Introduction
Materials and Methods
Results
Population Characteristics
Paper | Design | No of cases/gender | Interval | Ocular symptoms | Ocular signs | Investigations performed | Treatment | Follow-up | Outcome |
---|---|---|---|---|---|---|---|---|---|
Haritoglou et al. [5] | Case report | 1/F | 1 day | BOV BE; VA 6/150 BE; color vision severely affected | Small hemorrhages at NFL; exudative maculopathy BE | - Electrophysiological exam: • Prolonged latencies • Reduced amplitude of visually evoked cortical responses • Mild reduction of amplitudes in mfERG BE - Arden color contrast test: Abnormal | No treatment | 8 weeks | VA improved: RE 6/30 LE 6/9.5 |
Yamamoto et al. [13] | Case report | 1/M | 24 days | BOV BE; VA CF BE | Fundoscopy was normal; Optic neuropathy suspected as cause of visual disturbance | - MRI brain: no abnormalities in the optic nerves, cerebellum and cerebrum - MRI spine: multiple high-intensity spotty lesions from Th-7 to Th-11 - Lumbar puncture: mild elevation of protein and pleocytosis | IVMP | NA | Mild visual disturbance BE |
Cruz-Villegas et al. [14] | Case report | 1/M | 3 days | BOV BE; VA: RE 6/24 LE 6/120; scotomas BE; ocular pain BE | AC cells BE; AC shallow BE; Extensive bilateral choroidal effusions | - CT brain and MRI brain: normal - Lumbar puncture: negative | Topical prednisolone | 1 week | VA 6/6 BE; AC deeper with trace of cells; choroidal effusions subsided |
Lim et al. 2004 [6] | Case series | 6/M:F 1:5 | 6.8 days |
n = 11 eyes BOV (90.9%); VA range from 6/6 to HM; scotomas (36.4%) |
n = 11 eyes AC cells (18.2%); vitreous cells (18.2%); RPE changes (27.3%); RPE discoloration (9.1%); retinal hemorrhage (36.4%); retinal vasculitis (9.1%); intraretinal white lesions (18.2%); macular edema (54.5%) |
n = 11 eyes - FFA • Arteriolar focal knobby hyperfluorescence in the macula with mild vascular wall staining and leakage (27.3%) • Early hyperfluorescent spots at the level of the RPE (36.4%) • Transmission defects (36.4%) - ICG • Diffuse choroidal hyperfluorescence (81.8%) - HVF and Amsler chart: central scotoma (9.1%) | No treatment (1 case); steroid therapy (1 topical, 2 periocular, 2 oral) | 2 weeks– 4 months | RPE discoloration over affected areas; partial recovery of VA (3 cases); VA stable (3 cases) |
Siqueira et al. [7] | Case report | 1/F | 13 days | BOV BE; VA: RE 6/30 LE 6/60 | No AC or vitreous cells BE; pre-retinal hemorrhages at equator; CWS at macula; peripheral vascular sheathing BE | - FFA: areas of capillary nonperfusion in both the equator and macula. - MRI brain and carotid Doppler studies: normal | Oral anti-platelet therapy; LE Pars plana vitrectomy; RE PRP | 2 years | Poor visual acuity in LE; no further retinal vasculitis |
Nainiwal et al. [9] | Case report | 1/F | 2 days | BOV BE; VA: RE PL LE 6/18 | Vitreous hemorrhage BE | NA | Initially conservative; RE pars plana vitrectomy | 7 months | VA improved; VA 6/12 BE |
Chlebicki et al. [8] | Case series | 4/M:F 1:3 | 6.25 days |
n = 4 cases BOV (100%); VA reduced (100%); metamorphopsia (25%) |
n = 4 cases Blot hemorrhages within the vascular arcades BE (100%) | NA | Standard supportive care (4 cases); platelet transfusion (2 cases) | NA | Complete resolution within 2 days (3 cases); reduced VA & metamorphopsia after 2 months (1 case) |
