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A Colour Atlas

of Fundoscopy

First Edition
A Colour Atlas
of

Fundoscopy

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Edited by
Dr. Suman Chowdhury

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Contents

Topics Page

Hypertensive and Atherosclerotic 01


Diabetic 08
Vasculitic 19
Venous Occlusion 23
Arterial Occlusion 33
Radiation Retinopathy 44
Tumour 48
Acquired Macular Disease 57
Angioid Streaks 60
Acquired Macroaneurysm 62
Infections 67
Immunologic 93
Haematologic 99
Endocrine 106
GIT 109
Mesenchyme and Skeletal 111
Metabolic 117

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Hypertensive
and
Atherosclerotic

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2

Diagnosis: Hypertensive retinopathy Grade 4

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Diagnosis: Arteriosclerosis, of Retinal Vessels

Comment to photo: Sclerosis of arterial vessels is shown with thickening of the wall and disappearing visibility of
the blood column. Veins appear dilated, and there is edema of the macular area

Diagnosis: Arteriosclerosis, of Retinal Vessels

Comment to photo: Ensheathed arterial vessels are seen near the disc. Other signs of arterial hypertension are
small intraretinal hemorrhages, some exudates, slight edema of the inferior disc margin, and congested veins.

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Diagnosis: Arteriosclerosis, of Retinal Vessels

Comment to photo: Opaque and thickened wall of arterial vessel

Diagnosis: Hypertensive Retinopathy, Cotton-Wool Spots

Comment to photo: The caliber of the veins is increased in relation to the arteries. Several cotton-wool spots are
seen and a little bit of exudate.

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Diagnosis: Hypertensive Retinopathy

Comment to photo: Thinned straight arteries, increased caliber of the veins, small intraretinal hemorrhages,
exudates

Diagnosis: Hypertensive Retinopathy

Comment to photo: Additional features of hypertension are a blurred disc and exudates in the macula (star shaped)
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Diagnosis: Hypertensive Retinopathy, Intra Retinal Hemorrhage (#1)

Comment to photo: Sharply defined hemorrhage underneath the internal limiting membrane. (Note the reflexes).
Little globs of blood are seen where the blood is located when the patient is in prone position. The prominent white
circumferential line may represent the edge of vitreous detachment. Proteinaceous material is deposited in the
retina outside this ring. This photo was taken when the patient was lying on his right side to show the mobility of
the blood; the disc is above.

Diagnosis: Hypertensive Retinopathy, Intra Retinal Hemorrhage (#3)

Comment to photo: Same patient. The blood has completely absorbed. The folds in the internal limiting lamina and
the protein outside the ring have disappeared,
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Diagnosis: Hypertensive Retinopathy, Intraretinal Hemorrhage

Comment to photo: Centrally there is a well-defined accumulation of blood under detached internal limiting
lamina. A second ring of blood has less well-defined borders and represents preretinal blood where the vitreous is
detached from the retina.

Diagnosis: Papilledema showing blurred disc margins and dilated tortuous vessels

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Diabetic

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Diagnosis: Diabetes mellitus, Lipemia Retinalis in Hypertriglyceridemia

Comment to photo: 16 year old patient with diabetis mellitus with hypertriglyceridemia. The vessels look creamy
and the fundus has a much lighter coloration.

Diagnosis: Diabetic Retinopathy, Nonproliferative

Comment to photo: Many red dots, microaneurysms, are seen together with hard exudate in a circinate pattern.

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Diagnosis: Diabetic Retinopathy, Nonproliferative, Angiogram

Comment to photo: The angiogram reveals even more bright spots than the clinical picture suggests with subtle
staining of the retina surrounding these areas. In addition, there is a lack of capillaries where there are no
aneurysms.

Diagnosis : Diabetic retinopathy, non-proliferative

Comment to photo: The hard exudates temporal to the fovea indicate chronic leakage from a microaneurysm. This
becomes clearly visible in the angiogram. In addition one recognizes a subtle stain of the retina (edema) in this
area.

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Diagnosis: Diabetic Retinopathy, Nonproliferative

Comment to photo: Many superficial, intraretinal hemorrhages. The fluffy, white spots, cotton wool spots, are
microinfarcts.

Diagnosis: Diabetic Retinopathy, nonproliferative

Comment to photo: The upper picture shows the fundus of a diabetic with very early changes. There is a small
cotton-wool spot and a small intraretinal hemorrhage. Some time later a lot of microaneurysms and accumulations
microaneurysms are found above the fovea along the supero-temporal artery in the eye of the same patient (lower
picture). Hard exudates become visible.

