Ophthalmology Book

Diagnostics in
Macular Disorders
(An Overview of FFA, ICGA and OCT)
Diagnostics in
Macular Disorders
(An Overview of FFA, ICGA and OCT)
S Natarajan DO
Sumita Sharma DNB (Ophthal) FMRF
Hitendra B Mehta MS FMRF
Aditya Jyot Eye Hospital Pvt. Ltd.

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Diagnostics in Macular Disorders

2005, S Natarajan, Sumita Sharma, Hitendra B Mehta
All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or
transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or
otherwise, without the prior written permission of the authors and the publisher.
This book has been published in good faith that the material provided by authors is original. Every
effort is made to ensure accuracy of material, but the publisher, printer and authors will not be
held responsible for any inadvertent error(s). In case of any dispute, all legal matters are to be
settled under Delhi jurisdiction only.
First Edition: 2005

ISBN 81-8061-485-9
Typeset at JPBMP typesetting unit
Printed at Paras Offset
Preface
In disorders of the macula, along with a detailed clinical examination, modern day ophthalmologists
have come to depend heavily on allied investigations like Fluorescein and Indocyanine Green
Angiography along with Optical Coherence Tomography. These investigations provide valuable
information, thus helping the clinician reach to an accurate diagnosis. They have also provided
additional information, which has bettered the understanding of the disease pathogenesis, which in
turn has brought about a change in the management and treatment of the respective diseases.
The aim of this book is to provide a quick reference guide for each of the macular disorders and
their features as seen as FFA, ICGA and OCT. All photographs without exception are from cases
seen at Aditya Jyot Eye Hospital Pvt. Ltd., Mumbai. A detailed legend is given for all photographs.
An attempt has been to make the book as user-friendly as possible.
This book would not have been possible but for the invaluable help provided by our Fellows
Dr Chaitra Jayadev, Dr Santosh Mahapatra, Dr Hemant Todkar and Dr Dharmesh Kar. Our sincere
thanks to them.
Special thanks go to Ms. Laxmi, our Academic Secretary. The entire credit of getting this book
together goes to her.
Our sincere thanks to our publishers, Jaypee Brothers Medical Publishers (P) Ltd., New Delhi.
We would also like to thank all our patients for entrusting themselves to us.
S Natarajan
Sumita Sharma
Hitendra B Mehta
Contents
1. Introduction ........................................................................................................ 1
2. Age-Related Macular Degeneration (ARMD) ................................................... 11
3. Choroidal Neovascular Membranes (CNVM) other than

Age-Related Macular Degeneration (ARMD) ................................................... 31
4. Idiopathic Polypoidal Choroidal Vasculopathy (IPCV) .................................. 36
5. Parafoveal Telangiectasia (PFT) ....................................................................... 40
6. Diabetic Retinopathy ........................................................................................ 46
7. Retinal Vascular Occlusions ............................................................................ 53
8. Central Serous Chorioretinopathy (CSCR) ..................................................... 60
9. Epiretinal Membrane (ERM) ............................................................................. 63
10. Macular Hole .................................................................................................... 66
11. Heredomacular Degenerations (HMD) ............................................................. 69
12. Inflammatory Diseases of the Retina and Choroid ....................................... 75
Index ..................................................................................................................................... 77
Foreword
The field of macular diseases has seen very exciting progress in recent years. We
have new diagnostic procedures that have quickly become important in the
management of macular diseases. Our therapeutic options have also dramatically
improved. Over the past several years, we have been using photodynamic therapy
extensively in the management of subfoveal choroidal neovascularization. Now,
just prior to the publication of this text, the Food and Drug Administration of
the United States approved the anti-vascular endothelial growth factor drug Macugen for all wet
(exudative) age-related macular degeneration (AMD). These therapeutic advances have increased
the importance of our optimal understanding of complex macular diseases such as AMD.
This book provides a concise, clearly written outline of the essential elements in common diseases
of the macula. It describes the important roles certain diagnostic procedures play in the evaluation
of these diseases. These diagnostic tests include fluorescein angiography, indocyanine green
angiography, and optical coherence tomography. The principles of these techniques are skillfully briefly
reviewed. Their ability to detect, precisely measure, demonstrate changes over time, and otherwise
facilitate our optimal evaluation of macula diseases is emphasized. Both these diagnostic procedures
and the diseases that are being evaluated are extensively illustrated by excellent figures.
I have had the privilege of visiting Dr. Natarajans program in Mumbai and witnessing first hand
his commitment to providing state-of-the-art ophthalmological care to the people of India. Together
he and I presented what was, to my knowledge, the first symposium in India on photodynamic therapy.
This text is another major contribution Dr. Natarajan has made to enhance the care of retinal diseases
in India. I congratulate him for providing a well organized, cogent, timely book that will be valuable
to ophthalmology clinicians and residents in training, enhancing their ability to provide excellent
evaluation and management of their patients macular diseases.
Lawrence J Singerman MD, FACS

Clinical Professor of Ophthalmology
Case School of Medicine, Cleveland, Ohio, USA
Voluntary Professor of Clinical Ophthalmology
Bascom Palmer Eye Institute, Miami, Florida, USA
Introduction
C HAPTER
1 Introduction
Introduction
FUNDUS FLUORESCEIN ANGIOGRAPHY (FFA)

Fundus fluorescein angiography (FFA) is the study of retinal and choroidal vasculature using fluorescein
dye.
Features
Fluorescence is a physical property of certain substances that, on exposure to light of short wave-
length, emit light of longer wavelength in a characteristics spectral range. Sodium fluorescein, a yellow-
red substance, absorbs light between 485 and 490 nm in aqueous solution and exhibits a maximum
emission between 525 and 530 nm.
Method
A 5 ml bolus of (1 gm {20 %} in 5 ml) sodium fluorescein dye is rapidly injected via the antecubital
vein, and rapid retinal photographs are taken with a fundus camera containing an excitatory filter
with maximum transmission between 485 nm and 500 nm and a barrier filter peaking close to the
maximum of the fluorescein.
Principle
Eighty percent of the dye is bound to plasma protein and not available for fluorescence, remaining
20 percent of unbound fluorescein is responsible for the fluorescence. Angiography is based on the
fact that fluorescein dye leaks freely from the normal choriocapillaries but does not penetrate healthy
RPE and normal retinal capillaries because of the tight endothelial junction present in the latter. Under
optimum conditions, the smallest retinal capillaries (5-10 in diameter) can be seen with this
technique, a feat impossible by ophthalmoscopy or by color photography.
Adverse Reactions
The adverse reactions can be mild, moderate and severe extending from nausea, vomiting and pruritus
1
to bronchospasm, laryngospasm, anaphylaxis, circulatory shocks and a tonic clonic seizure. The
reaction might be true or pseudo-allergic, exact mechanism being poorly understood.
Normal Fluorescein Angiogram

Sequence
Preinjection
Autofluorescence
Pseudofluorescence.
Early Choroidal and Cilioretinal Artery Filling

Choroidal flush is seen 3-5 sec after the first appearance of the dye in the choroid
10-12 sec after injection, the dye is seen in the choroidal and cilioretinal artery
12-15 sec after injection: in old patients.
Retinal Arterial Filling and Increase Choroidal Filling

10-15 sec post-injection (1-3 sec postchoroidal filling)
After the central retinal artery fills, dye flows to retinal arterioles, precapillary arterioles, capillaries
and then the retinal veins.
Retinal A-V Filling and Full Choroidal Filling

15-20 sec post-injection (5-10 sec after choroid filling)
Dye flows from venules into veins resulting in Laminar flow. With time, the laminae become
thicker and finally fuse.
Full Retinal AV Filling and Choroidal Filling (Fig 1.1)

20-25 sec post-injection. Perifoveal capillary network is seen clearly. Fovea appears hypofluorescent
Dye completely fills the lumen of the blood vessels.
AV Recirculation Phase and Decrease of Retinal and Choroidal Fluorescence

30 sec post-injection. Lasts for 2 1/2 min
Fluorescence within the vessels decreases.
Reduced Retinal and Choroidal Fluorescence

Late staining of disc and visible sclera. 3-5 min post-injection
2 Total emptying of major vessels seen in 10 min.
Introduction
Fig. 1.1: Mid AV phase of FFA showing normal filling of the retinal
arterioles and venules. The fovea appears hypofluorescent
Elimination Phase
Removal (intravascular30 min, extravascular60 min)
Average time of the fluorescein angiogram:
Arterial phase: 10.9 sec
Early venous: 13.0 sec
Late venous: 17.6 sec.
AV Transit Time
Period from first appearance of dye in the retinal arteries to the complete filling of veins, lasts 8-12
sec.
Abnormal Fluorescence
Hypofluorescence
Can be:
i. Blocked fluorescence: due to hemorrhage, tumors blocking underlying fluorescence, choroidal
fluorescence being blocked by lipofuschin like material in Stargardts disease.
ii. Vascular filling defect: In areas of vascular non-filling, either retinal or choroidal.
Hyperfluorescence
i. Transmitted: Due to atrophy or absence of RPE. 3
ii. Leakage: Either from neovascularization or membranes, an increase in size and intensity of the
hyperfluorescence in late phases.
iii. Pooling: Accumulation of dye in an anatomical space.
iv. Staining: Taking up of the dye by scar tissue or sclera.
INDOCYANINE GREEN ANGIOGRAPHY (ICGA)
Introduction
The choroidal circulation accounts for a significant part of the intraocular blood flow, and the use
of indocyanine green angiography has made a major contribution in adding to our knowledge of
the three-dimensional choroidal circulation.
Features
1. The water soluble, tricarbocyanine dye absorbs infrared light at 805 nm and maximally fluoresces
at 835 nm. These spectral properties make penetration through ocular pigment and media opacities
possible.
2. It is retained in the vascular space because of the 98 percent binding with globulin proteins,
allowing imaging of choroid and any associated abnormalities, as the globulin- ICG complex cannot
leak out of choroidal vessels.
Adverse Reactions
The tricarbocyanine dye contains about 5 percent iodine so it should be used with caution in patients
with a history of allergy to iodides.
The adverse reactions can be mild, moderate and severe extending from nausea, vomiting and
pruritus to bronchospasm, laryngospasm, anaphylaxis, circulatory shock and a tonic clonic seizure.
The reaction might be true or pseudo-allergic, exact mechanism being poorly understood. The adverse
reaction may be caused by sodium iodide or the ICG molecule itself.
Adverse reactions to ICG dye occur less frequently than with fluorescein (0.15-0.2% with ICG
as compared to between 1 and 10% with fluorescein)
Method
Patient Preparation
Possible iodine sensitivity needs to be ruled out, and a thorough medical history taken. Maximum
4 pupillary dilatation is required.
Introduction
Dosage
At an average, 25 mg of ICG dissolved in 2 ml of aqueous solvent provides high contrast images.
If necessary, it is possible to perform ICG angiography simultaneously with or sequential to FFA.
Photographic Technique
Preinjection monochromatic photographs: using red free filters (560 nm) or green free filters (640
nm) which enhance retinal and choroidal vasculature respectively, photographs are taken.
With the excitation and barrier filter in place, a preinjection image to identify pseudo or auto-
fluorescence is taken.
Early phase angiography: Timer is started when ICG injection is begun.