Mehta [15] | Case series | 5/M:F 4:1 | NA |
n = 5 cases VA 6/6 (20%); rest had no VA done |
n = 10 eyes SCH (60%); roth spots (10%); intraretinal hemorrhage (60%); yellow thickening in choroid & retina (40%) | NA | NA | NA | NA |
Preechawat et al. [16] | Case report | 1/M | 10 days | BOV BE; VA CF BE | Flame-shaped hemorrhage at fovea RE; mild bilateral optic disc hyperemia | - FFA: no disc leakage - GVF: bilateral cecocentral scotomas - Lumbar puncture, brain and orbit MRI: all normal | IVMP followed by OPNL | 26 months | VA: 6/6 BE; color vision: normal |
Habot-Wilner et al. [17] | Case report | 1/M | 2 days | VA 6/6 BE; eyelid edema BE; conjunctival irritation BE; scotoma in central VF BE | Vitreous cells RE; retinal infiltrate with central dot hemorrhages and microhemorrhages infernosal to fovea of RE; macula edema RE; dot hemorrhage macula of LE | - Fundoscopy: optic disc normal BE - FFA: no retinal or choroidal leakage or staining | NA | 1 month | VA: 6/6 BE Fundocopy: normal BE Scotoma resolved |
Chan et al. [10] | Case series | 13/M:F 6:7 | 7 days |
n = 13 cases BOV (100%); VA range from 6/7.5 to CF; Scotomas (92.3%) |
n = 13 cases Anterior uveitis (7.7%); retinal vasculitis (30.8%); exudative retinal detachment (15.4%); macular edema (76.9%); blot hemorrhages at macula (69.2%); perifoveal telangectasia and CWS (7.7%) |
n = 13 cases - FFA: extensive fluid leakage from retinal vessels (30.8%) - HVF and Amsler chart: central scotomas (100%) - OCT: thickening of the macula (30.8%) | Conservative (11 cases); OPNL (1 case); IVMP followed by OPNL (1 case); topical prednisolone (1 case) | 2–20 weeks | Better than VA 6/9 (3 cases); central scotoma (13 cases); macular edema resolved (8 cases); macular edema improved (2 cases) |
Kapoor et al. [18] | Case series | 54/M 63.4% | NA |
n = 54 cases Retrobulbar pain (11.1%) |
n = 108 eyes SCH (76.9%); CWS (27.8%); dilatation and tortuosity of vessels (37.0%); isolated retinal hemorrhages (46.3%); superficial retinal hemorrhage sparing the macula with hard exudates and CWS (27.8%) | NA | No treatment | 2–8 weeks | SCH resolved (16 cases); posterior segment findings resolved (6 cases) |
Garcia et al. [19] | Case report | 1/M | 8 days | BOV BE; VA: RE 6/15 LE 6/9; retrobulbar pain | Vitritis BE; macular star exudates BE; optic disc swelling BE; optic disc hemorrhage BE; papillitis with areas of serous detachment in the posterior pole and vascular arcades BE | - Nuclear magnetic resonance of cranium and orbits: normal - HVF • Increased blind spot BE • Islands of sensitivity loss in the upper nasal and lower temporal regions of RE | No treatment | 2 months | VA 6/6 BE; neuroretinitis resolved; disappearance of perimetric alterations |
Teoh et al. [20] | Case series | 50/M:F 34:16 | 7 days |
n = 50 cases BOV (60%); VA range from 6/6 to CF central scotoma (30%); micropsia/metamorphopsia (4%); VF defect (2%); floaters (6%); near visual disturbance (6%); redness (2%); |
n = 65 eyes SCH (4.6%); anterior uveitis (7.7%); intermediate uveitis (12.3%); posterior vitreous cells (10.8%); retinal vasculitis (23%); macular edema (77%); macular hemorrhage (69%); optic neuritis (1.5%); optic disc swelling (3.1%) |
