Area of juxtafoveal atrophy with progressive extension and increasing functional repercussions.
Follow-up by SD-OCT .................................................................................................................................................... Page 348
CLINICAL CASE No. 02: DRY AMD: EARLY EXTRAFOVEAL FORM
Areas of localized and nonconfluent juxtafoveal atrophy.
Spectral-Domain OCT examination .............................................................................................................................
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12
CLINICAL CASE No. 03: DRY AMD: ADVANCED PERIFOVEAL AND SUBFOVEAL FORM
Extensive and almost confluent areas of perifoveal and subfoveal atrophy.
Spectral-Domain OCT examination .............................................................................................................................
Page 354
Chapter 12 · Atrophic Forms (Dry AMD)
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12
CLINICAL CASES OF DRY AMD
CLINICAL CASE No. 04: MACULAR ATROPHY FOLLOWING RPE TEAR
Vascularized pigment epithelium detachment with an old large tear and atrophy in the zone of RPE retraction.
TD-OCT and SD-OCT examination ............................................................................................................................
Page 356
CLINICAL CASE No. 05: MACULAR ATROPHY DUE TO VITELLIFORM MACULAR DYSTROPHY
Bilateral, asymmetrical adult-onset vitelliform macular dystrophy progressing to atrophy
TD-OCT and SD-OCT examination ............................................................................................................................
Page 360
CLINICAL CASE No. 06: MACULAR ATROPHY DUE TO VITELLIFORM MACULAR DYSTROPHY
Bilateral, asymmetrical adult-onset vitelliform macular dystrophy progressing to atrophy
Area of juxtafoveal atrophy with progressive extension and increasing functional repercussions. Follow-up by SD-OCT.
Clinical Signs
An 89-year-old woman with precursor signs of AMD had been regularly followed for 3 years with a moderate decline in visual acuity. She did not have any metamorphopsias, but her vision was consistently deteriorating, and she was having difficult with reading.
VA RE: 20/80 - VA LE: 20/80.
Biomicroscopic Examination
Fairly numerous disseminated drusen of various sizes located away from the center. A depigmented grayish 1/3 DD area with a dark peripheral border was observed in the paracentral and inferior juxtafoveal region.
Fluorescein Angiography (Figures 2c and d)
Several soft drusen, which slowly stained. The juxtafoveal area presented slight late hyper-fluorescence without fluorescein leakage.
SLO-ICG Angiography (Figures 2e and f)
Few abnormalities in the central macular region apart from several disseminated soft drusen.
Visualization of choroidal vessels in the central region, which were crossed by large veins. The small choroidal vessels were only visible in the superior temporal sector.
Poorly contrasted, poorly-delineated area of initial RPE
spots, forming several parts of a network, especially in
atrophy, possibly related to regression of a group of
the region between the optic disk and the macula (Fig-
drusen.
12
ures 2a and b).
Contribution of OCT (Spectralis*)
Horizontal section
The section, slightly off-centered inferiorly, demonstrated a normal retinal profile with decreased thickness of 218 μm (Spectralis*).
The RPE also showed numerous irregularities with small drusen.
In particular, highly suggestive, but diffuse and nonlocalized thinning of the RPE was observed in the central and paracentral zone.
Vertical section
The off-centered section visualized the atrophic zone (detected by biomicroscopy and monochromatic films). The external limiting membrane was readily visible almost everywhere on this section and was raised by numerous drusen.
In the de-pigmented area (marked by the calliper), the IS/OS interface and external limiting membrane were no longer visible. They were replaced by a moderately dense zone masking the outer nuclear layer which was otherwise normal (circle).
Diagnosis
presence of early RPE. In the absence of any exuda-
The OCT examination demonstrated minor changes
tive signs, it was decided to regularly follow-up the
at this stage, with moderate thinning of the retina,
patient, who was offered continued use of AREDS
relative thinning of the RPE, and localized damage to
formulated supplements.
the IS/OS interface and external limiting membrane.
