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Ординатура / Офтальмология / Английские материалы / Electrodiagnosis of Retinal Disease_Miyake_2005

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4.7 Age-Related Macular Degeneration 225

4.7.1How Does Subretinal Hemorrhage Damage the Retina?

A thick subretinal hemorrhage situated under the fovea occasionally has a natural course that leads to severely decreased visual function even in those without choroidal neovascularization. Animal experiments have shown that the photoreceptors can be damaged by the toxic effects of the iron released by red blood cells as well as by mechanical blockage by the hemorrhage, which hinders metabolic exchange between the photoreceptors and the RPE cells [1].

Subretinal macular hemorrhages can be successfully removed by vitrectomy using tissue plasminogen activator [2], leading to an improvement in the postoperative visual acuity of patients whose vision was good before the hemorrhage [3] (Fig. 4.31).

Changes in the focal macular ERGs elicited by a 10° spot in five patients who had a submacular hemorrhage removed by vitrectomy using tissue plasminogen activator are shown in Fig. 4.32. The submacular hemorrhage was caused by rupture of a macroaneurysm in three of the eyes, AMD in one of the eyes, and an unknown reason in the fifth eye. The duration of the subretinal hemorrhage ranged from 3 to 14 days. All of the patients had extremely poor visual acuity (from hand motion to 0.05) before

surgery, and the visual acuity improved to 0.1–0.7 after surgery.

Three points can be made based on these results. First, focal macular ERGs were undetectable in all of these patients before surgery regardless of the duration of the submacular hemorrhage. This is definitely different from the results from patients with idiopathic central serous chorioretinopathy, where serous fluid, not blood, accumulates between the photoreceptors and the RPE. Patients with central serous chorioretinopathy usually have much better visual acuity with good-amplitude focal macular ERGs (see Section 4.1). This difference indicates that blood can alter the function of the photoreceptors much more so than can serous fluid.

Second, the decline of photoreceptor function is partially reversible when blood is removed from the subretinal space even after 14 days, as shown by the recovery of the focal macular ERG in case 5. However, it is more likely that the earlier the blood is removed the better is the recovery. The third point is that there is little if any toxic effect of recombinant tissue plasminogen activator injected at clinically useful doses.

226 4 Acquired Macular Diseases

Fig. 4.31. Top: Preoperative fundus photograph with a 10° spot superimposed on the fovea. The image was obtained while recording the macular ERG in a patient with submacular hemorrhage. Bottom: Postoperative photograph. The retinal blood is almost completely removed. (From Terasaki et al. [3], with permission)

Fig. 4.32. Focal macular ERGs recorded from five patients with subretinal macular hemorrhage. The stimulus spot was 10° in diameter. The fellow eye of case 5 shows no response because of macular degeneration of unknown cause. After surgical drainage of the subretinal hemorrhage using human plasminogen activator, there is partial recovery of the ERG in all patients. (From Terasaki et al. [3], with permission)

4.7 Age-Related Macular Degeneration 227

4.7.2Removal of Choroidal Neovascular Lesions

Surgical removal of choroidal neovascular (CNV) lesions is undertaken to preserve or regain central neurosensory retinal function as an alternative treatment to conventional laser photocoagulation in eyes with AMD (Fig. 4.33). The focal macular ERGs elicited by a 15° spot before and after removing CNV lesions in three patients are shown [4] in Fig. 4.34. The amplitudes of the a-waves, b-waves, and OPs were markedly reduced or unrecordable with prolonged implicit times before surgery. These findings indicated severe impairment of macular cone function, which agrees with a histological report that there is marked photoreceptor loss in eyes with AMD. Most patients had

a significant recovery at 3 months after surgery, with the b-wave amplitude selectively increased without an increase in the a-wave amplitude. These findings suggest further recovery of inner retinal function, particularly the on bipolar cells.