Rapid, sequential photographs (1/sec) are acquired, beginning 8 to 10 seconds after dye injection.
To get the earliest vascular filling phase, images should be acquired even before the appearance
of fluorescence.
Mid phase study: All images should be reviewed as they are taken (Fig 1.2).
Images are taken one every 2-3 seconds.
Late phase study: Images are taken at 7 min, 10 min, 12 min and 20 min (Fig 1.3).
Fig. 1.2: Mid phase of ICG angiography showing normal filling

of the choroidal vasculature. The retinal arterioles and venules
also show filling. Disc appears hypofluorescent
5
Fig. 1.3: Late phase of ICG angiography showing uniform

background choroidal fluorescence. The disc appears hypo-
fluorescent
The Normal Angiogram

The much more complex choroidal vascular architecture produces a wide variation of normal
circulation patterns. The landmarks to keep in mind are the optic disc and the choroidal watershed
zone.
Interpretation of the ICG Angiogram

It is important to examine an entire study, not just a single frame.
The stages in the normal angiogram are:
Early phase (within 2-60 sec)

Prominent filling of the choroidal arteries
The choroidal lobules are well defined at the end of the early phase
Early filling of the choroidal veins
Well-defined watershed zone seen, with hypofluorescent optic disc
Dye appears in retinal arteries.
Mid phase (1-15 min)

i. Early Midphase:
6 Watershed zone fills up
Introduction
Prominent choroidal veins

Dye seen in retinal arteries and veins.
ii. Late Midphase
Choroidal vessels are less prominent
Diffuse choroidal hyperfluorescence because of diffusion of dye from choriocapillaries
Dye present in retinal vasculature.
Late phase (15-30 min)

Choroidal vasculature seen as dark bands against the background hyperfluorescence of stained
extravascular choroidal tissue
Retinal vasculature not seen
Dye remains in abnormal tissue and appears hyperfluorescent against the background.
The first step is to determine whether an area exhibits hyperfluorescence (increased fluorescence)
or hypofluorescence (reduced fluorescence). Depending on the phase of the angiogram, the
interpretation may vary.
Features in an Angiogram
Hypofluorescence
Watershed zone, disc in late phase, choroidal arteries and veins in late phase; overlying haemorrhage.
Hyperfluorescence
Choroidal vessels in early phase, hot spot, plaque, polyps.
OPTICAL COHERENCE TOMOGRAPHY (OCT)

OCT is a non-contact, non-invasive diagnostic imaging modality that can perform high resolution
cross-sectional imaging in biologic tissues using light waves.
Since retina is accessible to external light, OCT is especially suited for retinal disorders. It provides
information regarding the retinal tomography/cross-sectional imaging that is akin to in vivo
histopathology of the retina.
Features
1. Principle: The imaging is based on the principle of Low Coherence Interferometry
A broad bandwidth near infrared light beam (820 nm) is projected on the retina. This light
gets backscattered or reflected from the microstructures, with the degree of backscatter depending
upon the refractive index of the structure. The echo time delay of the light reflected from the retina 7
is compared with that from a reference mirror at a known distance, thus leading to the phenomenon
of interference. This interference is then measured by a photodetector, and the range of time delays
compared.
A realtime tomogram is created by integrating data points over a depth of 2 mm, using a false
color scale. Red and white colors represent the highest back scattering, whereas the lowest is
represented by blue and black.
It is important to note that the colouring of different structures represent different optical
properties and not necessarily different tissue morphology.
2. It provides < 10 axial resolution and 20-50 transverse resolution, depending upon the ocular
structure being viewed.
3. Different cross sectional planes in the anterior segment or fundus are measured. Also, quanti-
tative information on dimensions of intraocular structures gives it the potential to stage disease
progression or response to therapy.
4. Because OCT images are acquired rapidly and the measurement beam is infrared, patient
discomfort is minimized.
Methods
1. It is preferable to dilate the pupil before examination.
2. Based upon the visual acuity of the patient, the internal or external fixation target is used. The
internal fixation target is reliable because of its reproducibility.
3. The various scan acquisition protocols available in the current OCT machines are specific for
the kind of information needed. They are either image processing protocols or quantitative analysis
protocols.
4. For macular diseases, the protocols useful are:
i. Line scan: The default angle is 0.The length of the scan is 5 mm. Multiple scans of different
parameters can be obtained, by changing the length and angle of the line.
ii. Radial lines: It consists of 6 to 24 equally spaced parallel line scans that can be varied in
parameters. Useful for macular scan and retinal thickness/volume analysis.
iii. Macular thickness map: Using radial lines, within a circle at a fixed diameter of 6 mm, the
retinal thickness can be measured.
iv. Fast macular thickness map: It takes 1.92 seconds to acquire six scans of 6 mm length each.
It can be used for bilateral comparative retinal thickness/volume analysis.
v. Raster lines: A series of equally spaced parallel lines placed in a rectangular box, can be used
to cover the entire area of pathology by altering the size of the box.
8 vi. Repeat protocol: It allows one to repeat any of the previously saved protocols using the same
set of parameters. Helpful for a comparative assessment with the previous scan.
Introduction
Features of the Normal Macula

The large field optical tomogram through the macula demarcates the optic nerve head and foveal
architecture along with vitreoretinal interface.
The optic nerve head is identified by its contour, with the central depression corresponding to
the optic disc cup.
The anterior and posterior margins of the retina are denoted as highly reflective layers
corresponding to the nerve fiber layer and the RPE-choriocapillaries respectively. Above the RPE-
choriocapillaries layer is the minimally reflective layer of photoreceptors, above which moderate
backscattering is seen from the outer and inner plexiform layer. The nuclear layer, like the
photoreceptors shows only minimal backscattering. The retinal blood vessels are defined by their
increased backscatter and shadowing on the reflection below. The larger choroidal vessels are seen
occasionally as minimally reflective dark lumens, below the RPEchoriocapillaris layer.
The fovea shows the characteristics thinning of the retinal layers, alongwith the angulation of retina
anterior to the photoreceptor layer. The RPE appears distinct from the choriocapillaries directly beneath
the fovea.
The vitreoretinal interface is demarcated by the contrast between the non-reflecting vitreous and
the backscattering surface of the retina (Fig 1.4).
Fig. 1.4: The normal retinal tomogram denoting the uniform

reflectivity of the retinal layers with the increased reflectivity of the
RPEchoriocapillaris layer
Interpretation
In an OCT scan, the reflectivity pattern of the scanned images is studied.
Hyperreflective
i. Hard exudates: Hyperreflective shadows in neurosensory retina that completely block the
9
reflections from underlying retina.
ii. Scar tissue and neovascular membranes: varying hyperreflectivity.

iii. Blood: If thin layer, it is hyperreflective. If a thick layer, it blocks the underlying reflections.
Hyporeflective
i. Serous fluid: optically empty space with absence of backscattering.
ii. Cystoid spaces in the retina.
SUGGESTGED READING
1. H Schatz, TC Burton, LA Yannuzzi, F Rabb. Interpretation of Fundus Fluorescein Angiography. CV Mosby, St Louis
1978.
2. Lawrence A Yannuzzi, Robert W Flower, Jason Slakter. Indocyanine Green Angiography. Mosby St. Louis, Missouri,
1997.
3. Puliafito CA, Hee MR, Schuman JS, Fujimoto JG. Optical Coherence Tomography of Ocular Diseases. Slack Inc.
1996.
4. Vishali Gupta, Amod Gupta, Mangat R Dogra. Atlas: Optical Coherence Tomography of Macular Diseases. Jaypee
Brothers Medical Publishers (P) Ltd., 2004.
10
Age-Related Macular Degeneration (ARMD)
C HAPTER
2 Introduction
Age-Related Macular Degeneration
(ARMD)
ARMD has been classified into Non-neovascular (Dry) and Neovascular (Wet or Exudative) forms
of the disease.
Non-neovascular ARMD
It includes abnormalities at the level of the RPEBruchs membrane-choriocapillaris complex that
lead to the formation of drusen and pigmentary abnormalities including focal hyperpigmentation,
RPE degeneration and geographic atrophy.
Drusen
Drusen can be:
Hard, which are less than 63 m in size, yellow white discrete deposits external to the RPE.
On FFA, hard drusen be have as pinpoint window defects.
On OCT, they are seen as focal elevation of the RPE with mildly thinned overlying retina. The
elevated RPE does not shadow the reflections below it (Fig 2.1).
Soft drusen, are greater than or equal to 63 m in diameter, have ill defined borders and vary in
size and shape (Figs 2.2 and 2.3).
On FFA, soft drusen hyperfluoresce early and either fade or stain in the late phase of the study
(Fig 2.4).
OCT images show small modulations in the contour of the retinal pigment epithelium with lack
of shadowing below the contour changes, similar to serous RPE detachment. However, unlike a PED,
soft drusen have shallow margins and a mild backscatter into the choroid.
ICG angiography, both early and late phase, for both hard and soft drusen, demonstrate focal
spots of hypofluorescence resulting from blockage by the drusen (Fig. 2.5).
Geographic Atrophy
It describes discrete areas of absent or attenuated RPE forming an areolar pattern, with thin overlying 11
retina and atrophy of underlying choriocapillaris (Fig. 2.6).
Fig. 2.1: OCT line scan through the drusen shows

irregular elevations of the RPE with no shadowing in
the underlying choroid
Fig. 2.2: Fundus photograph of the right eye

showing multiple soft drusen in the macula
Fig. 2.3: Red free photograph of the right eye

delineating the drusen
FFA shows early hyperfluorescence (background choroidal fluorescence) within the zones of
geographic atrophy. Choriocapillaris may be entirely absent. Late stage shows a persistent hyper-
12
fluorescence within the area of geographic atrophy because of scleral tissue staining (Fig. 2.7).
Fig. 2.4: Fundus fluorescein angiography of the right eye

in the mid phase showing the hyperfluorescence
corresponding to the drusen
Fig. 2.5: Indocyanine green angiography reveals areas of

hypofluorescence corresponding clinically with the drusen
Fig. 2.6: Fundus photograph of right eye showing

area of geographic atrophy at the macula.
Associated drusen seen scattered along the 13
posterior pole
Fig. 2.7: Late phase of FFA of right eye showing area of

geographic atrophy at the macula with prominent choroidal
vessels. Associated drusen seen scattered along the posterior
pole
ICG angiography in geographic atrophy shows good visibility of the choroidal vasculature in
the macula. Late phase reveals hypofluorescence in the area of atrophic degeneration.
OCT shows a thinned out overlying retina and decreased reflectivity of RPE with enhanced
choroidal layer imaging (Fig. 2.8).
Fig. 2.8: OCT through the area of atrophy revealing thinned out neurosensory
retina and enhanced shadow of the RPE choriocapillaris layer
14
Neovascular (exudative) Age-Related Macular Degeneration