n = 65 eyes - OCT: foveal elevation (33.8%) - HVF and Amsler chart: central scotoma (39%) | Conservative (37 cases); OPNL (6 cases); IVMP followed by OPNL (7 cases); topical prednisolone (5 cases) | 1 year |
n = 50 cases VA 6/12 or better in 3 months (76%); and at 1 year (86.2%); 11 eyes from 7 patients (17%) who received IVMP had vision remaining between 6/12 to 6/60; persistent scotoma at 3 months (80%) and at 1 year (6%) |
Seet et al. [21] | Case series | 156/M:F 89:67 | 4.6 days |
n = 156 cases Eye strain (29.5%); retro-ocular pain (19.9%); BOV (10.3%); double vision (3.2%); foreign body sensation (3.2%); eye flashes (1.9%); floaters (1.3%) |
n = 156 cases Retinal hemorrhages (1.3%) | - Slit-lamp examination: normal - Fundoscopy: normal | NA | NA | NA |
Chang et al. [22] | Case report | 1/F | 7 days | BOV BE; VA 6/120 BE | Mild vitreous hemorrhage BE; disc hemorrhage and swelling BE; retinal hemorrhage and vasculitis BE; sheathing of macular arterioles BE; macular detachment BE | - FFA: bilateral macular arteriolar leakage | IVMP followed by IVIG, followed by IVMP and OPNL | 1 week | VA: 6/12 BE |
Tan et al. [23] | Case report | 1/M | 8 days | BOV BE; VA: RE 6/30 LE 6/50; reduced colour vision | Flame and blot hemorrhages in posterior pole BE; soft exudates and macula ischemia; hyperemic optic discs; dilated veins | - FFA: no leakages - ICG: spots of hyperfluorescence | High dose steroids | NA | VA and color vision improved markedly |
Bacsal et al. [11] | Case series | 41/M:F 17:24 | 6.9 days |
n = 71 eyes BOV (87%); VA range from 6/6 to HM; scotomas (63%); floaters (1%) |
n = 71 eyes AC cells (17%); vitreous cells (31%); both anterior and vitreous cells (11%); vitreous hemorrhage (1%); RPE mottling (17%); intraretinal precipitates (1%); yellow subretinal dots (28%); arteriolar sheathing (4%); venular sheathing (45%); retinal hemorrhage (45%); optic disc hyperemia (14%); optic disc edema (11%); foveal swelling (15%) |
n = 71 eyes - FFA • Arteriolar leakage (3%) • Venular occlusion (25%) • Venular leakage (13%) - ICG: • Early large-vessel hyperfluorescence (31%) • Mid to late hypofluorescent spots (29%) - HVF: central or paracentral scotomas (63%) - OCT: • Exudative RD (10%) • RPE thickening at the fovea (26%) • RPE atrophy (2%) - Amsler chart: bilateral large dense central scotomas (2.8%) |
n = 41 cases Steroid therapy (28 cases): - IVMP followed by OPNL (12 cases) - OPNL (11 cases) - IVIG with IV hydrocortisone & OPNL (3 cases) - Periocular methylprednisolone (10 eyes) - Intravitreal triamcinolone acetonide (2 eyes) - Topical prednisolone acetate (1 eye) | 5.4 months |
n = 71 eyes Improvement in VA (100%); 52.1% of eyes at initial follow-up improved to better than 6/12 vision, followed by 85.9% of eyes at final follow-up |
Su et al. [12] | Case series | 197/M:F 119:78 | 6.8 days | BOV; VA range from 6/6 to 6/36; scotomas; visual complaints | Maculopathy (27 eyes); white spots at macula (15 eyes); yellow spots at macula (3 eyes); PED (1 eye); vascular sheathing (1 eye); RPE mottling (4 eyes); hyperemic optic disc (2 eyes); swollen optic disc (1 eye); optic disc hemorrhages (1 eye) | - FFA: mild anteriolar and/or venular leakage in some eyes - ICG: hypofluorescence in mid and late phases in some areas - OCT: outer neurosensory retina/RPE thickening at fovea (1 case) - Amsler chart: abnormalities noted | NA | NA | NA |