The postoperative increase in b-wave amplitude in eyes with preoperative recordable b- waves significantly correlated with the decrease in the mean parafoveal thickness measured by OCT [4] (Fig. 4.35). This suggests that inner retinal function is impaired by retinal edema, and the decreased thickness of the macula contributes to the recovery of the inner layer function of the macula.

Fig. 4.33. OCT images obtained from a 56-year-old man with AMD before and after choroidal neovascular (CNV) lesion removal

228 4 Acquired Macular Diseases

Fig. 4.34. Focal macular ERGs recorded from a normal control and three patients with AMD before and after CNV removal. The visual acuity is also shown. (From Terrasaki et al. [4])

Fig. 4.35. Percentage increase in the amplitude of the b-wave and the percentage decrease in the mean parafoveal thickness measured at four points on four sides of the fovea in vertical and horizontal scans. The results are significantly correlated (r = 0.688, P = 0.0076). (From Terasaki et al. [4], with permission)

4.7 Age-Related Macular Degeneration 229

4.7.3Macular Translocation Surgery

Although surgical removal of a subfoveal CNV can be performed in some patients with AMD, the main disadvantage of the procedure is reduced visual function that results from damage to the RPE underneath the macula. Foveal or macular translocation surgery is an operative procedure in which the fovea is moved from diseased RPE onto healthy RPE [5]. This surgery has the potential to improve or preserve central visual function in eyes after removing a subfoveal CNV.

One common technique of macular translocation surgery involves a 360° circumferential retinotomy followed by a subretinal infusion of fluid to create total retinal detachment from the RPE. The completely detached retina is then rotated to move the macula from the original position on the RPE to healthy RPE, resulting in a new retina and RPE complex. This complicated surgery has been well performed by Terasaki, my colleague, in many patients, and the results have been evaluated [6–8]. The fundus photographs and OCT images of a patient with AMD before and after macular translocation surgery are shown in Fig. 4.36.

From the point of view of retinal physiology, this technique poses several interesting and important issues that should be considered. First, what is the visual function of the entire retina after it is translocated to the new RPE? To answer this question, full-field ERGs

recorded from three representative patients with AMD before and after macular translocation with 360° retinotomy were compared [6] (Fig. 4.37). In summary of many patients, the amplitudes of the rod and cone components of the full-field ERGs were reduced by 25%–40%, with slight prolongation of the implicit times after the surgery. Interpreting these results has not been easy because, in addition to translocating the entire retina to the new RPE, we had to take into consideration surgical trauma to the retina caused by transient total retinal detachment, the 360° circumferential retinotomy, and the massive photocoagulation after reattaching the retina. However, the degree of reduction of the full-field ERGs after surgery was surprisingly small, suggesting that the sensory retina can function well at the new RPE site.

Second, how does the shifted macula function on the new RPE? The answer is obtained from the changes in visual acuity and the focal macular ERG before and after surgery [7]. In our large series of patients, visual acuity improved significantly after surgery; moreover, the amplitudes of the focal macular ERGs increased and the implicit times decreased significantly after surgery (Fig. 4.38). These results indicate that the newly located macula does maintain and organize the neural components well, resulting in improved visual acuity.

230 4 Acquired Macular Diseases

Fig. 4.36. Fundus photographs (top) and OCT images (bottom) obtained from a 77-year-old woman with AMD before and after macular translocation with 360° retinotomy. The macula was shifted to the inferior part of the retina, where the RPE underneath the macula was healthy. Her visual acuity improved from 0.06 to 0.60 following surgery. (From Terasaki et al. [8], with permission)

Fig. 4.37. Full-field ERGs recorded from three representative patients with AMD before and after macular translocation with 360° retinotomy. (From Terasaki et al. [6])

Fig. 4.38. Focal macular ERGs recorded from three representative patients with AMD before as well as early (6–12 months) and late (18–30 months) after macular translocation with 360° retinotomy. (From Terasaki et al. [7])