Neovascular ARMD is characterized by:
Choroidal Neovascular Membrane

i. Classic
ii. Occult
a. Fibrovascular pigment epithelial detachment
b. Leakage from undetermined source
Pigment Epithelial Detachment

i. Serous
ii. Fibrovascular
iii. Hemorrhagic
iv. Drusenoid.
Classic Choroidal Neovascular Membrane

It clinically appears as a gray-green subretinal lesion that may be accompanied by subretinal fluid,
subretinal hemorrhage or overlying cystic retinal edema (Fig. 2.9).
FFA reveals the membrane as an area of bright, well-demarcated hyperfluorescence starting as
a lacy pattern in the early phase of the angiogram, with an increase in intensity and fuzziness of
the margins of the lesion in the late phase (Figs 2.10 and 2.11).
Fig. 2.9: Fundus photograph of the left eye showing subfoveal

type 2 CNVM with surrounding subretinal hemorrhage
15
Figs 2.10A and B: Mid AV phase of FFA eveals a well defined

lacy hyperfluorescence which shows an increase in size
and intensity in the late phases. The hyperfluorescent lesion
corresponds to the clinically seen lesion
ICG Angiography reveals similar features. It additionally helps in confirming the location of
the lesion as the CNVM shows hyperfluorescence, though less bright than FFA (Fig. 2.12).
OCT shows retinal thickening with mild backscattering from outer retinal layers, with disruption
16 in the RPE layer. Just anterior to the disrupted RPE is an area of moderate reflectivity with fusiform
Fig. 2.11: In the late AV phase the lacy hyperfluorescence

shows an increase in size and intensity. The hyperfluo-
rescent lesion corresponds to the clinically seen lesion
Fig. 2.12: ICG angiography does not add any further

information about the behavior of the membrane. The
features remain the same as that of FFA
Fig. 2.13: OCT reveals fusiform hyperreflective echo

at the level of the RPE-choriocapillaris layer corres-
ponding to the CNVM. Overlying subretinal fluid is 17
seen.
Fig. 2.14: Fundus photograph of the right eye showing a PED at

the fovea, with surrounding drusen. Hemorrhage is seen at the
foveal center
thickening of the reflective band corresponding to the CNVM. The lesion merges with the RPE
choriocapillaris layer (Fig. 2.13).
Occult CNVM
It cannot be clinically defined, but may present along with a classic CNVM, under subretinal blood
or as a part of the fibrovascular PED.
On FFA, occult CNVM presents as two types:
a. Fibrovascular PED: An irregular RPE elevation with stippled hyperfluorescence is seen. Persistence
of leakage or staining is seen in late phases (Figs 2.14 to 2.17)
b. Late leakage of undetermined source: It consists of areas of leakage at the level of the RPE, seen
in the late phase of the angiogram, not corresponding to an area of classic CNV or fibrovascular
PED to account for the leakage (Figs 2.18 and 2.19).
ICG angiography reveals early hyperfluorescence and leakage or staining in late phase. It helps
in defining the extent of the membrane. Based on the hyperfluorescence seen in ICG angiography,
occult CNVM is divided into:
HOT SPOT or Focal CNVM, an area of occult CNVM that is well delineated and less than 1
disc diameter (DD) in size (Fig. 2.20).
18 PLAQUE is an area larger than 1 DD in size, could be well or ill defined (Fig. 2.21).
MIXED CNVM has both hot spot and plaque.
Fig. 2.15: Red free photograph of the right eye Fig. 2.16: Infrared photograph of the right eye
Fig. 2.17: Mid AV phase of the FFA shows slow filling of

the PED. Faint irregular stippled fluorescence is seen in
the inferior part of the PED
19
Fig. 2.18: Fundus photograph of the left eye shows an area of

RPE changes at the macula with associated SRF and
extensive hard exudates trickling inferiorly
Fig. 2.19: Mid phase of the FFA showing irregular stippled

hyperfluorescence not corresponding to any clinical lesion.
Also seen is a hypofluorescence corresponding to the clinically
seen hard exudates
OCT shows an enhanced optical scatter signal at the choroidal level extending into sub-RPE space,
which is thicker than the adjacent reflection from the pigment epithelium and choriocapillaris,
consistent with a neovascular membrane. The retina overlying the lesion appears thickened and
20 elevated, with reduced reflectivity, suggesting fluid accumulation (Figs 2.22 and 2.23).
Fig. 2.20: ICG angiography picture of right eye shows hypo-

fluorescence corresponding to the PED, inferonasal hot
spot corresponds to an occult CNVM
Fig. 2.21: ICG angiography shows a plaque in the late

phase delineating the occult CNVM
Angiographic Features that can Obscure CNVMS

They may be caused by one of the following features:
21
1. Blocked fluorescence due to visible blood contiguous with the CNVM.
Fig. 2.22: OCT line scan through the PED shows elevation of the
hyperreflective shadow corresponding to the RPE with modera-
tely high backscatter beneath suggesting the presence of a
fibrovascular PED
Fig. 2.23: OCT shows a thickened area of moderate hyper-

reflectivity along and below the RPE-choroid layer corresponding
to the occult CNVM. Overlying areas of hyporeflectivity in the
sub-RPE and subretinal space suggest accumulation of
subretinal and sub-RPE fluid fluid
2. Blocked fluorescence not due to visible blood. It could be due to hyperpigmentation, fibrin or fibrous
tissue.
3. Hyperfluorescence from a serous PED.
These conditions may obscure ones ability to angiographically detect hyperfluorescence from
classic or occult CNVM. OCT provides invaluable information in these conditions as it gives us a
tomographic image of the involved area.
Retinal Pigment Epithelium Detachment (PED)

PEDs can be differentiated into the following types:
i. Serous PED
ii. Fibrovascular PED
iii. Hemorrhagic PED
iv. Drusenoid PED
22
Serous PED: Clinically seen as an area of RPE detachment (Fig. 2.24).
Fig. 2.24: Fundus photograph of the right eye shows a PED

temporal to fovea. Smaller PED are seen nasal to the fovea
FFA shows a uniform, bright hyperfluorescence in the early transit phase, increasing in intensity
but not in size in the late phase of the angiogram, with smooth and sharp boundaries (Figs 2.25
and 2.26).
ICG Angiography only reveals a minimal blockage of the normal choroidal vessels.
OCT reveals an elevation of the retina and RPE above an optically clear space. The reflections
from the choroid below the detachment are attenuated due to shadowing from the highly reflective,
detached PED (Fig 2.27). It may have associated overlying serous detachment of the neurosensory
retina, which will be seen as elevated neurosensory retina with underlying optically clear space followed
by the elevated RPE denoting the PED.
Fibrovascular PED: On clinical examination, a PED with mottled pigmentation, which may be
associated with subretinal fluid or subretinal hemorrhage, will be seen.
On FFA, it appears less fluorescent than a serous PED, with a stippled appearance to the staining
of the RPE surface seen in mid to late phase giving rise to a notch in the PED. A persistent staining
or leakage of the dye in the overlying subretinal space and adjoining area are seen in the late phase
(Fig. 2.17).
ICG reveals a hot spot or plaque underlying or adjacent to the area of stippled and persisting
fluorescence (Fig. 2.20).
OCT shows elevated RPE, along with mild to moderate backscatter persisting beneath the RPE,
consistent with the fibrovascular PED. It may have associated overlying neurosensory detachment,
or overlying cystoid macular edema (Fig. 2.22).
23
Fig. 2.25: Early AV phase of the fluorescein angiogram showing

hypofluorescence at the PED. Margins of the PED are well-
defined
Fig. 2.26: Late AV-phase of the fluorescein angiogram showing

hyperfluoresing PEDs. Margins of the PEDs are well-defined
24
Fig. 2.27: OCT reveals a elevation of the retina and RPE above an
optically clear space. The reflections from the choroid below the
detachment are attenuated due to shadowing from the highly reflective
PED
Hemorrhagic PED: It is clinically seen as PED with associated subretinal hemorrhage or sub-RPE
blood.
FFA reveals blocked fluorescence due to hemorrhage associated with hyperfluorescence within
the PED, with or without late leakage.
ICG angiography reveals focal area of hyperfluroscence below or adjacent to the PED suspicious
for CNVM. Depending upon the thickness of the blood film, there may be choroidal hypofluorescence.
OCT through the hemorrhagic PED shows elevated RPE with moderate backscatter, which
attenuates quickly with depth below the RPE. This backscatter, appearing immediately below the
raised RPE is consistent with the hemorrhage. The RPE band may be indistinguishable from
hemorrhage. There may be associated neurosensory elevation and cystoid changes in the overlying
retina.
Drusenoid PED: Clinically appears as an area of confluent soft drusen larger than 500 m with pooled
serous fluid, with overlying reticulated pigment clumping over it.
On FFA, a drusenoid PED fluoresces faintly during the transit phase, has no stippled appearance,
usually has an irregularly elevated surface and does not progress to bright hyperfluorescence or leakage
in the late phase of the angiogram. May have blocked fluorescence where the pigment overlies.
ICG angiography confirms the FFA findings and may detect the presence of a CNVM.
An OCT defines a RPE elevation with optical backscatter below the RPE, with small modulations
in the RPE contour with moderate optical reflectivity below the lesion.
Other Features of Wet ARMD

Fading CNV: It describes a CNV detected in the arteriovenous phase of the fluorescein angiogram
with fading in the late phase, so not much fluorescein staining or leakage is seen in the late phase. 25
These areas are usually not associated with overlying subretinal fluid.
Feeder vessels: The feeder vessels are identified during transit phase of the fluorescein angiogram
as vessels connected to leaking choroidal capillaris. They are seen either in post-laser treated eyes
or in untreated eyes where peripheral areas of CNV may connect through feeder vessels to the central
areas of CNVM undergoing natural scar formation. ICGA defines the choroidal connection of the
vessels.
OCT may reveal area of increased backscatter corresponding to the scarring membrane, with
associated thickening of RPE choriocapillaris layer.
Loculated fluid: Loculated fluid consists of a well-demarcated area of hyperfluorescence more in late
phase, representing pooling of fluorescence in a compartmentalized space anterior to the choroidal
neovascular leakage. It may resemble a pattern typical of cystoid macular edema, or may pool within
an area deep to sensory retina in a shape bearing no resemblance to cystoid macular oedema.
OCT shows a well-defined region of hyper-reflectivity at the level of the RPE-choriocapillaris,
consistent with the CNVM. The neurosensory retina above the membrane shows thickening as well
as focal areas of reduced optical reflectivity, corresponding with the fluid.
RPE RIP: Clinically seen as a PED with an RPE tear creating an area of RPE loss along the edges
of the PED (Fig. 2.28).
FFA displays early bright sharply demarcated hyperfluorescence within the torn region, with blocked
fluorescence corresponding to the heaped up RPE at one side of the bright area. There is associated
increased visualization of the choroidal vasculature and late staining consistent with the absent retinal
pigment epithelium (Fig. 2.29A and B).
Fig. 2.28: Fundus photograph of the right eye showing a broad