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Diagnosis: Diabetic Retinopathy, Nonproliferative, Severe

Comment to photo: The fundus shows multiple blot and dot hemorrhages. Many hard exudates are a sign of
chronic leakage. The difficulty to visualize a clear choroidal pattern also indicates diffuse edema of the retina. The
disc is pale indicating optic atrophy.

Diagnosis: Retinopathia diabetica, Non-proliferative

Comment to photo: This is the fundus of a 56 year old man with slight decrease in vision and type 2 diabetes for 7
years. Only with close observation microaneurysms are detected near the fovea.

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Diagnosis: Diabetic Retinopathy, Proliferative (PDR)

Comment to photo: One has to look carefully to see the heavy neovascularization that extends along the posterior
surface of vitreous which is adherent to the disc but detached elsewhere.

Diagnosis :Proliferative Diabetic Retinopathy (PDR) #1

Comment to photo: On the temporal side of the optic nerve head are new vessels. The posterior pole shows
microaneurysms, small dot hemorrhages, and hard intraretinal exudate. The slight discoloration of the retina in the
area of the superior arcade indicates retinal edema.

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Diagnosis: Proliferative Diabetic Retinopathy (PDR), Severe #1

Comment to photo: Fibrovascular proliferations at the disc and along the superior and inferior arcade have
contracted and because of their intimate connections with the retina, detached the retina. Over the macula the
posterior vitreous is detached since there are no vascular connectons. The elevated retina together with the flat
posterior vitreous detachment looks like a table mountain, the posterior vitreous membrane over the macula is the
"tabletop" (see also ultrasound picture)

Diagnosis: Diabetic Retinopathy, Proliferative (PDR)

Comment to photo: Preretinal hemorrhages occur after traction on new vessels. They can be suspected to be
located at the proliferation of the 6 o'clock vein and at 7 o'clock. The hemorrhagic streaks toward the macula
indicate a subhyaloid connection to this area.
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Diagnosis: Diabetic Retinopathy, Proliferative (PDR)

Comment to photo: There are multiple signs of diabetic retinopathy: venous beading, hemorrhages, cotton-wool
spots, intraretinal vascular abnormalities (IRMA), but most important for the prognosis is the neovascularization of
the disc (NVD).

Diagnosis: Diabetic Retinopathy, Proliferative (PDR)

Comment to photo: Massive neovascularization along the retinal surface (this indicates that the vitreous is not
detache
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Diagnosis: Diabetic Retinopathy, Proliferative, (PDR)

Comment to photo: Severe macular edema with hard exudate. Neovascularization above the disc.

Diagnosis: Retinopathia diabetica, RA, Peripherie, Fluoreszenzangiographie

Comment to photo: ausgeprgte kapillarfreie Zonen, am Rand davon ausgeprgte Gefneubildung von retinalen
Netzhautgefen ausgehend (Neovaskularisation)

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Diagnosis: Retinopathia diabetica, RA, zentraler Fundus, Farbbild

Comment to photo: retinale Blutungen, ausgeprgte Gefneubildung von papillennahen Netzhautgefen


ausgehend (Neovaskularisation oder Wundernetz)

Diagnosis: Diabetic Retinopathy (#1), Proliferative after Vitrectomy

Comment to photo: Rarely, despite successful vitreous surgery, small convoluted new vessels do develop. They
grow very little and may bleed.
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Diagnosis: Diabetic Retinopathy , Proliferative after Vitrectomy, Angiogram

Comment to photo: In the arterio-venous phase several grape-like lesions light up, only one of which is visible on
the fundus photograph. This may indicate that the new vessels initially are intraretinal and only later break through
the internal lamina.

Diagnosis: Proliferative diabetic retinopathy

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Vasculitic

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Diagnosis: Polyarteritis (Periarteritis) Nodosa

Comment to photo: As an expression of the widespread arteritis many arterial occlusions (cotton-wool spots) and
hemorrhages are seen. The vessels are dilated and tortuous and leak as evidenced by the exudates in the macular
area.

Diagnosis: Lupus Erythematodes (LE)

Comment to photo: Multiple cotton-wool spots as a sign of the microvascular occlusive disease.
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21

Diagnosis: Multiple Sclerosis

Comment to photo: Subtly ensheathed veins.

Diagnosis: Multiple Sclerosis (MS), Rucker's Sign

Comment to photo: Ensheathed veins can be found in the fundusperiphery in cases of multiple Sclerosis. (30yo
female patient with confirmed MS)

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Diagnosis: Multiple Sclerosis, Ensheathed Veins (M. Rucker)

Comment to photo: Peripheral veins are surrounded by a whitish inflammatory infiltrate and are therefore
obscured. This finding occurs in about 25% of the cases of MS.

