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8/6/2012

Fundus Autofluorescence ( FAF or AF) is a novel, non-invasive imaging


procedure that often yields abnormalities that are invisible to
Panoramic Fundus ophthalmoscopy and standard color fundus photography. It allows
mapping of lipofuscin distribution in the outer retina which are crucial
findings in complex retinal diseases. FAF, however, is limited to outer
Autofluorescence: retinal disorders.

Clinical Applications and FAF is likely due to lipofuscin, the wear and tear pigment found in retinal
cells, especially RPE cells.
Limitations The normal retinal pigment epithelium (RPE) yields a slightly granular AF glow
in contrast to the optic disc and retinal blood vessels which appear black.

By Jerome Sherman, OD, FAAO The accumulation of lipofuscin, often due to lysosomal dysfunction, results in
increased AF and suggests RPE dysfunction or stress.
Decreased FAF suggests loss of RPE cells (as well as possibly photoreceptors)
and correlates to reduced levels of lipofuscin.

As revealed in a series of cases to follow, Panoramic FAF is now possible and


appears to reveal abnormalities throughout the entire retina, often invisible
to other imaging modalities

Case 1: Optomap Color Fundus Image OD Case 1: Optomap Fundus Auto Fluorescence Image OD

A 25 year old moderate myope exhibits an essentially normal color and AF A typical AF image from a normal fundus reveals a slightly granular glow to the RPE. The disc and
panoramic images in each eye. blood vessels appear as black in distinct contrast to the RPE glow.

Case 1: Optomap Color Fundus Image OS Case 1: Optomap Fundus Auto Fluorescence Image OS

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8/6/2012

Case 1: Optomap OD Case 1: Optomap OS


KEY:: KEY::
Color Optomap Color Optomap
Red Separation Red Separation
Green Separation Green Separation
Auto Fluorescence Auto Fluorescence

Case 1: Optomap OD and OS Case 2: Optomap Color Fundus Image OD


KEY::
Color Optomap
Auto Fluorescence

A 56 year old, 5 diopter myopic Hispanic female presented for examination as a glaucoma suspect.
BCVA is 20/20-2 in the right eye and 20/400 with eccentric viewing in the left eye. In the color
fundus image, note the visibility of the choroidal vessels, most likely due to myopic stretching and
reduction in RPE. Note the nasal, large arcuate pattern of increased visibility of choroidal vessels,
most likely due to a posterior staphyloma.

Case 2: Optomap FAF Image OD Case 2: Optomap Color Fundus Image OS

In addition to similar findings in the fundus of the right eye, the left eye exhibits
some subtle areas of pigmentary migration most marked in the mid temporal
AF image is essentially within normal limits. region. With standard fundus photography of 40-50 degrees of the central fundus,
none of these pigmentary changes are imaged.

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Case 2: Optomap FAF Image OS Case 2: Optomap Fundus Image OS


KEY::
Color Optomap
Red Separation
Green Separation
Auto Fluorescence

Note the dramatic FAF in the left eye. Large black (hypo AF) circular and oval zones represent
loss of the RPE and lipofuscin. A similar hypo AF zone is surrounding the disc. However, the
central part of the image exhibits hyper AF which is suggestive of metabolically stressed RPE.

Case 2: Optomap Color Fundus and FAF Comparison OS Case 2: Optomap FAF Comparison OD and OS
Patient reported
sudden loss of vision
in her left eye about
3 decades earlier.
Evaluation at that
time failed to reveal
any specific etiology.
When questioned
she does not recall
any trauma prior to
vision loss and has
no family history of
eye problems.
Differential diagnosis
includes the very rare
unilateral RP, DUSN,
and trauma such as
uniltaral ophthalmic
occlusion. Patient is
presently being
evaluated.

Although ophthalmoscopy, standard fundus photography reveal very little difference between the
two eyes, Panoramic AF reveals a profound contrast between the normal right eye and the affected
left eye.

