hyperfluorescent dots (Fig. 3c) as well as on ICGA frames in the form of hypofluorescent dots (Fig. 4c). Along the superior temporal arcade, FA hyperfluorescent areas had disappeared (Fig. 3c) and on FAF a normal homogeneous aspect was seen (Fig. 5c).
Indocyanine green angiography at presentation (4a), after 1 month (4b), and at 4 months (4c). Confluent peripapillary hypofluorescence and scattered hypofluorescent areas are seen superiorly at presentation (4a), which almost completely resolved after one month (4b). However at 4 months (4c), hypofluorescent pinpoints are seen indicating chorioretinal scars.
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More precise information on the diseased areas was revealed by ICGA: there was extensive peripapillary hypofluorescence extending inferiorly together with centripetal hypofluorescent dots along the superior temporal arcade (Fig. 4a).
Fundus autofluorescence pictures at presentation (5a), after 1 month (5b), and at 4 months (5c). On the right column (left eye), hyper-autofluorescent areas corresponding to FA and ICGA lesions can be seen at presentation (5a) which, on the middle picture, slightly progressed after one month (5b) with return to normal at 4 months (5c).
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These areas were precisely delineated by FAF, showing hyper-autofluorescence corresponding to ICGA hypofluorescent areas (Fig. 5a).
Optical coherence tomography scans at presentation through ICGA hypofluorescent areas (6a), and at the border of ICGA hypofluorescent area (6b and 6c). The top set of figures (6a) represents a scan going through the ICGA hypofluorescent area showing damaged photoreceptor outer segment layer (POSL) with clumps throughout the whole scan. 6b and 6c show scans at the border of the ICGA hypofluorescent demonstrating sectoral damage to POSL (arrows).
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The whole POSL was damaged on scans through the ICGA hypofluorescent area (Fig. 6a; arrows), but damage was partial in bordering areas (Figures 6b and 6c; arrows).
Second episode of choriocapillaritis consistent with multifocal choroiditis. Fundus images (7a) show chorioretinal scars nasal to the optic disc. Both fundus autofluorescence (7b) and fluorescein angiography (7c) show only faint lesions, while on indocyanine green angiography, hypofluorescence is substantial (7d), but resolves one month after periocular triamcinolone injection (7e) in parallel with recovery of the visual field (see figure 1).
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On fundus examination peripapillary and superior nasal scars were more numerous than 12 months earlier (Fig. 7a). FA there were less intensely hyperfluorescent zones indicating more atrophic areas than acutely involved retina (Fig. 7c). FAF did not show the intense hyper-autofluorescence seen during the first episode (Fig. 7b). Only ICGA accounted for the functional impairment recorded by visual field testing and microperimetry, and allowed to identify the involved areas showing hypofluorescence precisely corresponding to the scotoma (Fig. 7d). ICGA where only small hypofluorescent dots representing previous chorioretinal scars were still visible (Fig. 7e) and corresponded to POSL reconstitution (Fig. 8b) as well as visual field recovery (Fig. 1, right column). In parallel, microperimetry improved from 328/560 to 424/560.
Spectral domain OCT showing scans going through ICGA hypofluorescent area during acute (8a) and convalescent (8b) stages of the disease. During the acute stage (8a) there is damage to the photoreceptor outer segment layer (POSL) showing clumps of photoreceptor outer segments (arrows) in ICGA hypofluorescent areas. During the convalescent stage (8b) regeneration of the POSL (arrows) is noted.
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The hypofluorescent ICGA areas also corresponded to POSL damage (Fig. 8a; arrows).