Diagnosis: Perivasculitis

Comment to photo: Predominently veins are ensheathed and show perivascular infiltrates. The differential
diagnoses includes infectious diseases (i.e. syphilis), collagenoses, sarcoid and idiopathic retinal vasculitis.

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Diagnosis: Purtscher's-like Retinopathy

Comment to photo: In collagen vascular disease (here polyarteritis) a coagulopathy may occur with occlusion of
small vessels. One sees many cotton-wool spots similar to Purtscher's retinopathy.

Diagnosis :Eales' Disease

Comment to photo: Area of occluded arterioles and veins with some proliferation and atrophic retinal area.
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Venous Occlusion

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Diagnosis: Branch Retinal Vein Occlusion (BRVO), Superior and Inferior Temporal

Comment to photo: Both, superior temporal and inferior temporal veins are dilated and tortuous. Numerous flame
shaped hemorrhages and cotton wool spots in the temporal retina with macular edema. Simultaneous superior and
inferior temporal branch retinal vein occlusion is not commonly encountered. 55 year old hypertensive lady with
end stage renal failure, presented with 3 days history of acute blurring of vision.

Diagnosis: Branch Vein Occlusion (BVO)

Comment to photo: The temporal inferior vein is congested. Multiple small intraretinal hemorrhages indicate the
vein occlusion
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Diagnosis: Branch Vein and Artery Occlusion, Old

Comment to photo: There are corkscew vessels near the temporal raphe. They indicate connections between the
inferior and superior vein.

Diagnosis: Branch Vein and Artery Occlusion, Old, Angiogram

Comment to photo: The vascular connections are much better seen in the angiogram. The corkscew vessels leak as
well as the artery. The artery may have been occluded at the kink. Loss of capillary network is seen in the area of
previous combined occclusion.
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Diagnosis: Branch Vein Occlusion (BVO) (#1)

Comment to photo: Intense retinal hemorrhage in area of vein occlusion.

Diagnosis: Branch Vein Occlusion (BVO) (#2)

Comment to photo: 1 1/2 years later there are small retinal hemorrhages, tortuous shunt vessels and some hard
exudates.

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Diagnosis: Branch Vein Occlusion (BVO) (#3)

Comment to photo: After 3 1/2 years there are even more heavy exudates in the area of previous vein occlusion.

Diagnosis: Branch-Vein Occlusion, with Neovascularization

Comment to photo: Some time after occlusion of the temporal superior vein the vessel is still dilated and tortuous
in some areas. Small retinal hemorrhages and some hard exudates near the fovea are seen in the retina. The view
to the retina is obscured by membranes that are in front of the retina. A barely visible traction band pulls on the
vein in the direction of the disc where one sees neovascular tissue. The retinal neovascularization is easily
overlooked but becomes very obvious in the angiogram.
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Diagnosis: Branch Vein Occlusion (BVO) (#4)

Comment to photo: Nine months later the hemorrhage is absorbed. There are several corkscrew vessels as a sign of
slowed-down blood flow.

Diagnosis: Branch Vein Occlusion (BVO), Retinal Hemorrhage (#1)

Comment to photo: Occlusion of the infero-temporal vein. The hemorrhage is located in the inner retinal layers and
follows the nerve fibers along their course.

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Diagnosis: Macular Edema (Cystoid Macular Edema) Secondary to Branch Retinal Vein Occlusion (BRVO)

Comment to photo: In the area of the inferior temporal vein the retina shows multiple intra-retinal hemorrhages up
to and including the foveal area and cotton-wool spots. The angiogram demonstrates leakage of the parafoveal
vessels. Because of the hemorrhages the effect of the leaking vesels on the fovea are not well visible.

Diagnosis: Venostasis Retinopathy

Comment to photo: Chronic elevation of pressure on the venous side leads to tortuous, enlarged veins, but only
discrete retinal hemorrhages.
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Diagnosis: Central Vein Occlusion

Comment to photo: She came back 4 months later after severe loss of her vision with the findings of a full blown
central retinal vein occlusion with elevated disc and blurred margins, semicircular retinal folds and massive
intraretinal hemorrhages

Diagnosis: Impending Vein Occlusion

Comment to photo: This 27 year old female patient on examination of the right eye with normal VA showed
considerable tortuosity of the vessels with enlargement of the veins and slight hyperemia and blurring of the disc
margin: signs of an impending vein occlusion.

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Diagnosis: Central Retinal Vein Occlusion, Non-ischemic

Comment to photo: The vessels are tortuous and dilated. Intraretinal hemorrhages are all over the fundus.