Case 2: Optomap Color Fundus and FAF Comparison OD and OS Case 3: Optomap Color Fundus Image OD
KEY::
Color Optomap
Auto Fluorescence

Note that the color fundus images exhibit only a minor difference between the two eyes A 63 yo BF patient presented for follow up of diabetic retinopathy previously treated with
but the PAF differences are dramatic and unmistakable. focal laser OU. The patient denied having any difficulty seeing at night. BCVA 20/20 -2
OD and 20/30+2 OS.

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8/6/2012

Case 3: Optomap FAF Image OD Case 3: Optomap Color Fundus and FAF Comparison OD

There is also a peri-macular ring of hyper AF.

Note the large hypo AF zone around the disc with an inferior arcuate extension into the temporal retina.

Case 3: Optomap Color Fundus Image OS Case 3: Optomap FAF Image OS

Note very similar findings in the left eye.

Case 3: Optomap Color Fundus and FAF Comparison OS Case 3: Optomap FAF Comparison OD and OS

In the color fundus image, note the


hard exudates which are typically in
the outer plexiform layer. Hard Note the remarkable symmetry in the
exudates in Henles Fiber Layer tend PAF images.
to form a macular star. As expected,
hard exudates are virtually invisible
with FAF, since FAF is essentially a
RPE phenomena.

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Case 3: Optomap OD and OS Case 4: Optomap Color Fundus Image OD


KEY::
Color Optomap
Auto Fluorescence

Such symmetry suggests a retinal degeneration. This is most likely a case of peri-central A 60 yo HM who is being followed for primary open angle glaucoma and RP. He notes severe visual field
RP which often has an autosomal dominant inheritance pattern and typically progresses constriction and on occasion reports that he walks into walls. BCVA is 20/30 OU. The flash ERG was extinguished
(flat) in both eyes. The fundus appearance is typical of RP and the flat ERG confirms the diagnosis.
slowly. We have recommended ERGs and genetic testing.

Case 4: Optomap FAF Image OD Case 4: Optomap Color Fundus and FAF Comparison OD

The hypo AF zones are not


predictable from either
ophthalmoscopy or Optos
color fundus images

PAF reveals large, mid-peripheral round and oval dark zones of various sizes.

Case 4: Optomap Color Fundus Image OS Case 4: Optomap FAF Image OS

Similar PAF findings are revealed in the left eye. Note also the hyper AF areas (both eyes) surrounding the macula
which suggest that this large zone is under metabolic stress. Such zones of hyper AF most often progress to
zones of hypo AF. Initially, it appears as if the RPE is stressed before cell death.

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Case 4: Optomap Color Fundus and FAF Comparison OS Case 4: Optomap FAF Comparison OD and OS

Similar, comparative findings between


color fundus and FAF are revealed in the
left eye.
Note the symmetry of the PAF
images which is quite typical of
retina degenerations.

Case 4: Optomap OD and OU Case 5 : Topcon 3D OCT Fundus Image OD


KEY::
Color Optomap
Auto Fluorescence

A 25 yo WM presented with blurred vision and difficulty reading in each eye for the past
month although his BCVA is 20/20 OD and 20/20 OS. The patient reported that he
began using Accutane 2 months prior to vision loss. Accutane has recently been
discontinued.

Case 5 : Optomap Color Fundus OD Case 5: Optomap FAF OD

Ophthalmoscopy, standard fundus photography and optos color, red separation and
green separation images were all normal. In contrast, the PAF images reveal a peri-foveal ring of hypo AF in each eye.