26 crescentic depigmented zone in the superotemporal macula.
The choroidal vessels seen easily in that area
Figs 2.29A and B: Mid and late AV phase of the FFA shows
a sharp edged hyperfluorescence delineating the edge of
the RPE rip.The hyperfluorescence corresponds to the
crescentic area of the lost RPE. The scrolled up RPE
blocks the background choroidal fluorescence inferior to
the hyperfluorescence giving rise to a band of hypofluore-
scence. Also seen is the outlined collapsed PED
ICG angiography shows near normal choroidal fluorescence in the area of the rip, with varying
degrees of hyperfluorescence in the area of the rolled up flap, denoting the area of CNVM. Area of
blocked fluorescence will denote the area of the rolled up flap (Fig. 2.30). 27
Fig. 2.30: Late phase ICG angiography shows the

hypofluorescence corresponding to the rolled RPE
Fig. 2.31: OCT though the lesion shows the elevated neuro-
sensory retina and RPE. In the area of RPE rip, two highly
reflective layers are seen below the sensory retina
corresponding with a folded double-layer of pigment epithelium,
with attenuation of reflections below the layer
OCT though the lesion shows the elevated neurosensory retina and RPE which shadows the
reflections from the choroid below. In the area of RPE rip, two highly reflective layers are seen below
the sensory retina corresponding with a folded double-layer of pigment epithelium, with attenuation
of reflections below the layer. In the area corresponding with absent RPE, enhanced reflectivity and
optical penetration of the choroid is noted (Fig. 2.31).
Disciform scar: The scarred CNVM forms a fibrovascular scar which hyperfluoresces both from
28 fluorescein leakage as well as staining (Figs 2.32 and 2.33).
Figs 2.32A and B: Fundus photograph and red free

photograph of the left eye showing a disciform scar
Fig. 2.33: Late AV phase of FFA of the left eye showing 29

hyperfluorescent staining of the disciform scar
Fig. 2.34: Mid phase of ICG of the left eye showing

hyperfluorescent staining of the disciform scar. No active
leakage seen
Fig. 2.35: OCT reveals a focal area of high reflectivity with

enhanced backscatter corresponding to the chorioretinal
scar. Overlying neurosensory retina shows thinning
throughout
ICG Angiography reveals a hypofluorescence corresponding with the scar, especially in the late
phase (Fig. 2.34).
OCT reveals a focal area of enhanced backscatter corresponding to the chorioretinal scar. The
overlying neurosensory retina shows thinning throughout (Fig. 2.35).
SUGGESTED READING
1. Bressler NM, Bressler SB, Fine SL. Age-related macular degeneration. Surv Ophthalmol 1988;32:375-413.
2. Folk JC. Aging macular degeneration: Clinical features of treatable diseases. Ophthalmology 1985;92:594-602.
3. Arnold JJ, Quaranta M, Soubrane G, Sarks SH, Coscos G. ICG angiography of drusen. AM J Ophthalmol 1997;124:
344-356.
4. Gass JDM. Stereoscopic atlas of macular diseases. Diagnosis and treatment. Vol 2, ed. 4. CV Mosby.
5. Destro M, Puliafito CA. Indocyanine green video angiography of Choroidal neovascularisation. Ophthalmology
1989;96:846-53.
30 6. Puliafito CA, Hee MR, Lin CA et al. Imaging of macular disease with Optical Coherence Tomography. Ophthalmology
1995;102:217-19.
CNVM other than ARMD
C HAPTER
3
Choroidal Neovascular Membranes
Introduction
(CNVM) other than Age-Related
Macular Degeneration (ARMD)
Choroidal neovascular membranes when not associated with ARMD are usually of the classic
variety.
Conditions that lead to development of these CNVM are associated with rupture in the Bruchs
membrane and disturbance in RPE. The causes include:
1. Idiopathic
2. Myopic
3. Angioid streaks
4. Postinflammatory
5. Choroidal rupture
Features of these classic CNVMs on FFA, ICGA and OCT are:
FFA: Early phase reveals a lacy hyperfluorescence that increases in size and intensity within mid
phase to late phase. The margins appear fuzzy.
ICG Angiography reveals similar features. It additionally helps in confirming the location of the
lesion as the CNVM shows hyperfluorescence, though less bright than FFA.
OCT: It reveals an area of moderate hyperreflectivity in the outer retinal areas corresponding to the
area of CNVM. The reflectivity below this lesion is attenuated. There may be associated hypo-
reflectivity in the subretinal space corresponding to subretinal fluid or in the inner retinal layers
corresponding with overlying cystoid changes in the retina.
Brief features of each include:

1. Idiopathic CNVM: Usually a classic membrane, it could be extrafoveal, juxtafoveal or subfoveal.
2. Myopic CNVM: Usually associated with lacquer cracks, these membranes are small in size,
located near fovea with minimal leakage of dye (Figs 3.1 to 3.3). ICG angiography additionally
defines the peripapillary and macular choroidal atrophy well. It delineates the choroidal vasculature
including choriocapillaries. This hyperfluorescence needs to be differentiated from staining of the
CNVM. Lacquer cracks are seen as hypofluorescent streaks (Figs 3.4 and 3.5).
31
Fig. 3.1: Infrared photograph of the left eye showing a small

subfoveal CNVM. Also seen is the myopic peripapillary
atrophic zone
Fig. 3.2: Mid AV Phase of the FFA which shows a lacy hyper-
fluorescent lesion corresponding clinically with the CNVM
3. Angioid streaks: The CNVM usually grows at the serrated edge of the angioid streaks (Figs
3.6 and 3.7) The hyperfluorescence of the CNVM is more intense than that of the streaks (Figs
3.8 and 3.9). ICG may reveal variable hyperfluorescence or hypofluorescence in the area of the
32 angioid streaks, depending upon the extent of atrophy of RPE Choriocapillaris (Fig. 3.10).
4. Postinflammatory: CNVMs usually arise from the healed edge of the choroiditis.
Fig. 3.3: Late phase of the FFA shows an increase in

size and intensity of the hyperfluorescent lesion. The
margins of the lesion have become fuzzy
Fig. 3.4: ICG angiography shows hyperfluorescence

corresponding to the boundaries of the clinical lesion
Fig. 3.5: OCT reveals a thinned neurosensory retina

with a hyperreflective lesion at the level of the RPE
Choriocapillaris layer corresponding to the subfo- 33
veal CNVM
Fig. 3.6: Fundus photograph of the right eye showing the

peripapillary and radiating angioid streaks. Also seen is
a greyish macular choroidal neovascular membrane
Fig. 3.7: Red free photograph of the right eye showing

the angioid streaks along with the CNVM.
Fig. 3.8: Mid AV Phase of FFA reveals a hyperfluorescent

lesion corresponding with the CNVM Also seen is the
34 peripapillary part of the angioid streaks seen as a
hyperfluorescent ring around the disc
Fig. 3.9: Late phase of the FFA showing hyperfluorescence at

the macula due to staining of the membrane and associated
cystoid changes of the overlying retina
Fig. 3.10: OCT through the macula reveals a moderately hyper-

reflective area in the outer layer of the neurosensory retina with
adjoining hyporeflective area suggesting the presence of subretinal
fluid. A discontinuity in the RPE choriocapillaris complex indicates
the site of break in the Bruchs membrane
5. Choroidal rupture: The FFA shows hypofluorescence in the early phases with late hyper-
fluorescence seen due to staining of the tissue at the site of rupture and underlying sclera. The
CNVM appears as an increasing hyperfluorescence with fuzzy margins in the late phase.
SUGGESTED READING
1. Hampton GR, Kohen D, Bird AC. Visual prognosis of disciform degeneration in myopia. Ophthalmology 1983;
90:923-26.
2. Clarkson JG, Altman RD. Angioid Streaks. Surv Ophthalmol 1982; 26:235-46.
35
CHAPTER
4
Idiopathic Polypoidal Choroidal
Introduction
Vasculopathy (IPCV)
IPCV, also called as Posterior Uveal Bleeding Syndrome, is associated with multiple hemorrhagic
pigment epithelium detachments and extensive hard exudates.
The lesions arise from the inner choroid plexus and consist of a network of choroidal vessels that
terminate into polyp like lesions. These polyps can be seen clinically as orange-red subretinal nodules
(Figs 4.1 and 4.2).
Fig. 4.1: Fundus photograph of the left eye showing the

exudates temporal to the macula. Central Reddish nodular
lesions seen. Also seen are associated hard drusen
36 Fig. 4.2: Red free photograph of the left eye showing the
exudates temporal to the macula
Idiopathic Polypoidal Choroidal Vasculopathy (IPVC)
Fig. 4.3: Mid AV phase of the FFA shows a stippled hyper-

fluorescence at the macula. Also seen are transmission defects
diffusely along the posterior pole
Fig. 4.4: Late Phase of the FFA shows an increased fuzzy

hyperfluorescence at the macula, especially temporal to the
disc, indicative of leakage
FFA shows areas of stippled hyperfluorescence with increasing intensity in the late phases (Figs
4.3 and 4.4).
ICG Angiography is diagnostic for IPCV, as it can delineate the choroidal network of vessels
37
and polyps. In the midphase of the angiogram, the polyps are seen as clusters at the termination
Fig. 4.5: Mid phase ICG Angiography shows a

prominent hyperfluorescent lesion temporal to the disc
suggestive of a polyp
Fig. 4.6: Late phase ICG Angiography shows the

choroidal polyp with surrounding area of hyper-
fluorescence suggestive of leakage
Fig. 4.7: OCT shows a uniformly

38 reflective RPE choriocapillaris layer with
dome shaped protrusions
Idiopathic Polypoidal Choroidal Vasculopathy (IPVC)
of the choroidal vascular complexes. These polyps fill slowly and leak intensely in the late phases.
It may also show a washout of the dye from the polyps and leakage of surrounding tissue in late
phases (Figs 4.5 and 4.6).
OCT cannot image below the RPE clearly, so the reliability of OCT for IPCV has not been definitely
established. Few researchers have mentioned a characteristics hyperreflectivity in the choroidal layers
on OCT, while others mention an anterior dome-shaped protrusion of the RPE layer, with moderate
reflectivity or a nodular appearance beneath the RPE shadow (Fig. 4.7).
SUGGESTED READING
1. Yannuzzi LA, Sorenson J, Spaide RF, Lipson B. Idiopathic Polypoidal Choroidal Vasculopathy. Retina 1990; 10(1):
1-8. 98, 105(8): 1380-85.
2. Uyama M, Wada M, Nagari Y, Matsubara T, Matsunaga H, Fukushima I, Takahashi K, Matsumura M. Polypoidal
choroidal vasculopathy: Natural history. Am J Ophthalmol 2002;133(5): 639-48.
3. Ciardella AP, Donsoff IM, Yannuzzi LA. Polypoidal Choroidal vasculopathy. Ophthalmol Clin North Am 2002;1514:
537-54.
4. Yannuzzi LA, Sorenson J, Spaide RF, Lipson B. Idiopathic Polypoidal Choroidal Vasculopathy. Retina 1990;10:1-8.
39
CHAPTER
5
Parafoveal Telangiectasia
Introduction
(PFT)
Parafoveal Telangiectasia (PFT) is characterized by the dilation of the parafoveal capillary network.
It has been divided into three types, with the capillary network behaving differently in each
type.
Group I: Unilateral Parafoveal Telangiectasia