Diagnosis: Central Retinal Vein Occlusion, Non-ischemic, Angiogram

Comment to photo: Tortuous vessels are well visible and are perfused, though slowly.

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Diagnosis: Central Retinal Vein Occlusion (CRVO), Ischemic, Angiogram

Comment to photo: Although the fundus picture may be very similar to the one with a non-ischemic vein occlusion,
the angiogram is very different. The veins are not perfused. Only the arteries fill slowly. Such a case has a bad
prognosis for visual recovery.

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Arterial Occlusion

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Diagnosis: Branch Artery Occlusion, acute

Comment to photo: Multiple cotton-wool spots indicate many micro-infarcts in the area of the temporal superior
artery.

Diagnosis: Branch Artery Occlusion, acute

Comment to photo: White material (calcified?) occludes the superior artery and causes edema of the retina in its
area of distribution.

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Diagnosis: Branch Artery Occlusion, Calcified Plaques

Comment to photo: White, calcified plaques are seen in the arteries of the optic nerve head. They are thought to
derive from arteriosclerotic plaques in the carotis. They are not always leading to occlusion of the affected artery

Diagnosis: Branch Artery Occlusion, Calcium Plaque, Chronic

Comment to photo: White particles in the course of an artery are most likely from calcium plaques of the carotid.
The passage for blood may remain intact.

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Diagnosis: Branch Artery Occlusion, Hollenhorst Plaque

Comment to photo: A yellowish plaque in the course of an artery (Hollenhorst Plaque) is thought to be an embolus
from vessels with arterosclerotic changes (Carotid). It is composed of cholesterol and lipid. It may or may not
occlude the vessel.

Diagnosis: Abnormal Retinal Artery, Occluded

Comment to photo: There is an abnormal Artery, which passes to the temporal side close to the fovea. This artery
originates near the emerging central vessels as a very early branch of the inferior arcade. The retina is edematous
in its area of distribution. Careful examination reveals what looks like a yellowish obstruction near the nasal margin
of the disc at 4 o'clock. Multiple yellowish particles are seen in the vessel above the fovea.

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Diagnosis: Branch Artery Occlusion

Comment to photo: Closure of the temporal inferior artery at its bifurcation results in edema of the retina
(whitening). The embolus is visible.

Diagnosis: Old Branch Artery Occlusion

Comment to photo: The temporal superior artery is barely visible near the disc.

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Diagnosis: Old Branch Artery Occlusion

Comment to photo: In the periphery one sees so-called ghost vessels.

Diagnosis: Hollenhorst Plaque, Hypertension, Carotid Artery Disease

Comment to photo: This eye shows signs of hypertension: enlarged veins, vascular tuortuosity, arterio-venous
crossing. At the biforcation of an artery a yellowish plaque (Hollenhorst Plaque) is thought to be an embolus from
vessels with arterosclerotic changes (Carotid). It is composed of cholesterol and lipid. It may or may not occlude the
vessel.
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Diagnosis: Central Retinal Artery Occlusion (CRAO), Few Days Old

Comment to photo: Regressing edema of the retina, disappearing red spot in fovea and narrow arteries. The disc
not yet atropic

Diagnosis: Central Retinal Artery Occlusion, Angiogram

Comment to photo: The late angiogram shows very slow perfusion of the retinal vessels as indicated by the so
called box-car formation in the vessels.
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Diagnosis: Central Retinal Artery Occlusion (#1,1), with Cilioretinal Artery

Comment to photo: Faded whitening (a sign of a little older event) of the retina and cherry-red spot. Only the
papillo-macular bundle looks normal. Vision is 0.01.

Diagnosis: Central Retinal Artery Occlusion (#1,2) with Cilioretinal Artery. Angiogram.

Comment to photo: While all other vessels are not perfused the cilio-retinal artery with its veins is open.

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Diagnosis: Central Retinal Artery Occlusion, Old

Comment to photo: The arteries are very thin, the veins have very slow blood flow as indicated by box-car
formation of the blood column. The disc is pale.

Diagnosis: Central Retinal Artery Occlusion, Old, Angiogram

Comment to photo: In this late angiogram the stained blood lingers in the veins because of very slow blood flow,
and the interrupted blood column (box-car formation) is well visible.
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Diagnosis: Central Retinal Artery Occlusion

Comment to photo: Edema makes the retina look whitish exept for the fovea where it is so thin that one can see
the perfused underlying tissues (cherry red spot)

Diagnosis: Central Retinal Artery Occlusion

Comment to photo: After a while the retinal edema turns into atrophy and the retina becomes transparent again.
In some of the vessels one sees an interrupted blood column, so called box-car formation, as a sign of very slow
perfusion.
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Diagnosis: Central Retinal Artery Occlusion, recent, with Open Cilioretinal Arteriole

Comment to photo: Diffuse ischemic infarction of the inner retinal layers.White swollen retina, a sign of recent
artery occlusion. Box-carring of the blood collumn in some vein cause by stagnant blood flow. A patent cilioretinal
vessel maintains a small area of normal appearing retina between disc and fovea but does not save the fovea.