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Case 5 : Topcon 3D OCT Horizontal Raster Image OD Cirrus High Resolution Horizontal Scan Image of Normal Control OD

Normal PIL Present


ELM Present

Patient PIL Present


ELM Present
Although the retinal pigment epithelium (RPE) is
External only a single layer of cells, it appears as two
Limiting Membrane
PIL reflective layers with a dark zone in between on
OS/RPE
high definition OCT scans. The inner reflection has
been referred to by some authors as the outer
PIL Missing segment-RPE interdigitation (OS/RPE) or Verhoeffs
membrane. The outer reflection is the RPE/Bruchs
membrane complex (RPE/BM). Note the two
reflections from the RPE complex are only visible
The top SD OCT from a control subject reveals a normal photoreceptor integrity line (PIL) that extends
uniformly across the 6 mm scan.
on high definition scans. On lower resolution OCT
RPE/BM
Below is a horizontal scan through the fovea of the affected right eye. Note that the PIL is present under scans the RPE generally appears as one solid thick
the fovea, is absent perifoveally and reappears beyond the perifoveal area. RPE Complex band.

Case 5: Topcon 3D OCT Fundus Image OS Case 5 : Optomap Color Fundus OD

Similarly, ophthalmoscopy, standard fundus photography, Optos color, green separation


and red separation images were all normal in the left.

Case 5: Optomap FAF OS


Case 5 : Topcon 3D OCT Radial Raster Image OS

Normal PIL Present


ELM Present

Patient PIL Present


ELM Present

PIL Missing

A horizontal section through the fovea of the left eye reveals similar findings as displayed previously in the right
eye.
A small, but intact, PIL is present under the fovea and a perifoveal absence of the PIL is documented. With loss of
The PAF image in the left eye reveals a peri-foveal ring of hypo AF as well. the PIL, the intact external limiting membrane (ELM) appears to drape over the missing tissue.

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Case 5: Fundus Comparison OD Case 5: Fundus Comparison OS


KEY:: KEY::
Topcon 3D OCT Topcon 3D OCT
Fundus Photo Fundus Photo
Color Optomap Color Optomap
Green Separation Green Separation
Auto Fluorescence Auto Fluorescence

As in the right eye, only the AF image reveals an abnormality in the left eye.
Only the AF image reveals an abnormality in the right eye.

Case 5: Optomap plus with Resmax FAF OD and OS


Case 6: Optomap Fundus Autofluorescence Image of Left Eye

The AF Bulls Eye Maculopathy above is quite symmetric and could represent a retinal
toxicity, a cone degeneration or an early cone- rod degeneration. Flash ERGs were
performed and were normal under all conditions, effectively ruling out a cone or a cone-rod
degeneration. Optomap image of the left eye of a patient with geographic atrophy of the RPE several disc
The findings are rather typical of plaquenil toxicity but the patient has never been on diameters in size. Note the choroidal vessels which are obvious within the lesion because of the
plaquenil. dropout of the overlying RPE and choriocapillaris. In addition to drusen nasal to the disc, note
widespread peripheral drusen. *Courtesy of the Reykjavik Eye Study

Case 6: Optomap plus with Resmax Color Fundus Image of Left Eye Case 6: Optomap plus with Resmax OS

Note that the


hypo AF
lesions
nasally do not
correspond to
the drusen
and hence
the AF
images are
yielding
additional
information.

At higher magnification the underlying choroidal vessels are more easily visualized
within the macular lesion.
*Courtesy of the Reykjavik Eye Study *Courtesy of the Reykjavik Eye Study

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8/6/2012

Case 7: Optomap Color Fundus Image OD Case 7: Optomap FAF OD

An 89 yo WF presented for f/u for choroidal neovasculairzation in the OS which was treated with laser 6 years The PAF image in the right eye has some correspondence with the color image.
previously. The BCVA remains 20/30 in each eye. Although the macula has no drusen or pigmentary changes, the
temporal retina displays drusen and the nasal retina reveals pigmentary clumping.

Case 7: Optomap plus with Resmax FAF OD Case 7: Optomap Color Fundus Image OS

The dark spots below the disc are in different positions in each image and represent In the left eye which was treated years earlier with laser, the retina, RPE and some of the choroid
has been destroyed. The CNV membrane was successfully eliminated and the fovea was spared.
remnants of a PVD.
See RR #32 for microperimety and SD OCT findings confirming this.