SD-OCT demonstrated thinning then atrophy of the
These changes were localized to the small area of RPE
RPE, localized disappearance of the IS/OS interface
depigmentation, more clearly visible on biomicros-
and
external limiting membrane
, followed by loss of
copy and monochromatic films than on angiography.
the
outer nuclear layer
. This brought the outer plexi-
These alterations, associated with reduction in the
form layer directly in contact with Bruch’s membrane,
density of centrally located drusen, suggested the
with localized loss of photoreceptors.
Chapter 12 · Atrophic Forms (Dry AMD)
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12
CLINICAL CASE No. 01: DRY AMD: INITIAL STAGE
GREEN
AUTOFLUO
a
b
FA
ICG
c
d
Figure 2: Dry AMD: initial stage. VA: 20/80.
a and b): Red-free and autofluorescence: disseminated drusen of various sizes located away from the center. A de-pigmented grayish 1/3 DD area with a dark peripheral border can be seen in the paracentral and inferior juxtafoveal region (circle).
Autofluorescence: numerous, irregularly disposed hyper-fluorescent spots in the region between the optic disc and the macula. c): Fluorescein angiography: juxtafoveal area with slight late hyper-fluorescence without fluorescein leakage.
d): SLO-ICG angiography: disseminated soft drusen. Reduction of choroidal vessels in the central region, which was only crossed by large veins with no smaller choroidal vessels.
e): Horizontal section: normal retinal profile but with decreased thickness. The RPE presented numerous irregularities with small drusen. Highly suggestive, but diffuse and non-localized thinning of the RPE in the central and paracentral zone. Central retinal thickness: 218 μm. f): Vertical section : The external limiting membrane was readily visible almost everywhere and was raised by numerous drusen. In the depigmented area (marked by the calliper), the IS/OS interface and external limiting membrane were no longer visible and were replaced by a moderately dense zone masking the outer nuclear layer, which was otherwise normal (circle).
350 Chapter 12 · Atrophic Forms (Dry AMD)
CLINICAL CASE No. 01: DRY AMD: INITIAL STAGE
GREEN FA ICG
Figure 3: Dry AMD: six months later (same patient as in Figure 1). VA: 20/100.
Spectralis* horizontal section: localized juxtafoveal atrophy in the de-pigmented area of the RPE, which was now clearly visible.
In this zone, the outer retinal layers were no longer visible, apart from the outer nuclear layer.
Note the hypertrophy of the RPE at the edges of the atrophy and the alterations of the outer retinal layers over each large
12
drusen.
AUTOFLUO
FA
ICG
GREEN
Figure 4: Dry AMD: one year later (same patient as in Figure 1). VA: 20/100.
Spectralis* horizontal section: the area of atrophy was slightly larger and the outer nuclear layer had completely disappeared in this zone, which induced a juxtafoveal scotoma that interfered with reading.
Chapter 12 · Atrophic Forms (Dry AMD)
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12
CLINICAL CASE No. 01: DRY AMD: INITIAL STAGE
________________
ENLARGED IMAGES
Figure 5: Dry AMD: Enlarged images of Figures 2, 3, and 4 (same patient).
At this Stage
Comparison of images on 3 successive examinations over a period of 1 year clearly demonstrated the causes
of decreased visual acuity and difficulty with reading: appearance of a small area of juxtafoveal atrophy was barely visible on biomicroscopy but was detected by autofluorescence.
352 Chapter 12 · Atrophic Forms (Dry AMD)
CLINICAL CASE No. 02: Dry AMD: Early Extrafoveal Form
Areas of localized and nonconfluent juxtafoveal atrophy
Spectral-Domain OCT examination.
Clinical Signs
An 82-year-old woman, regularly followed for AMD for 3 years, presented for a check-up due to a moderate decline in visual acuity without metamorphopsias.
VA RE: 20/40 - VA LE: 20/40.
Biomicroscopic Examination
Five round, irregular, whitish, nonconfluent 1/5 DD spots without a peripheral border were observed in the paracentral and temporal extrafoveal region. Fairly numerous hard drusen were observed further away from the center.