Diagnosis: Central Retinal Artery Occlusion, Total Optic Atrophy

Comment to photo: After complete occlusion of the central artery ascending total optic atrophy and obliteration of
vessels.
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Radiation retinopathy

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Diagnosis: Radiation Retinopathy

Comment to photo: Small hemorrhages, vascular dilatations, macular edema and exudates have developed after
irradiation.

Diagnosis: Radiation Retinopathy

Comment to photo: Radiation changes are progressive: one year later there are more vascular abnormalities and
the edema and exudates have increased.

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Diagnosis: Radiation Retinopathy

Comment to photo :Multiple intraretinal hemorrhages after radiation of a conjunctival tumor.

Diagnosis: Radiation Retinopathy

Comment to photo: Left eye: very similar findings to right eye.

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Diagnosis: Radiation Retinopathy

Comment to photo: This retinopathy usually occurs 12-18 months after damage to the retina following either
orbital or ocular radiotherapy. It is a microangiopathy characterized by vascular occlusion and altered vascular
permeability. The fundus findings are similar to diabetic retinopathy. Cotton wool spots indicate infarction of the
nerve fiber layer and there are commonly noted in the posterior pole as the nerve fiber layer is thickest there. This
37 years old gentleman had radiotherapy a year ago for nasopharyngeal carcinoma. He presented with blurring of
vision of 2 weeks duration. VA of both eyes were 0.6. Right eye: multiple cotton wool spots in all 4 quadrants of the
posterior pole. Dot-blot hemorrhages, flame shaped hemorrhages and sub-RPE hemorrhage. No hard exudate or
neovascularization.

Diagnosis: Retinale angiomatse Proliferation (LA, Farbbild)

Comment to photo: paramakulres retinales dem, retinale Blutungen, harte Exsudate, Abhebung der
neurosensorischen Netzhaut, Drusen und Pigmentepithelvernderungen
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Tumour

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Diagnosis: Astrocytic (Glial) Hamartoma

Comment to photo: Congenital glial tumor that is sharply delineated, originally dome shaped, later flat, and
chalky-white because of calcification. Most frequently seen in tuberous sclerosis, sometimes in neurofibrosis.

Diagnosis: Astrocytoma of Optical Nerve

Comment to photo: Astrocytomas are usually found in conjunction with tuberous sclerosis but can also occur as
isolated findings as here in a healthy 74 year old man. The tumor seems to be composed of many globules.

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Diagnosis: Astrocytoma of Retina

Comment to photo: Calcified slightly elevated and well demarcated astrocytoma.

Diagnosis: Astrocytoma of Retina, Tuberous Sclerosis (Bourneville's, Pringle's Disease)

Comment to photo: Globular tumor of the retina, semi-translucent in early life. Angiography shows vascularization
with rapid leakage.

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Diagnosis: Reactive Astrocytic Hyperplasia

Comment to photo: Rapidly growing vascularized benign retinal tumor (thought to be caused by traction from
preretinal membranes).

Diagnosis: Combined Hamartoma of Retina and RPE (#1)

Comment to photo: A slightly elevated, greenish-gray mass overlies the optic disc and peripapillary retina.
Centrally, it has a gliotic appearance. The origins of the retinal vessels are obscured. Pigmentary proliferation can
be appreciated at the periphery of the lesion superonasally and inferonasally. Fine superficial radial folds indicate
preretinal proliferation. A single flame hemorrhage is seen at 11 o'clock.

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Diagnosis: Congenital Hypertrophy of the RPE (#1)

Comment to photo: Sharply defined jetblack to brown lesion underneath the retina. It may have areas of atrophy
(windows) through which one sees atrophic choroid. Corresponding to the lesion is a visual field defect.

Diagnosis: Congenital Hypertrophy of the RPE

Comment to photo: Sharply defined area of RPE hyper-pigmentation with corresponding visual field defect.
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Diagnosis: Congenital Hypertrophy of the RPE

Comment to photo: Iinitially hyperpigmented, later depigmented atrophic lesion in midperiphery.

Diagnosis: Congenital Hypertrophy of the RPE, Bear Track

Comment to photo: Multiple black lesions resemble animal tracks.