Case 7: Optomap FAF OS Case 7: Optomap plus with Resmax FAF OS

The AF image demonstrates hypo AF in the treated lesion, confirming the destruction of The preserved central vision in the left eye is due to the skill of the retinal surgeon
the RPE. Peripheral findings are similar to those in the right eye. combined with a little bit of good fortune. The CNVM has not recurred as well.

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Case 7: Optomap plus with Resmax FAF OD and OS Case 8: Optomap Color Fundus Image OD

Although the visual acuity is the same in each eye, the appearance of the central retina
is dramatically different. This is a case of similar peripheral abnormalities but of different
macula involvement.
Does peripheral drusen and pigmentary changes increase the risk of macula 35 yo HM with a history of dots and spots in his fundus over the past decade. BCVA has remained 20/20 OU
degeneration? throughout this period. The appearance of the fundus is rather typical of Stargardt Macular Degeneration with
Fundus Flavimaculatus. Note that the spots are not round as the vast majority of drusen are, but are fish tail or
pisciform in shape. Without a beaten bronze fovea and normal VA , the clinical diagnosis is Fundus
Flavimaculatus.

Case 8: Optomap plus with Resmax FAF OD Case 8: Optomap plus with Resmax OD

The hyper AF spots suggest that the RPE cells in these areas are stressed. These sick
Note that the FAF images accentuate the abnormalities, especially the arcuate peri- cells may go on to die and the corresponding spots will change from white to black.
foveal black zone which signifies death of RPE cells.

Case 8: Optomap Color Fundus Image OS Case 8: Optomap plus with Resmax FAF OS

Similar findings are displayed in the left eye. In addition to the retinal findings, note the tilted discs Equally dramatic AF images are exhibited in the left eye as well.
with very large cups. Pressures have remained normal and the peripapillary RNFL is normal and
has not changed in over a decade. The patient is still being monitored as a glaucoma suspect.

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Case 8: Optomap plus with Resmax OS Case 8: Optomap plus with Resmax FAF OD and OS

Note the relative symmetry between the two eyes suggestive of a retinal degeneration. Genetic
As in the right eye, the AF images are far more dramatic than the color images. testing did not reveal an abnormality in the ABCA4 gene and hence only a clinical diagnosis of
Stargardt Disease (or really Fundus Flavimaculatus) can be made at present. Another case of
Stargardt Disease can be found in and includes detailed SD-OCT images.

Case 9: Optomap Color Fundus Image OD Case 9: Optomap FAF Image OD

Case 9: A 56 year old, 5 diopter myopic Hispanic female presented for examination as a glaucoma suspect.
BCVA is 20/20-2 in the right eye and 20/400 with eccentric viewing in the left eye. In the color fundus image,
note the visibility of the choroidal vessels, most likely due to myopic stretching . Note the nasal, large
arcuate pattern of increased visibility of choroidal vessels, most likely due to a posterior staphyloma. AF image is essentially within normal limits.

Case 9: Optomap Fundus Image OD


Case 9:
1: Optomap Natural
Color Fundus
Fundus
Image
Image
OSOD
KEY:
Natural Optomap
Color Optomap
Auto Fluorescence

In addition to similar findings in the fundus of the right eye, the left eye exhibits some
areas of pigmentary migration most marked in the mid temporal region. With standard
fundus photography of 40-50 degrees of the central fundus, none of these pigmentary
changes are imaged.

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Case 9: Optomap FAF Image OS Case 9: Optomap Fundus Image OS


KEY:
Natural Optomap
Color Optomap
Auto Fluorescence

Note the dramatic FAF in the left eye. Large black (hypo AF) circular and oval zones represent loss of the
RPE and lipofuscin. A similar hypo A F zone is surrounding the disc. However, the central part of the image
exhibits hyper AF which is suggestive of metabolically stressed RPE.