It is seen in middle-aged men. It can be congenital or idiopathic. The temporal part of the parafoveal
capillary network is involved.
FFA shows a saccular dilatation of the temporal parafoveal capillaries with staining of tissues
in late stages.
Group II: Bilateral Acquired Parafoveal Telangiectasia

It is seen bilaterally, may involve the entire parafoveal capillary network (Figs 5.1 and 5.2). It is further
seen to progress in five stages, specific features of which include parafoveal retinal whitening, right-
angled venule, RPE hyperplasia, crystalline yellow deposits and neovascular membrane (Figs 5.3 and
5.4).
FFA shows dilated parafoveal capillaries with staining of adjacent retina in mid or late phases
that spares the fovea (Figs 5.5 and 5.6). The right-angled venules are seen to end abruptly in the
parafoveal venules. There may be associated choroidal neovascular membrane (Figs 5.7 and 5.8).
Group III: Bilateral Idiopathic Perifoveal Telangiectasia and Capillary Obliteration

It is associated with progressive obliteration of the perifoveal network.
FFA shows the obliterated perifoveal network along with some areas of retinal staining.
ICG Angiography in PFT: Highlights the areas of retinal vascular abnormalities, in the form
of a diffuse placoid hyperfluorescence corresponding with the area of retinal telangiectasia ICG. ICG
has also helped us understand the association of retinal angiomatous proliferans (RAP) with PFT
40 (Figs 5.9 and 5.10).
Parafoveal Telangiectasia (PFT)
Fig. 5.1: Fundus photograph of the left eye showing the

parafoveal retinal whitening typically seen with PFT
Fig. 5.2: Red free photograph of the left eye showing the
parafoveal retinal whitening typically seen with PFT
Fig. 5.3: Mid-AV phase of the FFA reveals parafoveal 41

capillary dilatation and telangiectasia
Fig. 5.4: Increased intensity of the hyperfluorescence sug-

gestive of leakage from the capillaries is seen, especially
in the temporal area.The foveal hypofluorescence typically
is spared
Fig. 5.5: OCT scan through the fovea shows a

hyporeflective area at the level of the RPE-
choriocapillaris complex suggesting a localised loss
of these layers.Despite the staining seen on FFA, no
increase in retinal thickness is seen, indicating the
diffusion of fluorescein in the damaged retinal cells.
Fig. 5.6: Fundus photograph the left eye showing PFT

with associated retinal angiomatous proliferans
42
Fig. 5.7: Infrared photograph of the left eye showing PFT

with associated retinal angiomatous proliferans
Fig. 5.8: Mid-AV phase of the FFA showing the capillary

dilatation and leakage of PFT with a retinoretinal anasto-
mosis
Fig. 5.9: Mid-AV phase of the FFA showing the capillary

dilatation and leakage of PFT with increased leakage from 43
the retinoretinal anastomosis
Fig. 5.10: Mid-phase ICG angiography also reveals an

anastomosis between the retinal and choroidal vessels
forming a retinochoroidal anastomosis
Fig. 5.11: Late phase ICG angiography reveals anastomosis

between the retinal and choroidal vessels forming a
retinochoroidal anastomosis
OCT in Parafoveal Telangiectasia

OCT features reveal hyporeflectivity in the outer retina suggesting a focal loss of these layers. It has
been theorized to be a loss of Muller cells (Fig. 5.11). Tomograms through hyper fluorescent areas
do not reveal any increase in retinal thickness, suggesting that the hyperfluorescence is due to diffusion
of fluorescein into the affected areas. Scans through the pigment layer reveal a hyperplasia of the
RPE.
OCT is helpful in visualizing the choroidal neovascular membranes that develop in stage V of
44 Type II PFT. OCT has been extremely useful, in adjunct to FFA to identify the level of Retinal
Fig. 5.12: OCT scan through the fovea shows a hyporeflective

area at the level of the RPE-choriocapillaris complex suggesting
a localized loss of layers. Also seen is an area of increased
reflectivity adjacent to the fovea at the level of the outer retinal
and RPE-choriocapillaris layer corresponding to the retinal
angiomatous proliferans associated with PFT
Angiomatous Proliferans, the RAP being intraretinal in stage I, subretinal neovascularisation (SRN)
in stage II, and choroidal neovascularisation (CNV) in stage III, where it is termed as retinochoroidal
anastomosis (Fig. 5.12).
SUGGESTED READING
1. Gass JD. Blodi BA. Idiopathic juxtafaveolar retinal telangiectasis: Update of classification and follow up study.
Ophthalmology 1993;100:1536-46.
2. Gass JDM. Congenital and acquired idiopathic juxtafoveolar retinal telangiectasia. In Stereoscopic Atlas of Macular
Disease: Diagnosis and Treatment. Mosby-Year Book. Inc 303-436.
45
CHAPTER
6 Introduction
Diabetic Retinopathy
Macular edema is the most common cause of treatable vision loss in patients with diabetic retinopathy.
It is seen as a consequence of abnormal vascular permeability in diabetic retinopathy.
The diagnosis of macular edema is made best by slit-lamp biomicroscopy of the posterior pole
of the eye. Clinical evaluation reveals the location of retinal thickening relative to the fovea, presence
and location of exudates and cystoid macular edema (Fig. 6.1).
Fig. 6.1: Fundus photograph of right eye showing

clinically significant macular edema
FFA helps topographically by delineating the areas of retinal capillary leakage and correlating
with areas of retinal thickening of the macula. FFA has revealed two general categories of macular
edemaFocal and Diffuse.
1. Focal macular edema is characterized by areas of focal fluorescein leakage from specific
capillary lesions. It may be surrounded with rings of hard exudates (Figs 6.2 and 6.3).
2. Diffuse macular edema is characterized by widespread retinal capillary abnormalities associated
with diffuse leakage. The leakage occurs from extensive breakdown of the inner blood retinal
46
barrier. Fluorescein angiography shows a stippled hyperfluorescence, which starts in the arterial
Fig. 6.2: Fundus photograph of the left eye showing scattered

microaneurysms, hard exudates and a few scattered dot
hemorrhages
Fig. 6.3: Mid-AV phase of the FFA showing hyperfluorescent

spots corresponding to the leaking microaneurysms
and early AV phase, increases in size and intensity to give a diffuse ground-glass appearance
in the late phases (Figs 6.4 and 6.5).
FFA may reveal the coexistence of cystoid macular edema, which, when centered on the fovea,
has a petalloid pattern (Figs 6.6 and 6.7) and when extramacular, has a honeycomb pattern.
FFA also reveals the extent of macular ischemia. The foveal avascular zone may become
irregular and enlarged because of nonperfusion. Closure of retinal arterioles may lead to large areas
of nonperfusion and progressive ischemia (Figs 6.8 and 6.9). 47
Fig. 6.4: Fundus photograph of left eye revealing cotton

wool spots,microaneurysms, hemorrhages and diffuse
macular edema
Fig. 6.5: Late phase of FFA reveals diffuse macular

edema, more in the area super-temporal to the disc
48 Fig. 6.6: Fundus picture of the right eye showing

cystoid macular edema
Fig. 6.7: Late phase FFA of the right eye showing typical
petalloid appearance of the cystoid macular edema
Fig. 6.8: Fundus photo of the right eye showing extensive

dot and blot hemorrhages, preretinal hemorrhage and
diffuse macular edema
Fig. 6.9: Late AV phase of FFA reveals extensive area

of capillary nonperfusion superotemporal to the FAZ
with loss of FAZ contour. Also seen is blocked fluores- 49
cence corresponding to the preretinal hemorrhage
ICG Angiography is less sensitive at detecting areas of retinal capillary nonperfusion because
of the background choroidal fluorescence and loss of dark background. It also does not highlight
areas of neovascularisation because of the lack of leakage of ICG dye and the less intensive staining.
Because of the multifactorial origin of diabetic macular edema, there are several manifestations
at an ultrastructural level within the retinal layers which can studied using the OCT.
OCT reveals a reduced backscattering seen mostly in the outer retinal layers, corresponding to
intraretinal fluid accumulation. Areas of increased reflectivity, seen more in the outer layers of the
retina, associated with shadowing, are suggestive of hard exudates. Intraretinal areas of hyporeflectivity
with well defined margins distributed in the outer plexiform layer; suggestive of cystoid macular edema
can also be seens (Figs 6.10 to 6.12).
Fig. 6.10: OCT through macula shows diffuse

spongy thickening of the neurosensory retina
with hyporeflectivity from outer retinal layer
suggesting intraretinal fluid collection. Also
seen are hyper-reflective lesions corres-
ponding with hard exudates

thickening of the neurosensory retina.
Associated cystic changes seen.Also seen
are hyperreflective lesions corresponding
with hard exudates

thickening of the neurosensory retina. Also seen
50 are hyper-reflective lesions corresponding with
hard exudates
Fig. 6.13: OCT scan through the macula reveals

the hyporeflective spaces in the neurosensory
retina typical of cystoid macular edema

thickening of the neurosensory retina due to the
taut posterior hyaloid.Also seen are hyper-reflective
lesions corresponding with hard exudates

thickening of the neurosensory retina. Also seen
are hyper-reflective lesions corresponding with
hard exudates.Traction on the retina from the
posterior hyaloid is causing a tractional lifting of
the neurosensory retina.
Lamellar macular holes associated with cystoid macular edema can also be seen, involving the
inner retinal layers (Fig 6.13).
A taut posterior hyaloid if not detected clinically, can be picked up on OCT as a hyper-reflective
membrane at the vitreoretinal interface. The underlying neurosensory retina may show thickening.
(Fig 6.14).
OCT is invaluable in detecting early tractional detachment of the fovea that may not be detected
clinically (Fig. 6.15).
OCT is useful in gauging effect of treatment on the macular edema. A pre-procedure OCT
comparison with a post-procedure OCT helps in detecting the degree of change in retinal thickening
and the effect on retinal cysts after the patient has undergone either focal or grid photocoagulation,
intravitreal triamcinolone acetonide injection or vitrectomy for macular edema. 51
SUGGESTED READING
1. Diabetic Retinopathy. Section V. Retinal vascular disease, Part 2. Disorders of the Retina & Vitreous. AAO. Retina
and Vitreous 2000-2001.
2. ETDRS Research Group Report No.11. Classification of diabetic retinopathy from fluorescein angiogram.
3. ETDRS Research Group: Focal photocoagulation treatment of diabetic macular oedema: relationship of treatment
effect to fluorescein angiographic and other retinal characteristics at baseline. ETDRS report No.19 Arch Ophthalmol
1995;113:1144-55.
4. Allen C Ho. Retina and Retinal Vascular lesions. Chapter 26. In Indocyanine Green Angiography. 1997, Mosby
St Louis.
5. Hee MR, Puliafito CA, Duker JS et al. Topography of diabetic macular oedema with optical coherence tomography.
6. Jaffe NS, Luscombe SM, Clayman HM, JDM Gass. A fluorescein angiographic study of cystoid macular oedema.
Am J. Ophthalmol 1981;92:775-80.
7. Fine BS, Brucker AJ. Macular edema and Cystoid Macular Edema. Am J Ophthalmol 1981;92:466-81.
52
Retinal Vascular Occlusions
C HAPTER
7 Introduction
A clinical examination usually is adequate for the diagnosis of retinal arterial or venous occlusions.
But investigation in the form of angiogram and OCT plays an important role in deciding treatment
protocol and prognosis of the occlusive pathology. The vascular occlusions can be either arterial or
venous.
Venous Occlusions
They can be:
1. Branch retinal vein occlusions (BRVO)
2. Central retinal vein occlusions (CRVO)
Branch Retinal Vein Occlusions (BRVO)