Autofluorescence
The same dark spots were surrounded by a large number of autofluorescent spots (Figures 6a and b).
Fluorescein Angiography
Progressive hyper-fluorescence of each of the atrophic spots due to abnormal transmission without fluorescein leakage (Figure 6c).
SLO-ICG Angiography
The same spots were observed on SLO-ICG angiography together with subfoveal hyper-fluorescence, reflecting extension of the lesions towards the central region (Figure 6d).
Suggested Diagnosis:
Nonconfluent areas of extrafoveal RPE atrophy, appar-
ently isolated and without an exudative reaction.
12
Contribution of OCT (Spectralis*)
Horizontal section
The RPE was clearly visible with normal thickness on either side of the fovea. Multiple peaks corresponded to the presence of nonconfluent soft drusen.
In the zone corresponding to the small and de-pigmented areas, the RPE was atrophic and no longer visible. Only the straight line of Bruch’s membrane was observed with hyper-reflectivityextending a long way posteriorly, only in the extrafoveal area.
Anterior to the RPE, the external limiting membrane and the IS/OS interface were initially clearly visible, but were disrupted over the area of atrophy, where the outer nuclear layer also disappeared.
This brought the outer plexiform layer in contact with Bruch’s membrane and confirmed the localized atrophy of the RPE and photoreceptors in this zone (Figure 7a).
The signs of damage to the outer retinal layers extended as far as the subfoveal region, but were less marked and spared the outer nuclear layer.
These alterations of the subfoveal region were well correlated with the ICG angiography findings.
Oblique section (Figure 7b)
This section also clearly demonstrated the same features of atrophic zones with an only slightly altered juxtafoveal region.
Conclusion
They were adjacent to areas of atrophy (probably
SD-OCT examination of this clinical case presented
related to regression of pre-existing drusen).
multiple, small, and nonconfluent
areas of extra-
foveal atrophy
, which confirmed the findings of
SD-OCT allowed detailed analysis of the degree of
the other imaging modalities (monochromatic,
alteration of the outer layers. Changes in the fove-
autofluorescence).
al region accounted for the progressive decline in
SD-OCT confirmed the presence of a small number
central visual acuity. Finally, SD-OCT confirmed the
of soft drusen, poorly visible on fundus examination.
absence of a PED and other signs of exudation.
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12
CLINICAL CASE No. 02: DRY AMD: EARLY EXTRAFOVEAL FORM
Figure 6: Early extrafoveal dry AMD. VA: 20/40.
a and b): Red-free and autofluorescence: five, round, irregular, whitish, nonconfluent spots. On autofluorescence, the same dark, hypo-fluorescent spots were surrounded by a large number of autofluorescent spots.
c): Fluorescein angiography: hyper-fluorescence of each of the spots due to abnormal transmission with no fluorescein leakage. d): SLO-ICG angiography: the same spots were observed, together with subfoveal hyper-fluorescence, which reflected extension of the lesions towards the central region.
Figure 7: Early extrafoveal dry AMD. VA: 20/40.
a): Horizontal section: the RPE was clearly visible with numerous drusen. Atrophy of the RPE over the hyper-fluorescent spots and visibility of the straight line of Bruch’s membrane with marked back-shadowing.
Anterior to the RPE, the external limiting membrane and IS/OS interface were clearly visible and were disrupted over the area of atrophy. Disruption of the external limiting membrane and IS/OS interface and especially loss of the outer nuclear layer. The outer plexiform layer was in contact with Bruch’s membrane. Note that signs of outer layer damage extended as far as the subfoveal region.
b): Vertical section: demonstration of one of the atrophic zones. The juxtafoveal region was only slightly altered.
354 Chapter 12 · Atrophic Forms (Dry AMD)
CLINICAL CASE No. 03: Dry AMD: Advanced Perifoveal and Subfoveal Form
Extensive and almost confluent areas of perifoveal and subfoveal atrophy.
Spectral-Domain OCT examination.