4.7 Age-Related Macular Degeneration 231

References

1.Glatt H, Machemer R (1982) Experimental subretinal hemorrhage in rabbits. Am J Ophthalmol 94: 762–773

2.Lewis H, Resnick SC, Flannery JG, Straatsma BR (1991) Tissue plasminogen activator treatment of experimental subretinal hemorrhage. Am J Ophthalmol 111:197–204

3.Terasaki H, Miyake Y, Kondo M, Tanikawa A (1997) Focal macular electroretinogram before and after drainage of macular subretinal hemorrhage. Am J Ophthalmol 123:207–211

4.Terasaki H, Miyake Y, Niwa T, Ito Y, Suzuki T, Kikuchi M, et al. (2002) Focal macular electroretinograms before and after removal of choroidal neovascular lesions. Invest Ophthalmol Vis Sci 43:1540–1545

5.Machemer R, Steinhorst UH (1993) Retinal separation, retinotomy, and macular relocation. II. A surgical approach for age-related macular degeneration? Graefes Arch Clin Exp Ophthalmol 231: 635–641

6.Terasaki H, Miyake Y, Suzuki T, Niwa T, Piao CH, Suzuki S, et al. (2002) Change in full-field ERGs after macular translocation surgery with 360° retinotomy. Invest Ophthalmol Vis Sci 43:452–457

7.Terasaki H, Ishikawa K, Niwa Y, Piao CH, Niwa T, Kondo M, et al. (2004) Changes in focal macular ERGs after macular translocation surgery with 360° retinotomy. Invest Ophthalmol Vis Sci 45:567–573

8.Terasaki H, Ishikawa K, Suzuki T, Nakamura M, Miyake K, Miyake Y (2003) Morphorogic and angiographic assessment of the macula after macular translocation surgery with 360° retinotomy. Ophthalmology 110:2403–2408

Subject Index

a-wave

3

 

choroidal detachment

183

endophthalmitis

186

 

Åaland Island eye disease 91

choroidal neovascular (CNV)

enhanced S-cone syndrome

68

ABCR

126, 161

 

lesions

227

 

EOG 1, 41, 76, 165, 182, 183

 

acute idiopathic blind spot

choroideremia

64

 

 

see also

 

 

 

 

enlargement syndrome

see also

 

 

 

base value

 

 

 

 

187

 

carrier state

 

 

 

dark trough

 

 

acute zonal outer retinopathy

complete form

90, 136

L/D ratio

 

 

 

 

(AZOOR) 187

cone 4

 

 

 

light peak

 

 

 

adult-onset foveomacular

cone (photopic) ERG

11

ERGs from pediatric patients

6

 

vitelliform dystrophy 165

cone dysfunction

114

 

 

 

 

age-related macular degeneration

cone dystrophy

116, 123

familial optic atrophy with negative

 

223

 

see also

 

 

 

amacrine cells

3, 29

central cone dystrophy

ERG

150

 

 

APB

8, 99

 

cone–rod dystrophy

123

fingerprint pattern

58

 

arrestin

119

 

cone–rod interaction

11

first-order kernel

35, 102

 

 

 

 

 

congenital stationary night

focal macular ERG

20–32, 78, 88,

 

 

 

 

blindness (CSNB) 90

102, 153, 176, 204, 209, 215,

b/a ratio

174, 180, 181, 186

see also

 

 

 

221, 224, 229

 

b-wave

3

 

complete form

 

 

foveal thickness 213

 

base value 183, 184

incomplete form

 

 

foveoschisis

73

 

 

batten disease

58

congenital syphilis

196

(full-field) dome

2

 

“bear-track” pigmentation

53

 

congenital tritanopia

141

full-field ERG

2–20, 45, 76, 95, 153,

Best’s disease

165

 

 

 

 

contact lens electrode

2

 

207, 224, 229

 

 

binary m-sequence

36

 

 

 

CRX 8

126

 

 

 

 

see also

 