Acute phase: The ophthalmoscopic findings of acute BRVO include superficial hemorrhages, retinal
edema and cotton wool spots in the sector of retina drained by the affected vein (Fig. 7.1).
Fig. 7.1: Fundus photograph of the right eye with a superotemporal 53

branch retinal vein occulsion with hard exudates at the macula
Fig. 7.2: Mid-AV phase of the FFA reveals blocked fluorescence

corresponding to the retinal hemorrhages along with minimal
hyperfluorescence at the macula suggestive of cystic changes
FFA in the acute stage will show blocked fluorescence due to the retinal hemorrhage, alongwith
retinal capillary nonperfusion, in the distribution of the retina drained by the obstructed vein. The
veins appear dilated, and show early vessel wall staining. As the BRVO occurs most commonly at
an anteriovenous crossing, the location of the site of blockage can be located. FFA also plays a very
important role in determining the extent of macular perfusion, as the degree of macular involvement
determines the level of visual impairment. Macular involvement will be seen as either macular
nonperfusion or as presence of cystoid spaces at the macula (Fig. 7.2).
OCT helps in documenting macular thickness. A tomogram through the macula in a patient with
BRVO shows loss of foveal contour with hyporeflective spaces corresponding with serous fluid in
the neurosensory retina. There may be evidence of intervening hyper-reflective walls with optically
clear spaces inside, in the neurosensory retina, consistent with cystoid spaces in the macula. There
is an increase in the retinal thickness (Fig 7.3).
Fig. 7.3: OCT through the macula reveals hyporeflective cystic lesions in
54 the inner neurosensory retina suggestive of cystoid macular edema
secondary to RVO
Fig. 7.4: Fundus photograph of the right eye with an old supero-
temporal branch retinal vein occulsion with hard exudates
along the sector involved and at the macula
Fig. 7.5: Late phase of the FFA reveals extensive areas of

capillary dropout along with vessel wall staining along the
sector of the affected venule.Also seen is a petalloid pattern
of hyper fluorescence at the macula .
Serial OCT done following therapy in the form of laser or intravitreal triamcinolone can be used
to document patient response to therapy. OCT can also detect presence of lamellar macular hole,
and epiretinal membrane.
Chronic phase: After resolution of intravitreal hemorrhage, underlying retinal vascular abnormalities
are identified mainly by ophthalmoscopy and FFA (Figs 7.4 and 7.5).
The vascular abnormalities seen on FFA will include collaterals around blockage site, retinal capillary
55
telangiectasia throughout the involved segment and areas of capillary nonperfusion. The development
of neovascularisation can also be detected by the leaking neovascularisation from the disc or peripheral
retina.
OCT will reveal cystoid changes at the macula, the extent of which depends upon the degree
of ischemia.
Hemiretinal Vein Occlusion

When an occlusion occurs at or near the optic nerve head, the entire half of the fundus, either superior
or inferior, is involved.
The clinical features are similar to branch retinal vein occlusion, except that either the entire
superior or inferior half of the fundus is involved.
Central Retinal Vein Occlusion (CRVO)

The clinical appearance of dilated, tortuous retinal veins, swollen optic disc, intraretinal hemorrhages
and retinal edema diagnose central retinal vein occlusion (Fig. 7.6). The two modes of presentation
are:
Non-ischemic
Ischemic
In the acute phase: FFA shows a prolongation of intraretinal circulation time with breakdown of
capillary permeability and limited areas of nonperfusion in cases of non-ischemic CRVO.
Ischemic CRVO is associated with widespread capillary nonperfusion along with prolonged
intraretinal circulation time, dilated retinal veins and late stage disc staining (Fig. 7.7).
56 Fig. 7.6: Fundus photograph of the right eye reveals hyperemic

disc and extensive hemorrhages across the fundus, charac-
teristic of CRVO
Fig. 7.7: Mid-AV phase shows dilated venules and blocke-

dfluorescence corresponding with the hemorrhages across
the fundus
OCT shows retinal thickening with multiple hyporeflective areas consistent with cystic spaces in
neurosensory retina. A reduced backscattering from the outer retinal layers is suggestive of intraretinal
fluid collection.
In the chronic phase: There is development of either disc collaterals or disc neovascularisation which
can be differentiated by fluorescein angiography because of the larger caliber and lack of leakage
of the former as opposed to the profuse leakage seen with the latter.
OCT reveals cystoid changes at the macula along with intraretinal fluid collection. Comparative
serial OCT may reveal a resolution of the cystoid spaces along with a decrease of the foveal thickness.
As in BRVO, OCT will help in quantifying and gauging improvement in macular edema following
therapy.
Retinal Arterial Obstruction

Retinal ischemia results because blood supply to the inner layers of the retina comes entirely from
the central retinal artery unless a cilioretinal artery is present. The obstruction can be either:
1. Branch retinal artery obstruction (BRAO), or
2. Central retinal artery obstruction (CRAO)
Branch Retinal Artery Obstruction (BRAO)

It may not be seen ophthalmoscopically initially, but within hours, it leads to edematous opacification
of the retina along the area of supply of the retinal arteriole.
57
FFA reveals delayed arterial filling in the affected area.

OCT shows hyper-reflectivity in the inner retinal layers with attenuation of the structures below
it, corresponding to the opaque retina seen clinically. The demarcation between the hyper-reflective
and normal reflective retina is very clear. On follow up, the reflectivity of the retina decreases.
Central Retinal Artery Occlusion (CRAO)

The clinical appearance of a yellow-white edematous and opaque retina with a cherry-red spot at
the macula is diagnostic of CRAO (Fig 7.8). With time the central retinal artery reopens or recanalises
and retinal edema clears.
FFA in the acute stage reveals a delay in retinal arterial filling. The most commonly seen
angiographic sign is a delay in the retinal arteriovenous transit time (Fig. 7.9).
Fig. 7.8: Fundus photograph of the right eye revealing

a generalized retinal whitening with a cherry-red spot
at the macula.An area of normally perfused retina is
seen in the inferior peripapillary region
Fig. 7.9: FFA reveals a delayed arterial filling with low

58 dye concentrations.Also seen is a delayed AV Transit
time
Fig. 7.10: OCT through the macula reveals a hyper-reflectivity in

the inner-retinal layers, corresponding clinically with the opaque
retina. Attenuation of the signal from the underlying retinal layers
is seen.Normal reflectivity of the fovea with underlying RPE-
choriocapillaris layer is seen
Fig. 7.11: OCT through the macula reveals normalizing reflectivity

of the neurosensory retina and increased visualization of the
underlying outer retinal layers in a case of resolving CRAO
As opposed to CRVO, retinal vessel wall staining is rare. The choroidal vascular bed usually fills
normally in patients of CRAO. If a prolonged filling of choroidal vasculature in the absence of a central
cherry-red spot in seen, a suspicion of ophthalmic or carotid artery obstruction should be made.
Usually the fluorescein angiogram can revert to normal at varying times after the acute occlusion.
OCT reveals a diffuse thickening and hyper-reflectivity of the neurosensory retina throughout the
macula with loss of foveal pit. Attenuation of the underlying shadows including the RPE choriocapillaris
layer is seen. There may be elevation at the fovea, but the RPE Choriocapillaris layer can be clearly
seen at the fovea (Figs 7.10 and 7.11).
SUGGESTED READING
1. Venous Occlusive Disease. Section V. Retinal vascular disease, Part 2. Disorders of the Retina & Vitreous. AAO.
Retina and Vitreous 2000-2001.
2. Arterial Occlusive Disease Section V Retinal vascular disease, Part 2. Disorders of the Retina & Vitreous. AAO. Retina
and Vitreous 2000-2001.
3. David NJ, Gass JDM, Beauchamp J. Fluorescein Angiography in Central Retinal Artery Occlusion. Arch Ophthalmol
1967;77:619-29.
4. Spaide RF, Lee JK, Klancnik JM, Gross NE. Optical Coherence Tomography of Branch Retinal Vein Occlusions.
Retina 2003;343-47.
59
CHAPTER
8
Central Serous Chorioretinopathy
Introduction
(CSCR)
Central Serous Chorioretinopathy (CSCR) is an idiopathic serous detachment of the macula due
to accumulation of serous fluid at the posterior pole.
On clinical examination, a round to oval, well-delineated shallow serous retinal detachment is
seen in the macula, which may vary in size. It may be associated with underlying pigment epithelial
detachment (PED). PED may be more than one, often less than disc diameter and often difficult
to visualize as it is obscured by turbid subretinal exudates. There may be surrounding RPE changes
corresponding to previous CSCR episodes (Fig. 8.1).
Fig. 8.1: Fundus Photograph of the left eye shows a serous

elevation of the macula involving the fovea.
FFA in acute CSCR has displayed two types of leak patterns. They are:
Inkblot sign in which the fluorescein starts as a single point and expands as a dot, in the late
phase.
Smoke stack sign in which the fluorescein starts as a single point and then ascends, to spread
laterally when it reaches the superior end of the detachment, giving the appearance of a smokestack
(Fig. 8.2).
60
Central Serous Chorioretinopathy (CSCR)
Fig. 8.2: Late Phase of the FFA showing the typical smoke
stack appearance typical of Central Serous Chorioretinopathy
ICG Angiography reveals stippled areas of hyperfluorescence surrounding the active leakage
site whereas FFA only reveals the active leakage site. ICG thus reveals the choroidal hyperpermeability,
seen even in the fellow eye. In chronic CSCR, ICG shows an increased area of choroidal hyper
permeability.
PEDs associated with CSR follows a unique pattern on ICGA. Early phase shows diffuse
hyperfluorescence with mid phase showing central hypofluorescence surrounding by a ring of
hyperfluorescence at the margin of the detachment.
OCT shows an elevated neurosensory retina with an optically clear space underneath and a
reflective layer corresponding to the RPE and choriocapillaries underneath it, corresponding to the
CSR.
With the advent of OCT, the existence of PED with CSR has been shown to be much more than
thought previously. In cases of associated PED, below the elevated neurosensory retina lies optically
Fig. 8.3: OCT shows an elevated neurosensory retina with an