Case 9: Optomap Color Fundus and Natural Fundus OD and OS Case 9: Optomap Color Fundus and FAF Comparison OD and OS
KEY: KEY:
Color Optomap Color Optomap
Natural Optomap Auto Fluorescence

Note that the color fundus images exhibit a modest difference between the two eyes but the PAF differences are dramatic
and unmistakable.
Recently obtained review of old records suggest that a unilateral ophthalmic artery occlusion secondary to facemask
anesthesia as a traumatic event was the etiology .
Some clinicians prefer the Natural image to the traditional Color image.

Case 10: Optomap Color Fundus Image OD Case 10: Optomap FAF Image OD

Case 10: A 45 year old Hispanic female presented with a recent vision blur in her right eye. She also reported that her vision
reduction in her left eye dated back about 3 years and was due to plaquenil toxicity. The patient was taking a long list of
drugs for lupus (SLE) but she discontinued the plaquenil after 2.5 years because of the vision reduction. The diagnosis of Best corrected VA was 20/80 in the right eye. The FAF image reveals 3 zones of hypo AF (red arrows) and
lupus was first made 19 years earlier and oral steroids have been used for virtually the entire 2 decades. She also reported hyper AF zones within the vascular arcade. In addition, a large stippled zone of hyper AF is also visible.
kidney failure, systemic hypertension and dialysis several times a week. (green border)

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Case 10: Optomap Color Fundus Image OS Case 10: Optomap FAF Image OS

The Panoramic AF image reveals that approximately 3 quarters of the fundus exhibits hypo AF suggesting non
The BC VA in the left eye was counting fingers are 6ft. viable or dead RPE. There is a zone of hyper AF suggesting that the RPE here is stressed. Near normal AF is
present superiorly. Plaquenil toxicity, as reported by the patient, is an unlikely etiology of this retinopathy.

Case 10: Optomap OD and OS Case 10: Optomap and Topcon 3D OCT Images OD
KEY:
Color Optomap
Normal
Auto Fluorescence

The SD OCT in the right eye reveals a large serous detachment of the neurosensory retina which includes
the macula. (A case series of central serous retinopathy complicating SLE has
been reported)1
Case 2

CirrusTM HD-OCT Horizontal Scan Image of Normal Control OD


Histological Section as Compared to the OCT Image

Histological Section Normal OCT Image

PIL

Inner
Segments
Although the retinal pigment epithelium (RPE) is Outer
External only a single layer of cells, it appears as two Segments RPE
Limiting Membrane
PIL reflective layers with a dark zone in between on
OS/RPE
OS/RPE
high definition OCT scans. The inner reflection has Retinal Pigment
been referred to by some authors as the outer Epithelium
segment-RPE interdigitation (OS/RPE) or Verhoeffs Although the photoreceptor integrity line, or the PIL (defined as the junction between the inner and outer
membrane. The outer reflection is the RPE/Bruchs segments) is barely visible in most histological sections, it is highly prominent in normal SD OCTs. The PIL, as
membrane complex (RPE/BM). Note the two shown above, should be continuous throughout the entire scan in normal eyes. The PIL is considered by some as
a mere artifact that is due to the difference in the index of refraction of the inner and outer segments but this
reflections from the RPE complex are only visible
artifact is remarkably useful in SD OCT interpretation.
on high definition scans. On lower resolution OCT
RPE/BM
scans the RPE generally appears as one solid thick * The PIL as Revealed by SD OCT is available at: http://www.lulu.com
RPE Complex band.