 

 

 

blue cone ERG 142

 

 

 

 

crystalline retinopathy

55

pediatic patients

 

 

blue cone monochromacy

138

cystoid macular edema (CME)

full-field stimulator

2

 

blue-sensitive opsin

142

 

 

 

203, 205

 

 

 

fundus albipunctatus

 

114

bone spicule pigmentation

45

 

 

 

 

 

 

 

fundus flavimaculatus

160

bull’s-eye maculopathy

58, 114,

 

 

 

 

 

 

 

 

 

 

 

123, 132, 148

 

 

 

 

d-wave

8, 9, 14

 

 

 

ganzfeld dome

 

 

 

Burian-Allen bipolar contact lens

dark adaptation

11, 93

 

6

 

 

23

 

 

 

 

 

dark choroid

148, 160

 

golden tapetal-like fundus reflex

 

 

 

 

 

 

dark trough

41

 

 

 

82

 

 

 

 

 

 

 

 

 

 

delay in dark adaptation

114

grouped pigmentation of retina 53

CACNA1F 91, 105

 

 

 

 

depolarizing bipolar cells

8, 99

GTP 126

 

 

 

 

carrier state

65

 

 

 

 

diabetic macular edema

177

GUCA1A

126

 

 

 

– of X-linked ocular albinism

 

diabetic maculopathy

176

GUCY2D

126

 

 

 

194

 

 

 

 

 

diabetic retinopathy

170

 

gyrate atrophy

66

 

 

central cone dystrophy

128

 

diabetic vitreous hemorrhage

 

 

 

 

 

central retinal artery occlusion

181

 

174

 

 

 

 

hexagonal elements

 

 

central retinal vein occlusion

180

diffuse unilateral subacute

 

34

central serous chorioretinopathy

 

neuroretinitis

191

horizontal cell

13

 

 

200

 

 

 

 

 

dominantly inherited juvenile optic

hyperpolarizing bipolar cells 8, 99

cGMP 126

 

 

 

 

 

 

atrophy

141

 

 

hypotonic maculopathy

183

234 Subject Index

idiopathic epimacular membranes

bull’s-eye maculopathy

 

 

focal macular ERGs in –

45

 

209

 

 

 

 

 

 

familial optic atrophy

 

 

see also

 

 

 

 

 

 

idiopathic macular hole

215

 

nematodes

191

 

 

 

 

 

full-field ERGs

 

 

 

 

 

incomplete form

136

 

 

neovascular glaucoma

180

 

macular OPs

 

 

 

 

 

infrared television fundus camera

Nettleship-Falls ocular albinism

multifocal ERG

 

 

 

 

 

 

21

 

 

 

 

 

 

 

87

 

 

 

 

 

 

 

rod (scotopic) ERG

4

 

 

inner retinal breaks

73

 

 

neuronal ceroid-lipofuscinosis 58

rod bipolar cells

8

 

 

 

 

intensity-response series

3, 146

nonischemic types

180

 

 

rod monochromacy

 

136

 

internal limiting membrane (ILM)

nyctalopin

105

 

 

 

 

 

rod-cone dysfunction syndrome

 

218, 219

 

 

 

 

nystagmus

136

 

 

 

 

 

 

144

 

 

 

 

 

 

interplexiform cells

29

 

 

NYX

91, 105

 

 

 

 

 

 

rod–cone dystrophy

 

44

 

 

interstitial keratitis

196

 

 

 

 

 

 

 

 

 

 

 

rod–cone interaction

13, 16

ISCEV

4

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

rubella retinitis

194

 

 

 

ISCEV standards

2, 6

 

 

occult macular dystrophy

128, 153

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

OCT

42, 87, 158, 165, 178, 199, 202,

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

215, 221, 227, 229

 

 

salt and pepper retinopathy

61

kearns-sayre syndrome

61

 

off bipolar cells

70, 90

 