optically clear space underneath and a reflective layer corres-
ponding to the RPE and choriocapillaries underneath it. Associated 61
serous PED is seen along the RPE-Choriocapillaris layer.
clear space below which highly reflective dome shaped elevation with attenuated shadows below
it suggest the presence of PED (Fig. 8.3).
There may be hyperreflective spots in the optically clear space below the neurosensory retina
corresponding with the subretinal exudation and fibrin seen in resolving or chronic CSCR.
CSCR may also be associated with Choroidal Neovascular Membrane (CNVM). In such situations,
there will be a delineated hyperfluorescence on angiogram corresponding with the CNVM. OCT will
show high reflectivity at the level of the RPE choriocapillaries suggestive of a CNVM.
SUGGESTED READING
1. Bennett G. Central Serous Chorioretinopathy. Br J Ophthalmol 1955;39:605-18.
2. Gass JDM Idiopathic Central Serous Chorioretinopathy, Stereoscopic atlas of macular diseases: Diagnosis and
treatment. 4th edition, St.Louis, CV Mosby, 1997
3. David R Guyer. Central Serous Chorioretinopathy. Chapter 22. In Indocyanine Green Angiography. Mosby St. Louis,
1997.
4. Hee MR, Puliafito CA, Wong C et al. Optical Coherence Tomography of Central Serous Chorioretinopathy. Am
J Ophthalmol 1995;120:65-74.
62
Epiretinal Membrane (ERM)
C HAPTER
9
Epiretinal Membrane
Introduction
(ERM)
Epiretinal membranes (ERM) are thin, transluscent fibrocellular membranes that form on the inner
retinal surface (Fig. 9.1). They may be:
Idiopathic, where there may be an associated abnormality of the vitreoretinal interface.
Secondary to a variety of conditions that include retinal vascular occlusions, uveitis, trauma and
ocular surgery.
Fig. 9.1: Fundus photograph of the left eye showing dragging

on the macular vessels by an epiretinal membrane
Depending upon their degree of contracture and severity of retinal distortion caused, ERMs are
graded as:
Grade 0: Cellophane maculopathy.
Grade 1: Wrinkled cellophane maculopathy.
Grade 2: Macular Pucker.
These membranes may be associated with CME, pseudo or true macular hole.
FFA reveals distortion of macular capillaries with straightening of adjacent vessels, with minimal
fluorescein leakage from the vessels seen in late stages. There might be optic nerve staining and cystoid 63
macular edema in advanced ERM (Fig. 9.2).
Fig. 9.2: Mid AV Phase of the FFA showing the dragging of

the macular vessels and diffuse macular leakage
OCT is a very useful tool in grading the ERM, and providing information about the underlying
retinal traction and secondary changes in the retina. OCT reveals a hyper-reflective membrane at
the vitreoretinal interface, adherent to the underlying retina, with distortion of the inner retinal layer.
The traction on the underlying retina is seen as folds in the inner retinal layer.
On OCT, ERMs are seen as:
Clearly separable where clear space is visible between the epiretinal membrane and inner retinal
surface (Fig. 9.3) and
Global ERM where no area of separation is seen between the ERM and inner retinal surface
(Fig 9.4).
Fig. 9.3: OCT reveals a hyper-reflective membrane

at the vitreoretinal interface, adherent to the under-
lying retina, with distortion of the inner retinal layer
.Associated neurosensory layer thickening along
with vitreoretinal traction is seen
Fig. 9.4: OCT reveals a hyper-reflective membrane

at the vitreoretinal interface, adherent to the under-
lying retina, with distortion of the inner retinal layer.
64 Associated neurosensory layer thickening along
with vitreoretinal traction is seen
Epiretinal Membrane (ERM)
An increased thickness of retina with optically empty spaces in retinal layers alongwith reduced
backscattering from outer retinal layers will indicate cystoid macular edema; a lamellar macular hole
may be associated with the ERM.
A macular pseudohole formed by the ERM needs to be differentiated from a true macular hole.
A macular pseudohole will show an abnormally steep foveal contour resembling a partial thickness
macular hole, but the outer retinal layer is seen intact throughout the image, ruling out a full thickness
macular hole.
It is important to distinguish an ERM from a detached posterior hyaloid as both appear as a
reflective band anterior to the retina on OCT. The posterior hyaloid has a weak, patchy reflection
that is thinner than an ERM.
SUGGESTED READING
1. ERM and vitreomacular traction syndrome, vitreoretinal interface abnormalities. Section IV. Acquired Diseases affecting
the macula. Part 2. Disorders of the Retina & Vitreous. AAO Retina and Vitreous 2000-2001.
2. Wilkins JR, Puliafito CA, Hee MR et al. Characterization of epiretinal membranes using Optical Coherence
Tomography. Ophthalmology 1996;103:2142-51.s
65
CHAPTER
10 Introduction
Macular Hole
Idiopathic Macular Hole formation is thought to be associated with tangential vitreomacular traction.
Depending upon pathogenesis, macular holes have been staged as:
1. Stage I: A foveal detachment occurs due to focal contraction of the vitreous. It appears as a
yellow spot (1A) or a yellow ring with loss of the foveal depression (Stage 1B)
2. Stage II: A central full thickness retinal defect <400 m in diameter, obscured by overlying pseudo-
operculum.
3. Stage III: Full thickness, >400 m in diameter retinal defect with attached pseudo-opercula.
4. Stage IV: Stage III hole with complete detachment of the posterior cortical vitreous (Fig. 10.1).
FFA in eyes with Stage II, III & IV holes reveals early foveal hyperfluorescence, which increases
in mid phase, and decrease in late phase (Fig. 10.2).
In cases with vitreomacular traction syndrome, angiography reveals the distorted retinal
vasculature along with leakage of dye from the retinal vessels in the macular region as well as from
the optic nerve. The disc shows leakage on angiography.
66 Fig. 10.1: Fundus photo of the right eye shows full thickness
macular hole at the fovea
Macular Hole
Fig. 10.2: Mid AV Phase of the FFA shows hyperfluorescence

due to transmission defects at the base of the macular hole.The
hyperfluorescence fades in the late phase
Since the advent of OCT, Gasss theories of pathogenesis of macular hole have been well
documented. It has also provided evidence that a localized perifoveal posterior hyaloid detachment
is always present in the early stage of macular hole formation.
In OCT-:
1. Stage IA of the macular hole is seen as a foveal pseudocyst in the inner retinal layers
2. Stage 1B is seen as a partial disruption of the outer retinal layers. The posterior vitreous is seen
attached to its edges. It may develop to a lamellar macular hole (Fig. 10.3).
3. Stage II Reveals as full thickness macular dehiscence with attached posterior vitreous. The
surrounding retina may show thickening and cystic changes.
4. Stage III Reveals a larger macular hole with attached posterior vitreous (Fig. 10.4).
5. Stage IV Reveals a macular hole larger than 400 in diameter, with a detached posterior hyaloid.
There may be an associated optically empty space below the neurosensory retina at the edges
of the dehiscence suggestive of subretinal fluid, alongwith thickening and cystic changes in the
retinal layer (Figs 10.5 and 10.6).
Fig. 10.3: OCT scan through the macula shows a

lamellar inner retinal hole. The RPE Choriocapillaris
layer is of uniform reflectivity 67
Fig. 10.4: Full thickness macular hole with cystic changes at the
margins, associated vitreous traction at the margin of the macular
hole
Fig. 10.5: OCT through the macular hole reveals a full thickness
macular hole with cystic changes at the margins of the hole.
Complete detachment of the posterior vitreous is seen.
Fig. 10.6: Traumatic Full thickness macular hole with cystic

changes at the margins. Associated choroidal rupture seen as a
hyperreflectivity at the level of the RPE Choriocapillaris layer
SUGGESTED READING
1. Idiopathic macular hole. Section IV. Acquired Diseases affecting the macula.Part 2. disorders of the Retina & Vitreous.
AAO Retina and Vitreous 2000-2001.
2. Gass JD. Reappraisal of biomicroscopic classification of stages of development of a macular hole. Am J Ophthalmol.
1995; 119:752-759.
3. Gass JDML. Stereoscopic atlas of macular diseases: Diagnosis and treatment, 4th edn, CV Mosby. St. Louis, 1997;922.
4. AAO. ICG. Ophthalmology 1998; 105:1564-69.
5. Johnson MW. Improvements in understanding and treatment of macular holes. Current Opinion of Ophthalmology
2002;13:152-60.
68
Heredomacular Degeneration (HMD)
C HAPTER
11
Heredomacular Degeneration
Introduction
(HMD)
BESTS DISEASE: Also termed as Vitelliform Macular Dystrophy, it presents in childhood

with the appearance of a yellow or orange yolk like lesion in the macula. The lesion evolves through
several stages over years (Fig. 11.1).
Fig. 11.1: Fundus photograph of the right eye showing the

vitelliform lesion characteristic of Bests disease
Though the hallmark of the disease is a markedly abnormal EOG, an FFA will reveal blocked
choroidal fluorescence corresponding to the vitelliform lesion. Rest of the angiogram remains normal.
In the late atrophic stage, a transmission window defect is noted. In case of development of a CNVM,
a corresponding area of hyperfluorescence is seen, with late leakage.
ICG Angiography reveals hypofluorescence in the area of the vitelliform lesion and mottled hyper
and hypofluorescence in the vitelloeruptive stage of the lesion.
OCT shows a characteristic dome shaped lifting of retina including the RPE layer, with a high
reflective deposit in the sub RPE space, corresponding to the accumulated lipofuschin. This is in
contrast to the presentation of a serous PED (Fig. 11.2). 69
Fig. 11.2: OCT through the lesion shows a characteristic dome shaped
lifting of retina including the RPE layer, with a high reflective deposit in
the sub-RPE space, corresponding to the accumulated lipofuschin
Fig. 11.3: Fundus photograph of the right eye showing Fig. 11.4: Late AV Phase of the FFA reveals minimal
the bicycle wheel pattern of foveal schisis hyperfluorescence at the macula.Foveal schisis on
FFA does not conform to any pattern
Fig. 11.5: OCT image of the fovea showing elevation

of the foveal pit with mild perifoveal splitting of the
neurosensory retina
Foveal Schisis
Congenital retinoschisis is characterized by bilateral foveal schisis. 50% of patients have a peripheral
retinoschisis. The clinical appearance resembles a spoke like bicycle wheel pattern on the fovea
(Fig. 11.3).
FFA does not reveal any abnormal fluorescence in the foveal area except for a transmission defect
in the late phase in occasional cases (Fig. 11.4).
OCT is diagnostic as it reveals an elevation of the foveal pit with perifoveal splitting of the
70
neurosensory retinal layers (Fig. 11.5).
Fig. 11.6: Fundus photograph of the right eye showing RPE