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Case 10: Optomap and Topcon 3D OCT Images OD Case 10: Optomap and Topcon 3D OCT Images OS

Normal

In contrast, the SD OCT of the left eye fails to reveal any large fluid elevations. In addition, the PIL is not present and hence
This horizontal OCT section in the right eye is below the fovea and reveals 2 well defined pigment epithelial explains the very poor visual acuity. Long standing fluid in central serous can lead to loss of the PIL but the photoreceptor
loss persists even after the fluid is eventually reabsorbed. Note that lesions inferior to the macula are common in CSCR and
detachments along with the larger serous detachment of the neurosensory retina and also edema residues.
are likely due to long term accumulation of fluid inferior to the initial leakage which then drips down because of gravity.
These findings confirm a diagnosis of central serous chorioretinopathy (CSCR) in the right eye. (Visual loss Note that the last zone to be affected is the superior quadrant since the drip begins inferiorly and eventually spreads
from CSCR in systemic lupus has been reported. ) superiorly.
Case 10 Edema Residues
PED Case 10

Case 10: Optomap and Topcon 3D OCT Images OS Case 10: Optomap FAF Image OD and OS

An OCT section inferior temporal to the macula


through the lesion reveals it to be elevated and
likely due to a fibro-vascular scar secondary to
previous choroidal neovascularization (CNV).
Chronic CSCR is known to sometimes result in
CNV.

Case 10
Although males get CSCR nearly ten times as often as females, females are far more likely to
have an auto-immune disease such as SLE. Others have demonstrated that CSCR as a
manifestation of SLE can be caused by various factors: systemic hypertension, renal disease,
RPE dysfunction and glucocorticoid therapy.1 All are applicable in our patient. It is now well
established that corticosteroids are a significant risk factor for CSC. 3 Other far less likely
diagnoses are certainly still plausible.

Case 11: Optomap Color Fundus Image OD Case 11: Optomap Fundus Auto Fluorescence Image OD

Case 11: A 47 year old black male presented on referral because of progressive difficulty seeing at night that began at most a Although pigmentary atrophy, hypertrophy and RPE migration was observable clinically and with the
decade earlier. He also reported some difficulty with reading in either eye. No family history of any significant eye problems Optomap color, red separation and green separation images, the most dramatic images were obtained with
was revealed. Best corrected VA was 20/30 with eccentric fixation in the right eye and 20/50- with eccentric fixation in the FAF. The bulls eye lesion in the macula was not at all apparent with the standard images but jumped out
left eye.
with FAF.

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Case 11: Optomap Color Fundus Image OS Case 11: Optomap Fundus Auto Fluorescence Image OS

The findings are quite similar in the left eye as well.

Case 11: Optomap OD Case 11: Optomap OS


KEY: KEY:
Color Optomap Color Optomap
Red Separation Red Separation
Green Separation Green Separation
Auto Fluorescence Auto Fluorescence

Case 11: Optomap OD and OS Case 11: Optomap and Topcon 3D OCT Images OD
KEY: In contrast to a
Color Optomap
Normal horizontal SD OCT
Auto Fluorescence
through the fovea in a
normal patient above,
the PIL is present under
Around the the fovea but thins and
bulls eye then collapses onto the
macula lesion, RPE several degrees
note that away from the fovea.
much of the The first change may be
posterior pole the progressive
revealed shortening of the outer
hyper AF segments.
ovals and
symmetric
lesions OU. PIL Present

PIL Absent PIL Absent

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Case 11: Optomap and Topcon 3D OCT Images OS Case 12: Optomap Color Fundus Image OD
Very similar findings
are present in the left
eye. The patient has a
retinal degeneration
which exhibits
symmetry between
the eyes and slow
progression OU.
Genetic analysis for
known RP defective
genes was
recommended. Also
note the attenuated
arteries, a very
common finding in
retinal degenerations.

PIL Present

Case 12: A 73 year old white female presented on referral because of unusual photopsias that the patient described as
looking like a golden PAC-MAN that moved across her visual field in her right eye. The symptom waxed and waned for
nearly a decade. Best corrected VA was 20/25 in each eye. General health history was unremarkable. Visual fields
PIL Absent PIL Absent revealed an enlarged blind spot in the right eye and a small scotoma below fixation in the left eye.