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Diagnosis: Congenital Hypertrophy of the RPE and Gardner's Syndrome

Comment to photo: Hypertrophy of the retinal pigment epithelium with a depigmented tail ("comet tail")
(arrow)towards posteriorly is seen frequently in combination with the Gardner syndrome (familial adenomatous
polyposis, FAP).

Diagnosis: Congenital Hypertrophy of the RPe and Gardner's Syndrome

Comment to photo: Hyperpigmented patches of the RPE, often segmental are found in connection with familial
adenomatous polyposis (which often develops into cancer) and other hamartomas of the skeleton and soft tissues.

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Diagnosis: Malignant Glioma (#1), (Astrocytoma) of Retina and Optic Nerve

Comment to photo: Increasing swelling of optic disc and adjacent retina with abnormal vessels and fuzzy borders. (
G.O.H. Naumann)

Diagnosis:RetinoblastomaComment to photo:Spontaneously regressed large tumor in 23 year old mother of


previous child

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Diagnosis:RetinoblastomaComment to photo:Small spontaneously regressing tumor beginning to calcify in 4 year


old child

Diagnosis: Retinoblastoma, After Irradiation

Comment to photo: Calcified remnant of tumor after radiation therapy

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Acquired Macular Disease

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Diagnosis: Cystoid Macular Edema

Comment to photo: The macula of this aphakic patient looks normal at first glance. The slight swelling and the
cystoid spaces of the foveal area are often beyond the resolution of a photograph but can be seen
biomicroscopically.

Diagnosis: Cystoid Macular Edema, Angiogram

Comment to photo: The late angiogram shows the accumulation of dye in the cystoid spaces in a petal-like pattern.

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Diagnosis: Cystoid Macular Edema

Comment to photo: One has to look very carfully to detect the two large cystoid spaces in the fovea. There are
many more smaller ones.

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Angioid Streaks

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Diagnosis: Angioid Streaks

Comment to photo: Irregular radiating lines that extend around the disc and from the disc margin into the
midperipheral fundus. The lines represent cracks in the collagenous and elastic parts of Bruch's membrane. Parts of
the cracked lines are hyperpigmented. In this case, despite the extensive lesions, no neovascularization has occured
and vision is normal. Angioid streaks are frequently correllated with pseudoxanthoma elasticum Groblad-
Strandberg.

Diagnosis: Angioid Streaks

Comment to photo :Prominent cracks in Bruch's membrane in a black woman

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Diagnosis: Angioid Streaks

Comment to photo : The right eye shows prominent cracks in Bruch's membrane and macular scars, an indication
of previous neovascularization and hemorrhage.

Diagnosis: Angioid Streaks

Comment to photo: The left eye has excessive concentrically and radially arranged cracks in Bruch's membrane.

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Acquired Macroaneurysm

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Diagnosis: Acquired Macroaneurysm

Comment to photo: Two aneurysms are visible, one large and one small, and are surrounded by circinate material
as an indication of chronic leakage

Diagnosis: Acquired Macroaneurysm, Angiogram

Comment to photo: The angiogram reveals three aneurysms


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Diagnosis: Acquired Macroaneurysm (#1)

Comment to photo: Typically, there are subretinal and intraretinal hemorrhages from the burst aneurysm (here
hidden behind the blood).Note the blood level behind the detached internal limiting membrane (this is not a
preretinal hemorrhage)

Diagnosis: Acquired Macroaneurysm (#2)

Comment to photo: Two weeks later the aneurysm becomes visible as the clot in front of it dissolved into the now
larger pool below.
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Diagnosis: Acquired Macroaneurysm

Comment to photo: Intraretinal hemorrhage underneath the internal limiting membrane. The blood is partially
bleached.

Diagnosis: Acquired Macroaneurysm

Comment to photo: Five weeks later the hemorrhage is absorbed, the aneurysm is visible. Note the arterial shunt
vessels.

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Diagnosis: Macroaneurysm of the Retina, Calcified

Comment to photo: The yellow sphere located in the innermost layers of the retina in the path of an artery
suggests an arterial aneurysm.

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Infections

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Diagnosis: Retinitis, Meningococcus

Comment to photo: The other eye reveals a larger white lesion surrounded by blood. Here one has to assume a
bacterial retinitis with necrosis.

Diagnosis: Retinitis, Meningococcus, Roth spot

Comment to photo: A typical sign of sepsis are centrally white lesions with surrounding hemorrhage (Roth's spot).
The center is an accumulation of white blood cells and does not always contain bacteria.

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71

Diagnosis: Retinitis, Septic

Comment to photo: Twelve year old child with staphylococcal septicemia: papilledema, macular edema, and
hemorrhages.

