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Table 1 This table resumes the exams used by previous authors to describe retinal drusen

From: Multimodal imaging of retinal pigment epithelial detachments in patients with C3 glomerulopathy: case report and review of the literature

Authors, Journal (Year of publication)

Number of cases

Symptoms

Clinical findings

Imaging Findings

Lesions

Savige J et al., Ophthalmic Genet (2016)

6

visual acuity normal or near normal initially. First symptom: impaired night vision, progression to loss of peripheral vision

bilateral symmetrical drusen. 2 types: 1. basal laminar drusen = small, numerous, yellow. 2. large soft whitish-yellow. Retinal atrophy after 15 years and choroidal neovascularization

OCT: irregularities in RPE surface, RPE detachments, neovascular membranes. Fluorescein angiography: starry sky appearance of druse, multiple small hyperfluorescent spots throughout retina and complications. Amsler grid:distorsion with late complications multifocal ERG: lower amplitudes and lower peak amplitude with retinal atrophy

typical drusen, PED, CNV and atrophy

Dalvin LA et al., Retin Cases Brief Rep (2016)

2

1. VA 20/300 in both eyes with eccentric fixation2. VA 20/20 inboth eyes

peripheral drusen, subretinal and RPE fibrosis, RPE hypertrophy, scarring

OCT: drusen Fluorescein angiography: areas of hypofluorescence corresponding to subretinal fibrosis surrounded by leakage, window defects, andmultiple drusen

typical drusen, subretinal fibrosis

Adhi M et al., Ophthalmic Surg Lasers Imaging Retina (2014)

1

progressive loss of vision secondary to CNV in RE, and new distorsion of vision in the LE

hemorrhage and subretinal fluid superonasal to the macula

OCT: RPE detachment, irregular and prominent Bruch’s membrane ICG angiography: no definitive signs of CNV

PED, suspicion of CNV

Empeslidis T et al., Case Rep Ophthalmol Med (2012)

1

problems with near vision tasks

signs consistent with RPE detachments and small drusen-like lesions

OCT: PEDs, intraretinal fluid in a cystoid form of less than 50um in the inner retinal layers. No subretinal fluidFluorescein angiography: early phase: window defect due to basal lamina drusen; late phase: staining of hyperfluorescence. No leakage. RPE layer intact.

typical drusen. PED, intraretinal fluid with no subretinal fluid. No CNV

Ritter M et al., Br J Ophthalmol (2010)

3

VA normal or slightly reduced

drusen: temporal in early cases, and throughout the retina in advanced cases

OCT: RPE elevations, areas of compression of the photoreceptor layer missing of IS/OS and ELM backreflection Microperimetry: reduced sensitivity in areas of high drusen density

typical drusen

Han et al., Arch Ophthalmol (2009)

1

VA normal in LE, and 20/30 in RE

bilateral multifocal, 200-300um yellowish lesions at choroid and subretinal pigment epithelial level

OCT: lucent focal elevations of RPE Fluorescein and indocyanine Angiography: staining of lesions throughout the fundi, more numerous than those observed by ophthalmoscopy

typical drusen

Amer Awan M et al., Clin Exp Optom (2008)

1

blurring vision, micropsia, metamorphopsia

elevated area overlying the macula in both eyes. Multiple pale areas without any drusen. Peripheral retina normal in both eyes.

OCT: tent-shaped RPE detachment with overlying detachment of neurosensory retina and loss of foveolar contour Fluorescein Angiography: early multiple hyperfluorescence areas corresponding to these pale areas. Mid-phase leakage of dye in subretinal space

atypical idiopathic serous central chorioretinopathy with spontaneous resolution at 6 weeks

Shenoy R et al., Eur J Ophthalmol (2006)

1

asymptomatic

multiple drusen-like lesions

Microperimetry: areas of reduced retinal sensitivity in areas which were laden with drusen-like deposits, probably indicating areas of reduced retinal function OCT: drusen Fluorescein Angiography: multiple window defects in the posterior pole of both eyes corresponding to the drusen-like lesions

typical drusen

Colville D et al., Am J Kidney Dis (2003)

1

poor night vision, complications resulted in severe visual loss

occasional drusen, widespread chorioretinal atrophy, macular pigmentation

dark adaption test: uniphasic- > consistent with severe rod dysfunction ERG: delayed rod and delayed combined rod and cone responses EOG: reduced light peak/dark through (or Arden ratio) possibly reflecting an ocbstruction to the passage of metabolites from the choriocapillaris Fluorescein angiography: subfoveal choroidal neovascularization

drusen, CNV and atrophy

Lahbil D et al., J Fr Ophtalmol (2002)

1

important decrease of visual acuity in both eyes

diffuse punctiform yellow subretinal lesions and central serous detachment in both eyes

Fluorescein Angiography: after resorbtion of serous fluid, punctiform hyperfluorescent lesions still staining at late phase. ERG:normal

CRSC- like lesion

Mullins RF et al., Eye (2001)

2

 

numerous subretinal RPE deposits similar to drusen seen in AMD

histochemistry/ immunohistochemistry: same composition of drusen in AMD

typical drusen

Polk T et al., Arch ophthalmol (1997)

1

blurred vision in right eye, with visual acuity 20/20 in RE, and 20/200 in LE

fine drusen in both eyes. Neurosensory detachment in the nasal macula of RE, and disciform scar in LE

Fluorescein agiography: multiple discrete hyperfluorescent spots corresponding to drusen, and early and late staining at RPE level in the area of the detachment, with 4 pinpoints sites of late leakage. Minimal pooling of dye in the subretinal space.

neurosensory detachment with spontaneous resolution at 2weeks

C O’Brien et al., Br J Ophthalmol (1993)

4

asymptomatic

multiple, yellow, drusen-like lesions

EOG: abnormal low Arden ratios PERG, flash ERG, and flicker ERG: waveform amplitudes essentially normal for all 4 subjects

typical drusen

Leys A et al., Graefes Arch Clin Exp Ophthalmol (1991)

23

small-sized subretinal nodules simulating basal laminar drusen. 4 patients displayed marked retinopathy and3 of them exhibited subretinal neovascularization

EOG: Light Peak/Dark peak ratio reduced in 4 (36%) of the 11 patients with EOG recordings. EOG and visual field: grossly normal in patients who did not exhibit choroidal neovascularization.

Typical drusen, CNV

Leys A et al., Pediatr Nephrol (1991)

3

granular retinal changes

Fluorescein angiography: numerous basal laminar drusen

drusen

Leys A et al., Eur J Ophthalmol (1991)

4

decrease of vision

drusenoid lesions

Fluorescein angiography: neovascular membranes

CNV

Duvall-Young J et al., Br J ophthalmol (1989)

1

VA 6/24 in both eyes

normal fundus

Fluorescein angiography: numerous hyperfluorescent discrete foci at posterior pole, becoming more intensely fluorescent with time

typical drusen

Duvall-Young J et al., Br J ophthalmol (1989)

11

asymptomatic

few drusen

Fluorescein angiography: few hyperfluoresent spots corresponding to scattered drusen

typical drusen

Raines MF et al., Br J Ophthalmol (1989)

5

asymptomatic

drusen

Vitreous Fluorophotometry: Breakdown of the blood retinal barrier: vitreous fluorophotometry readings and penetration ratios abnormally high, indicating that the deposits in Bruch’s membrane cause retinal pigment epithelial dysfunction. Fluorescein angiography: normal.

typical drusen