Case 12: Optomap Fundus Auto Fluorescence Image OD Case 12: Optomap Color Fundus Image OS

The FAF revealed a large hypo FAF zone around the disc and some small hyper AF spots
surrounding the lesion. Much of the temporal and inferior retina revealed a large hypo AF zone.

Case 12: Optomap Fundus Auto Fluorescence Image OS Case 12: Optomap OD
KEY:
Color Optomap
Red Separation
Green Separation
Auto Fluorescence

FAF revealed a one disc diameter hypo AF lesion within or near the superior arcade above the
macula.

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Case 12: Optomap OS Case 12: Optomap OD and OS


KEY: KEY:
Color Optomap Color Optomap
Red Separation Auto Fluorescence
Green Separation
Auto Fluorescence

Lack of
symmetry in
the two eyes
suggests that
this is not an
inherited
retinal
degeneration.

Case 12: Topcon 3D OCT Scan OD Case 12: Optomap and Topcon 3D OCT Images OD

Normal

Although the PIL appears normal under the fovea in this horizontal section, note the zone of a Although there is no perfect correspondance
poorly defined PIL and a zone with a fenestrated PIL between the fovea and the disc. between Topcon and Optos images (because of a
subtle increase in magnification outside the
Case 12 fovea with Optos), comparisons can be made
but are not as precise as desired. The PIL
PIL Poorly Defined appears quite attenuated where the FAF
demonstrates hypo FAF as shown above.

PIL Fenestrated

Case 12: Optomap and Topcon 3D OCT Images OD Case 12 : maia Sensitivity Map and Zeiss VF OS

3/2011
Based upon the subjective
photopsias and the large blind
spot, a diagnosis of AZOOR
(Acute Zonal Occult Outer
retinopathy) is quite possible.5
Although AZOOR often begins
invisible to ophthalmoscopy, it
often progresses and changes
in the RPE can be detected with
FAF. The etiology of AZOOR is 11/2011
not known but some believe it
to be an autoimmune disorder.
Several treated cases suggest
that treatment with a drug such
as Imuran may be beneficial to
prevent further progression.

Based upon the maia microperimeter which appears to demonstrate progressive loss of sensitivity over a
half year period (greater in the right eye than the left), the patient was informed and is now considering
seeing a rheumatologist for a workup and Imuran therapy.

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Case 13: Optomap Natural Fundus Image OD Case 13: Optomap Fundus Auto Fluorescence Image OD

Case 13: A 64 year old Hispanic female presents for a routine follow-up for a retinal disorder in each eye. As
previously, she denies having any visual difficulties. Both her day and her night vision are reported to be OK FAF reveals hypo AF in zone corresponding to the vascular arcades superior temporal and inferior temporal
with no change. No family members are reported to having any visual problem. Best corrected visual acuity
and a large area of dense hypo AF nasally. In addition, there is a ring of hyper AF immediately within the
is 20/30+ in the right eye and 20/25- in the left eye. arcades. The far periphery appears to be within normal limits.

Case 13: Optomap OD Case 13: Optomap Fundus Auto Fluorescence and Topcon 3D OCT OD
KEY:
Natural Optomap
Color Optomap
Auto Fluorescence

The PIL is somewhat disorganized under the fovea, normal in the perifoveal
area and appears to collapse away from the fovea.

The appearance of the fundus is Case 13


dramatic under all three image
conditions.

Case 13: Optomap Fundus Auto Fluorescence and Topcon 3D OCT OD Case 13: Optomap Fundus Auto Fluorescence and Topcon 3D OCT OD

Pigment migration to blood vessels in the inner retina is characteristic of many retinal
Hyper AF appears to correspond to the zones degenerations.
where the PIL becomes attenuated and then
collapses onto the RPE. This is best appreciated Pigment Migration
nasally.

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Case 13: Optomap Natural Fundus Image OD Case 13: Optomap Fundus Auto Fluorescence Image OS

Very similar findings are revealed in the left eye.