Diagnosis: Retinitis, Septic

Comment to photo: Peripheral septic retinitis and Roth's spots (whitish center surrounded by hemorrhage).

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72

Diagnosis: Papillitis in Cryptococcal Meningitis, Left Eye

Comment to photo: See description of right eye

Diagnosis: Papillitis and Retinitis in Cryptococcal Meningitis, Right Eye

Comment to photo: 27 yeas old gentleman, diagnosed with AIDS 4 years ago. He also had pulmonary tuberculosis
but compliance to HAART and anti-tuberculous therapy was very poor. He developed cryptococcal meningitis and
complained to blurring of vision bilaterally after 1 month. CD4 count was 171. BCVA was 6/12 OU. Pupils reacted
briskly to direct and consensual light reflexes and without relative afferent papillary defect (RAPD). There was
bilateral optic disk swelling, macular starfolds and increased venous filling, peripapillary flame shaped
hemorrhages. The right retina shows cotton wool spots. Densely white infiltrates of the retina are thought to be
foci of cryptococcal infection. The patient succumbed 2 months later due to fulminant pulmonary tuberculosis.
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73

Diagnosis: Periphlebitis, Frosted

Comment to photo: Frosted periphlebitis can occor as an early sign of a bacterial endophthalmitis (in this case
caused by streptococci). Branches of major veins appear ensheathed and are surrounded by intraretinal
hemorrhages. The angiogram shows leakage from the affected veins.

Diagnosis: Cytomegalo-Virus (CMV) Retinitis

Comment to photo: Very early CMV retinitis simulating HIV microangiopathy in patient with AIDS. White spots to
the right of the optic nerve represent early foci of retinitis, they later enlarged as the retinitis progressed.

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74

Diagnosis: Cytomegalo-Virus (CMV) Retinitis

Comment to photo: In this patient with HIV a severe ocular infection with CMV occurred: whitening of the retina
and hemorrhages from the vessels, which have similarity to a vein occlusion .

Diagnosis: Cytomegalo-Virus (CMV) Retinitis

Comment to photo: Shows the so called brushfire variant of CMV retinitis. Healed atrophic retina (to the left) and
active retinitis (abutting the optic nerve to the right).
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75

Diagnosis: Cytomegalo-Virus(CMV) Retinitis

Comment to photo: Shows the frosted-branch-variant of CMV retinitis. Note the sheathing of venules and area with
retinitis with retinal whitening and intraretinal hemorrhages to right.

Diagnosis: Cytomegalo-Virus(CMV) Retinitis

Comment to photo: CMV retinitis with retinal whitening, intraretinal hemorrhage and arteriitis in a patient with
Hodgkin's disease.

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76

Diagnosis: Cytomegalo-Virus (CMV) Retinits

Comment to photo: Diffuse retinal whitening from CMV retinitis in an AIDS patient

Diagnosis: Cytomegalo-Virus(CMV) Retinitis

Comment to photo: Necrotizing retinitis with intraretinal hemorrhages including the macula. Patient suffers from
AIDS

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77

Diagnosis: Retinitis (Candida Albicans)

Comment to photo: The media are hazy but one sees clearly a whitish lesion between disc and fovea which
represents a retinitis caused by candida

Diagnosis: Retinitis (Candida albicans)

Comment to photo: Several white, preretinal infiltrates

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78

Diagnosis: Retinitis Scar after Candida Albicans Infection

Comment to photo: The strong contraction of the scar resulting in a focal, star-shaped retinal detachment shows
that not only the retina but also the choroid was involved in the original inflammatory process. Underneath the flat,
but well visible retinal folds are glial proliferations. Hard exudate is seen in the macular area.

Diagnosis: Cysticercosis

Comment to photo: Subretinal cyst with invaginated scolex (head). This cyst was observed to have changed
position as documented by the changes in the pigment epithelium.

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79

Diagnosis: Cysticercosis

Comment to photo: Same patient. Intravitreal cyst with evaginated scolex (head). The cysticercus presumably
invaded the vitreous from the macular area where a large chorioretinal scar has developed.

Diagnosis: Cysticercosis

Comment to photo: Signs of intraocular inflammation with hazy media, retinal detachment and whitish subretinal
material. 7-year-old girl. Differential diagnosis: retinoblastoma

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80

Diagnosis: Cysticercosis, Subretinal

Comment to photo: In an area of retinal detachment one sees the Cysticercus. The invaginated white head or
scolex is seen inside the cyst (blurred uppermost part of the picture)

Diagnosis: Diffuse Unilateral Subacute Neuroretinitis (DUSN)

Comment to photo: This worm (Baylisascaris procyonis) is from a pet-racoon. It is usually destroying pigment
epithelium and outer retina from the subretinal space (see insert)resulting in optic atrophie.