Tan et al. [24] | Case report | 1/F | 6 days | BOV BE; VA: RE 6/120 LE CF; metamorphopsia BE | Yellowish retinal infiltrates BE; extensive retinal vasculitis in the macula BE complicated by BRVO RE; macular edema BE; blot hemorrhages at macula BE | - FFA: leakage indicative of panretinal vasculitis. Follow-up FFA at 10 weeks showed retinal capillary nonperfusion at the infero-nasal margin of the foveal avascular zone - OCT: central foveal thickening - Amsler chart: distortion in RE | Platelet transfusion; conservative treatment otherwise | 10 weeks | VA 6/6; No residual inflammation or macular edema; RE has mild red desaturation and visual distortion on Amsler grid monitoring |
Beral et al. [25] | Case series | 3/M:F 2:1 | 1 month (2 cases); 15 days (1 case) |
n = 6 eyes impaired vision (83.3%); VA range from 20/20 to HM; diffuse orbital pain (33.3%) |
n = 6 eyes Nasal parapapillary hemorrhage (33.3%); Optic disc swelling (83.3%) | - VF testing: severe global loss of contrast sensitivity - MRI brain: normal - Lumbar puncture: normal | IVMP | NA | VA: RE 6/15 LE PL (1 case); VA: RE 6/9 LE 6/7.5; inferior scotoma and enlargement of the blind spot BE; residual temporal atrophy of both optic nerves; color vision tests no residual abnormality (1 case); worsening of VA to HM (1 case) |
Filho et al. [26] | Case report | 1/F | 3 days | Impaired vision BE; VA HM BE; intense ocular pain; lacrimation; photophobia; redness; raised IOP | Corneal edema; shallow AC; iris atrophy; fixed and moderately dilated pupils BE; both crystalline lenses exhibited nuclear sclerosis 2 + and glaucomflecken | - Gonioscopy: closed angles - Fundoscopy: not clear - B scan ultrasonography: normal posterior segments BE - CT head: normal | Medical treatment followed by bilateral laser iridotomies | 1 month | VA: 6/60 BE; IOP normalized; no significant alterations on eye fundus exploration without dilatation |
Kanugo et al. [27] | Case report | 1/F | 7 days | BOV; VA 6/6 BE; RE inferior visual field defect; ocular pain | CWS LE; BRAO RE | - FFA: • Midphase narrowing of the affected arterial segment RE • Late phase staining and leakage of the occluded artery RE - Kinetic central field charting with Bjerrum’s screen: infero-nasal field defect RE | NA | 3 months | VA 6/6 BE; persistent inferior field defect RE |
Loh et al. [28] | Case series | 6/M:F 4:2 | 5–7 (8 eyes); 9 (2 eyes) |
n = 10 eyes BOV (80%); VA range from 6/7.5 to CF; central scotoma (90%) |
n = 10 eyes AC cells (20%); CWS (20%); venular sheathing (30%); BRVO (20%); blot hemorrhages (50%); retinal striae (20%); yellow-orange lesion at fovea (100%); macular edema (20%) |
n = 10 eyes - FFA: early hypofluorescence that persisted in the late phase at the fovea (30%) - Electrophysiological exam: mfERG-decreased foveal and parafoveal responses (100%) - OCT: • Outer neurosensory retina disruption (100%) • Elevation of fovea (20%) | IVMP followed by OPNL (1 case); OPNL (2 cases); IVIG (2 cases); periocular depomedrol (2 eyes); periocular traicinolone acetonide (1 eye); no treatment (1 eye) | 9.7 months |