Case 13: Optomap OS Case 13: Optomap Fundus Auto Fluorescence and Topcon 3D OCT OS
KEY:
Natural Optomap
Color Optomap
Auto Fluorescence

Similar findings here as well. The PIL is disrupted under the fovea and thins away from the macula. The diagnosis is
pericentral RP, a mild form of RP which is most often autosomal dominant. Attempts to evaluate family members, perform
ERGs and obtain genetics are presently underway. As in the right eye, this is most marked nasally here in the left eye as
well.

Case 14: Optomap Color Fundus Image OD Case 14: Optomap Color Fundus Image OD

Case 14: A 50 year old Hispanic female presented on referral for an unusual, localized pigmentation in each
eye. She had no family history of night blindness or any other serious eye problem.
Best corrected VA was 20/20- in each eye. Note the bone spicule pigmentation localized to the inferior
retina in the right eye. The crescent shaped bright zone is an artifact since it is not present in the next
image.

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Case 14: Optomap Fundus Auto Fluorescence Image OD Case 14: Optomap Fundus Auto Fluorescence Image OD

In the FAF images, there appears to be a zone of hyper AF between the pigmented area and the posterior
pole. This hyper AF indicates that the condition is active and will likely progress more posteriorly.

Case 14: Optomap Color Fundus Image OS Case 14: Optomap Fundus Auto Fluorescence Image OS

Similar findings are revealed in the left eye.

Case 14: Topcon 3D OCT Scan Images OD Case 14: Topcon 3D OCT Scan Images OD

A horizontal SD OCT
Normal section through the fovea Pigment Migration
OD reveals a normal PIL
under the fovea.
However, the PIL appears
to thin away from the
fovea.

Case 14

PIL RPE migration to the retinal vessels in the inner retina is revealed in this SD OCT section.

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Case 14: Topcon 3D OCT Scan Images OS Case 14: Topcon 3D OCT Scan Images OS

*PIL Missing RPE Present

Normal Similar findings are


revealed in the left eye.

In the section above, slightly posterior to the band of hypo AF, the OCT reveals that the PIL is absent.
Recommendations for a follow up evaluation were made and include a flash ERG and genetic testing.
Evaluating other family members was also suggested.
Case 14
PIL Pigment Migration

OD vs. OS AF OD vs. OS ResMax AF

OD vs. OS AF ResMax OD vs. OS AF

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OD vs. OS AF ResMax OD vs. OS AF

OD vs. OS AF ResMax OD vs. OS AF

OD vs. OS AF OD vs. OS AF

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OD vs. OS AF ResMax OD vs. OS AF

OD vs. OS AF ResMax OD vs. OS AF

OD vs. OS AF OD vs. OS AF

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OD vs. OS AF OD vs. OS AF ResMax

OD vs. OS AF OD vs. OS AF

OD vs. OS AF ResMax OD vs. OS AF

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OD vs. OS AF ResMax OD vs. OS AF

OD vs. OS AF ResMax OD vs. OS AF

OD vs. OS AF ResMax OD vs. OS AF

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OD vs. OS AF ResMax OD vs. OS AF

OD vs. OS ResMax AF OD vs. OS AF

OD vs. OS AF ResMax OD vs. OS AF

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OD vs. OS AF ResMax OD vs. OS AF

Comments and Conclusions


PAF allows 200 degrees of the fundus to be imaged without
dilation in a single 250 msec flash.
PAF documents the integrity of the RPE (and overlying
photoreceptors) throughout the entire image.
PAF abnormalities occur in myriad retinal disorders.
No other technology -including OCT and BIO-is capable of
providing this level of assessment of the RPE.
PAF reveals abnormalities often invisible to ophthalmoscopy.
Correlation of these myriad FAF patterns with genetics is a
future goal.
Additional studies are required to determine the clinical
significance of these remarkable images.

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