alterations around the fovea forming a Bulls eye pattern
A B
Figs 11.7A and B: Red free and infrared photograph of the right eye showing RPE alterations around the
fovea and flecks at the macula suggestive of Stargardts disease
Stargardts Disease
Characterized by an atrophic macular degeneration with surrounding yellowish ill-defined flecks,
Stargardts disease is an autosomal recessive inherited condition. When associated with yellowish
flecks, it is also called Fundus Flavimaculatus. The atrophic maculopathy may eventually show
a bulls eye pattern (Figs 11.6 and 11.7).
FFA shows a pigment epithelium window defect centrally, with surrounding horizontally ovoid
zone of faint hyperfluorescent flecks. Also seen is the characteristic choroidal silence in which
the background choroidal fluorescence is masked. As the disease progresses, the hyperfluorescent 71
flecks ultimately may also confluence because of increasing RPE atrophy (Fig. 11.8).
A B
Figs 11.8A and B: :Mid AV phase of the FFA shows transmitted hyper fluorescence in the region of the RPE
alterations and flecks.No choroidal fluorescence is seen in the area beyond the arcades. The intensity of the
hyperfluorescent lesion decreases in the late phase of the FFA, suggestive of Stargardts disease
ICG Angiography reveals hypofluorescence of the flecks with a background diffuse hyper
fluorescence in late phase. Associated atrophic changes of the macula are seen as a hypofluorescence
(Fig. 11.9).
OCT reveals a thinned out neurosensory retina along with increased reflectivity of the RPE-
Choriocapillaris layer (Fig. 11.10).
Fig. 11.9: ICG reveals hypofluorescence of the flecks.

72 Macula appears normal except in cases with atrophic
maculopathy
Fig. 11.10: OCT through the lesion shows thinned

out neurosensory retina and an increased reflec-
tivity of the RPE Choriocapillaris layer
Retinitis Pigmentosa
Retinitis pigmentosa (RP) is characterized by arteriolar attenuation, bony spicule pigmentation in
the mid peripheral fundus and disc pallor. There are associated mottled retinal pigment epithelial
changes throughout the fundus (Fig. 11.11).
FFA features depend on the severity of the retinal changes. In early phase, a mottled hyper-
fluorescence is seen in the midperipheral retina. With disease progression, patches of choriocapillary
nonfilling are noted. In late stage, Choroidal flush may be absent. A minimal leakage in the macular
area may be noted. Cystoid spaces may also be seen.
OCT reveals a thinned out neurosensory retina. Associated CME may be seen (Fig. 11.12).
Fig. 11.11: Fundus photograph of the left eye showing

generalized RPE alterations.Fovea shows a dull reflex
Fig. 11.12: OCT reveals a thinned neurosensory

retina with retinal pigment alterations. Also seen is 73
associated Cystoid changes at the fovea.
SUGGESTED READING
1. Gass JD. Stereoscopic Atlas of Macular Disease: Diagnosis and treatment. 4th ed. St. Louis: Mosby; 1997.
2. Tasman WS. Hyaloidoretinopathies. In: Regillo CD Brown GC, Flynn HW Jr. Eds. Vitreoretinal disease. The essentials.
New York: Thieme; 1999:365-76.
3. Miller SA. Fluorescence in Bests vitelliform dystrophy, lipofuschin and fundus flavimaculatus. Br J Ophthalmol 1978;
62:256-60.
4. Anmarkrud N. FFA in Fundus flavimaculatus and Stargardts ds. Acta Opthalmol 1979;57:172-82.
74
Inflammatory Disease of the Retina and Choroid
C HAPTER
12
Inflammatory Diseases of the
Introduction
Retina and Choroid
Inflammatory disease of the retina and choroid usually show similar FFA and ICGA features. OCT
helps in localizing the depth of the lesion and detecting secondary changes like CME, subretinal fluid
and epiretinal membranes.
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

APMPPE is characterized by multiple discrete yellow-white placoid lesions in the posterior pole at
the RPE-choroid level. With time, the lesions fade, showing RPE changes. Later new lesions may
crop up (Fig. 12.1).
FFA shows early hypofluroscence of the active lesions, followed by late hyperfluorescence. Healed
lesions show irregular hyperfluorescence corresponding to transmission defects in early phase, which
fade later.
ICG Angiography reveals an early and late phase hypofluorescence, seen both in the acute as
well as healed lesions (Fig. 12.2).
Fig. 12.1: Fundus photograph of the left eye showing the yellowish 75
placoid lesions at the macula characteristic of APMPPE
Fig. 12.2: Late phase ICG angiography reveals hypofluorescent

spots corresponding with the clinically seen lesions.
Fig. 12.3: OCT reveals an area of moderate hyper-reflectivity in

the outer retinal layers, corresponding to the active plaque
OCT reveals an area of moderate hyper-reflectivity in the outer retinal layers, corresponding to
the active plague. Healed lesions reveal evidence of RPE hyperplasia (Fig. 12.3).
SUGGESTED READING
1. Chorioretinal inflammatory diseases. Chapter 9, Optical Coherence Tomography of Ocular diseases. Puliafito CA,
Hee MR, Schuman JS, Fujimoto JG. Slack Inc. 1996; 249-68.
2. Gass JDM. Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 1968; 80; 177-85.
76
Index
Index
A D
Acute posterior multifocal placoid pigment epitheliopathy Diabetic maculopathy 46-51
(APMPPE) 75 fundus fluorescein angiography (FFA) 46-49
fundus fluorescein angiography (FFA) 75 indocyanine green angiography (ICGA) 50
indocyanine green angiography (ICGA) 75 optical coherence tomography (OCT) 50, 51
optical coherence tomography (OCT) 76
Age-related macular degeneration (ARMD) and its E
features on FFA, ICGA, and OCT 11
choroidal neovascularization 15-20 Epiretinal membrane 63-65
disciform scar 28
drusen associated 11 F
fibrovascular PED 23, 24
Foveal schisis 70
geographic atrophy 11-13
Fundus fluorescein angiography 1-3
pigment epithelium detachment 22-25
RPE RIP 26-28
Angiography H
fluorescein 1-3 Heredomacular degeneration 69
indocyanine green 4-7 Hyperfluorescence 3
Angioid streaks 32 Hypofluorescence 3
Arterial occlusive diseases 57, 58
branch retinal artery 57, 58
central retinal artery 58, 59 I
Autofluorescence 2
Indocyanine green angiography (ICGA) 4-7
B M
Bests disease 69
Macular edema 46-51
Blocked fluorescence 3
clinically significant 46
CME 47
C diffuse 46
Capillary nonperfusion 3 focal 46
Central serous chorioretinopathy 60 Macular hole 66-68
Cherry red spot in CRAO 58
Choroidal neovascular membrane O
idiopathic 31
Optical coherence tomography (OCT) 7-10
inflammatory 32
myopic 31
with angioid streaks 31, 32 P
with ARMD 31 Parafoveal telangiectasia (PFT) 40-45
Cystoid macular edema 47 77
Polypoidal choroidal vasculopathy 36-39
Diagnostics of Macular Disorders
R V
Retinitis pigmentosa (RP) 73 Venous occlusive disorders 53-59
branch retinal vein 53
S central retinal vein 55
Smoke stack appearance 61 hemiretinal vein 56
Stargardts disease 71 Vitreomacular traction syndrome 64
78

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Diagnostics in

Aditya Jyot Eye Hospital Pvt. Ltd.

Diagnostics in Macular Disorders

First Edition: 2005

2. Age-Related Macular Degeneration (ARMD) ................................................... 11

3. Choroidal Neovascular Membranes (CNVM) other than

4. Idiopathic Polypoidal Choroidal Vasculopathy (IPCV) .................................. 36

5. Parafoveal Telangiectasia (PFT) ....................................................................... 40

6. Diabetic Retinopathy ........................................................................................ 46

7. Retinal Vascular Occlusions ............................................................................ 53

8. Central Serous Chorioretinopathy (CSCR) ..................................................... 60

9. Epiretinal Membrane (ERM) ............................................................................. 63

10. Macular Hole .................................................................................................... 66

11. Heredomacular Degenerations (HMD) ............................................................. 69

12. Inflammatory Diseases of the Retina and Choroid ....................................... 75

Lawrence J Singerman MD, FACS

FUNDUS FLUORESCEIN ANGIOGRAPHY (FFA)

Normal Fluorescein Angiogram

Early Choroidal and Cilioretinal Artery Filling

Retinal Arterial Filling and Increase Choroidal Filling

Retinal A-V Filling and Full Choroidal Filling

Full Retinal AV Filling and Choroidal Filling (Fig 1.1)

AV Recirculation Phase and Decrease of Retinal and Choroidal Fluorescence

Reduced Retinal and Choroidal Fluorescence

INDOCYANINE GREEN ANGIOGRAPHY (ICGA)

Early phase angiography: Timer is started when ICG injection is begun.

Fig. 1.2: Mid phase of ICG angiography showing normal filling

Fig. 1.3: Late phase of ICG angiography showing uniform

The Normal Angiogram

Interpretation of the ICG Angiogram

Early phase (within 2-60 sec)

Mid phase (1-15 min)

Prominent choroidal veins

Late phase (15-30 min)

Choroidal vessels in early phase, hot spot, plaque, polyps.

OPTICAL COHERENCE TOMOGRAPHY (OCT)

Features of the Normal Macula

Fig. 1.4: The normal retinal tomogram denoting the uniform

ii. Scar tissue and neovascular membranes: varying hyperreflectivity.

Fig. 2.1: OCT line scan through the drusen shows

Fig. 2.2: Fundus photograph of the right eye

Fig. 2.3: Red free photograph of the right eye

Fig. 2.4: Fundus fluorescein angiography of the right eye

Fig. 2.5: Indocyanine green angiography reveals areas of

Fig. 2.6: Fundus photograph of right eye showing

Fig. 2.7: Late phase of FFA of right eye showing area of

Neovascular (exudative) Age-Related Macular Degeneration

Choroidal Neovascular Membrane

Pigment Epithelial Detachment

Classic Choroidal Neovascular Membrane

Fig. 2.9: Fundus photograph of the left eye showing subfoveal

Figs 2.10A and B: Mid AV phase of FFA eveals a well defined

Fig. 2.11: In the late AV phase the lacy hyperfluorescence

Fig. 2.12: ICG angiography does not add any further

Fig. 2.13: OCT reveals fusiform hyperreflective echo

Fig. 2.14: Fundus photograph of the right eye showing a PED at

Fig. 2.17: Mid AV phase of the FFA shows slow filling of

Fig. 2.18: Fundus photograph of the left eye shows an area of

Fig. 2.19: Mid phase of the FFA showing irregular stippled

Fig. 2.20: ICG angiography picture of right eye shows hypo-

Fig. 2.21: ICG angiography shows a plaque in the late

Angiographic Features that can Obscure CNVMS

Fig. 2.23: OCT shows a thickened area of moderate hyper-