Clinical Signs
An 80-year-old man presented with slowly progressive bilateral decline of visual acuity over many months. Symptoms predominantly affected his right eye with no metamorphopsias, but there were multiple central microscotomas which interfered with reading.
VA RE: 20/200 - VA LE: 20/125.
Biomicroscopic Examination
Multiple, round, but irregular and almost confluent whit-
ish areas, measuring 1/3 DD, with a slightly darker pe-
ripheral border forming an incomplete ring and a juxta-
foveolar central spot in the macular region. A few shiny
hard drusen were also observed.
Autofluorescence
The same spots were dark, hypo-fluorescent, and sur-
rounded by several fine autofluorescent spots (Figures 8a
12
and b).
Fluorescein Angiography
Fluorescein angiography showed progressive hyper-fluores- cence of each of the atrophic spots due to abnormal transmission without fluorescein leakage. Persistence of residual xanthophyll pigment in the central region (Figure 8c).
ICG Angiography
The same spots were observed on SLO-ICG angiography with abnormal visibility of the large choroidal vessels. Spots appeared to be larger and almost confluent, suggesting that the more or less intense lesions occupied almost all of the macular zone (Figure 8d).
Suggested Diagnosis:
Almost confluent areas of perifoveal RPE atrophy with
minimal foveal sparing but apparently isolated with no
exudative reaction.
Contribution of OCT (Spectralis*)
Horizontal section
The RPE was irregular, with small localized zones of proliferation, and areas in which the RPE was no longer visible. This was particularly apparent in the foveal region where only Bruch’s membrane remained clearly visible.
This subfoveal area was associated with marked backshadowing
Anterior to the RPE, the IS/OS interface was not visualized, and the external limiting membrane (occasionally visible) was absent over the area of atrophy.
Anterior to the RPE, the outer nuclear layer was also absent, bringing the outer plexiform layer in contact with Bruch’s membrane and confirming localized atrophy of RPE and photoreceptors in this zone (Figures 9a and b).
Signs of outer retinal layer damage extending beyond the central zone with numerous irregularities and jagged portions in the outer nuclear layer. These various alterations of the subfoveal region were well-correlated with ICG angiography findings. Central retinal thickness was 160 μm (Figures 9a and b).
Conclusion
SD-OCT provided detailed analysis of the degree of
This case presented multiple areas of fairly extensive
alteration of outer retinal layers, not only on areas of
and almost confluent
subfoveal atrophy
atrophy, but also in the foveal region.
The SD-OCT examination confirmed the findings of
These alterations accounted for the progressive and
the other imaging modalities (monochromatic, auto-
marked loss of central visual acuity. Finally, SD-OCT
fluorescence, and angiography) and demonstrated
confirmed the absence of an exudative reaction or
the presence of several residual hard drusen.
PED.
Chapter 12 · Atrophic Forms (Dry AMD)
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CLINICAL CASE No. 03: DRY AMD: ADVANCED PERIFOVEAL AND SUBFOVEAL FORM
Extensive and almost confluent areas of perifoveal and subfoveal atrophy.
a and b): Red-free and autofluorescence: multiple, round, but irregular and almost confluent whitish areas formed an incomplete ring and a juxtafoveolar central spot. A few shiny hard drusen were present all around these lesions.
On autofluorescence, the same spots were dark and hypo-fluorescent with several fine autofluorescent spots. c): Fluorescein angiography: progressive hyper-fluorescence of each of the spots due to abnormal transmission.
d): SLO-ICG angiography: spots appear larger and almost confluent, suggesting that the more or less intense lesions occupy almost the entire macular zone.
a): SD-OCT horizontal section: irregular RPE, no longer visible in the foveal region, where only Bruch’s membrane remained clearly visible with marked back-shadowing.
b): Enlarged images: anterior to the RPE, the IS/OS interface and external limiting membrane were no longer visible over the zone of atrophy. The outer nuclear layer was also lost, bringing the outer plexiform layer in contact with Bruch’s membrane, which confirmed the localized atrophy of the RPE and photoreceptors in this zone.