n = 10 eyes 6/6 by 3 months (60%); 6/6 by 5 months (10%); 6/7.5 by 3 months (10%); 6/7.5 by 1 year 3 months (10%); 6/12 with persistent yellow-orange lesion at fovea at 3 months (10%) |
Sanjay et al. [29] | Case series | 3/M:F 1:2 | 5–7 |
n = 6 eyes BOV (50%); VA range from 6/6 to CF; impaired color vision (16.7%); scotomas (33.3%); enlarged blindspot (16.7%) |
n = 6 eyes Retinal edema (16.7%); retinal hemorrhages (66.7%); CWS (66.7%); optic disc swelling (66.7%); optic disc atrophy (16.7%) |
n = 6 eyes - Electrophysiological exam: • mfERG-centrocecal scotoma (16.7%) • pVEP-delayed P100 latency (16.7%) and absent response (16.7%) - MRI: edema of optic nerve sheath complex (16.7%) - HVF: • Central scotoma (16.7%) • Paracentral scotoma (33.3%) • Enlarged blind spot (16.7%) | IVMP followed by OPNL (1 case) | 6 weeks–1 year | VA 6/9 with impaired color vision and paracentral scotoma (1 eye); NPL RE and resolved VF defects in LE (1 case); VA 6/6 with signs and symptoms completely resolved (1 case) |
Gupta et al. [30] | Case series | 6/M:F 2:4 | 3–5 months |
n = 7 eyes BOV (100%); VA range from 6/12 to CF; ocular pain (42.9%); redness (28.6%) |
n = 7 eyes Ciliary congestion (42.9%) anterior uveitis (85.7%); vitritis (14.3%); retinal vasculitis (14.3%); macular edema (14.3%) |
n = 7 eyes - FFA: vasculitis and retinal hemorrhages in perimacular region (14.3%) - OCT: macular edema (14.3%) | OPNL and topical medications (steroids and ocular hypotensive medications) | 3–5 months | All responded to treatment: 6/9 in 5 eyes; 6/24 and 6/18 in other 2 eyes due to cataract |
Quek et al. [31] | Case report | 1/F | 7 days; 5 daysa
| 1st infection: BOV BE; VA: RE 6/120 LE CF; metamorphopsia BE Recurrent infection: BOV BE; VA 6/6 BE; scotomas RE | 1st infection: Posterior uveitis; flame-shaped hemorrhages; CWS at macula; vascular sheathing at macula Recurrent infection: mild macular edema | 1st infection: - FFA: • Vascular leakage in all quadrants • Retinal periphlebitis with right macular BRVO and macular edema - OCT: cystic edema BE | Conservative treatment | 6 months; 2 years | 1st infection: Vision and metamorphopsia improved; VA 6/6 BE; posterior uveitis and vasculitis resolved; macular edema resolved; persistent, non-progressive paracentral relative scotoma Recurrent infection: symptoms resolved; scotoma persisted |
Chee et al. [32] | Case series | 87/M:F 59:28 | 6.2 days | Normal VA | None | - Amsler chart: normal - Fundoscopy: normal - Slit lamp: normal | NA | 14 days | No change |
Teoh et al. 2010 [33] | Case series | 41/M:F 22:19 | 7 days |
n = 41 cases BOV (51.2%); VA ≥6/24 (41.9% of affected eyes); <6/24 (50% of affected eyes); scotomas (34.1%); floaters (4.9%); micropsia (4.9%); Near vision disturbance (4.9%) |
n = 41 cases Anterior uveitis (7.3%); intermediate uveitis (4.9%); optic neuritis (2.4%) |
n = 74 eyes - OCT • Diffuse oedema (44.6%) • Macular oedema (21.6%) • Cystic foveolitis (33.8%) - Amsler chart: relative scotomas (91.9%) | NA | 2 years | VA < 6/24 (50% of affected eyes at presentation and 2.7% after 2 years) 59.5% of affected eyes retained a persistent relative central/paracentral scotomata even after 2 years, but only in 1.4% was it visually disturbing. |