 

salt-and-pepper change

194

KYN

9, 99

 

 

 

 

 

off response

 

8

 

 

 

 

 

Schubert-Bornschein type

90

 

 

 

 

 

 

 

 

Oguchi’s disease

119

 

 

 

S-cone ERG

10, 68, 77, 101

 

 

 

 

 

 

 

 

on bipolar cells

70, 90

 

 

scotopic threshold response (STR)

L/D ratio

41, 183

 

 

 

on response

 

8

 

 

 

 

 

 

3, 97

 

 

 

 

 

 

LED

5, 9

 

 

 

 

 

 

OPA1

142

 

 

 

 

 

 

 

second-order kernel

 

35, 102

light adaptation

11

 

 

 

origin of ERG

1

 

 

 

 

 

short-flash b-wave

14

 

 

light peak

41

 

 

 

 

ornithine aminotransferase

66

sign-inverting synapses

8

 

LM cone ERG

10

 

 

 

oscillating potentials

 

2, 170

 

sign-preserving synapses

 

8

long-flash photopic ERG

8, 9

 

 

 

 

 

 

 

 

 

 

silent choroid

160

 

 

 

 

luetic chorioretinitis

196

 

 

 

 

 

 

 

 

 

 

Stargardt disease

148, 160

 

 

 

 

 

 

 

 

 

panretinal photocoagulation

171

stray light 20

 

 

 

 

 

 

 

 

 

 

 

 

 

paravenous retinochoroidal atrophy

subjective blue sensitivity

101

m-sequence

34

 

 

 

 

 

51

 

 

 

 

 

 

 

subretinal hemorrhage

225

macular hole surgery

217

 

PDA

9, 99

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

macular OPs

27–32, 46

 

 

perimetry

158

 

 

 

 

 

30-Hz flicker ERG

 

96

 

 

macular pseudohole

221

 

peripheral cone system

128

 

 

 

 

macular translocation 229

 

peripheral retinoschisis

73

 

tritan

138, 141

 

 

 

 

 

MEWDS

187

 

 

 

 

peripherin/RDS

126

 

 

 

two-color perimetry

 

131, 133, 158

mitochondrial DNA

61

 

 

photophobia

123, 136

 

 

 

 

 

 

 

 

 

 

mitochondrial myopathy

61

 

photopic hill phenomenon

14

unilateral cone dysfunction

 

mixed cone–rod ERG

4

 

 

photopic long-flash ERGs

15

132

Mizuo phenomenon

119

 

photoreceptor potential

13

 

 

 

 

 

 

 

 

 

monitoring ERGs

7

 

 

 

plasminogen activator

226

 

VER

 

 

 

 

 

 

 

monitoring during surgery

7

PNR

68

 

 

 

 

 

 

 

23, 24

 

 

 

 

 

 

Mueller cells

3, 74, 219

 

 

proliferative diabetic retinopathy

visual acuity

75, 92

 

 

 

multifocal ERG

34–40, 46, 79, 102,

 

174

 

 

 

 

 

 

 

vitelliform macular dystrophy

 

153, 188, 215, 224

 

 

pseudohypopyon

165

 

 

 

165

 

 

 

 

 

 

multiple evanescent white dot

 

 

 

 

 

 

 

 

 

 

vitelliruptive

165

 

 

 

 

 

syndrome (MEWDS)

187

 

 

 

 

 

 

 

 

 

vitreous fluorophotometry

123

multiple white flecks

86

 

 

RDH

86

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

RDH5

116

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

refractive error

75, 92

 

 

X-linked ocular albinism

87, 194

nasotemporal asymmetry

28

 

retinal detachment

183

 

 

X-linked retinoschisis

72, 148

negative ERG

90, 147, 191

 

retinitis pigmentosa

 

44, 45

 

XLRS1

148

 

 

 

 

 

 

see also

 

 

 

 

 

 

atypical –

 

 

 

 

 

 

 

X-wave 16