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81

Diagnosis: Diffuse Unilateral Subacute Neuroretinitis (DUSN)

Comment to photo: After laser coagulation of the worm (see scar at 10 o'clock in the periphery) atrophy of pigment
epithelium and the optic nerve have progressed and the retina shows signs of subretinal glial proliferation.

Diagnosis: Diffuse Unilateral Subacute Neuroretinitis (DUSN)

Comment to photo: Subretinal parasite (nematode), causing visual loss with vitritis, papillitis, vasculitis and
subretinal lesions.

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82

Diagnosis: Echinococcosis

Comment to photo: Parasite, Echinococcus granulosus (dog tapeworm) in hydatoid cyst, can be found in many
tissues, here in the vitreous of a child.

Diagnosis: Ophthalmomyiasis

Comment to photo: At the level of the retinal pigment epithelium are irregular hypo-pigmented tracks. These
represent most likely the course a sub retinal fly larva took. In this case larvae could not be identified. ( G.O.H.
Naumann)

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83

Diagnosis: Ophthalmomyiasis (#1)

Comment to photo: Subretinal tracks in the RPE layer caused by a fly larva (order of Diptera).

Diagnosis: Toxocariasis

Comment to photo: Inactive lesion of parasite. Scar tissue typically extends from the periphery to the disc area. The
diagnosis is presumptive based on history of contact with dogs and serological tests for toxocara canis.

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84

Diagnosis: Toxocariasis

Comment to photo: The scar tissue of parasite has pulled on retina at the disc and dragged it nasally. The fovea is
now near the edge of the disc.

Diagnosis: Toxocariasis

Comment to photo: Traction is exerted on the macula which is dragged upward and exudate is seen in the fovea.
Visual acuity is 0.05
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85

Diagnosis: Toxocariasis

Comment to photo: Large subretinal granuloma with contraction of the retina and preretinal strand formation. The
peripheral retina is detached.

Diagnosis: Toxocariasis

Comment to photo: Parasitosis, the disc is dragged towards nasally. A fine vitreous strand extends from the disc to
the periphery.

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86

Diagnosis: Toxocariasis

Comment to photo: Scarred down granuloma in the periphery with localized traction detachment. Note the
pigmented subretinal strands as a sign of longstanding detachment.

Diagnosis: Progressive Outer Retinal Necrosis (PORN), With Optic Neuritis

Comment to photo: The fundus of the left eye shows a clear vitreous, multiple whitish lesions in the retina
especially in midperiphery and optociliary vessels of the disc. This patient was under treatment for AIDS, had herpes
zoster several months ago. and experienced sudden loss of vision in the left eye one week ago.

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87

Diagnosis: Progressive Outer Retinal Necrosis (PORN), With Optic Neuritis

Comment to photo: Eight weeks later, despite antiviral therapy increased retinal whitening and retinal vasculitis
with hemorrhages (rare). Now an optic atrophy is visible. Other, right eye, also effected.

Diagnosis: Progressive Outer Retinal Necrosis (PORN), With Optic Neuritis

Comment to photo: One week after change in antiviral therapy the retinal lesions look less active.

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88

Diagnosis: Herpes Zoster Retinitis

Comment to photo: The media are hazy. The retina shows whitening in multiple areas and hemorrhages due to an
occlusive vasculitis with retinal necrosis.

Diagnosis: Herpes Zoster Retinitis

Comment to photo: Advanced stage with vitreous haze, optic atrophy, ensheathed and occluded vessels and retinal
necrosis. Clinically it is not possible to differentiate between herpes simplex and herpes zoster. Herpes zoster proven
by in situ hybridization of the retina in the enucleated eye.

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89

Diagnosis: Progressive Outer Retinal Necrosis (PORN), with AIDS

Comment to photo: Atypical manifestation of progressive outer retinal necrosis (PORN) in AIDS patient with CD4+
T-cell counts more than 100 cells/uL on highly active antiretroviral therapy. Initial visit: Multiple white lesions in the
outer retina with no vitreous haze or signs of vasculitis. Optic disc edema

Diagnosis: Progressive Outer Retinal Necrosis (PORN), with AIDS

Comment to photo: Subsequent rapid spread from the posterior pole to involve the entire retina within 5 days.
Hemorrhages appear, indicating involvement of the inner retina as well.

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90

Diagnosis: Progressive Outer Retinal Necrosis (PORN), with AIDS

Comment to photo: After one month the media are hazy, the whole retina is atrophic. Vessels are ensheathed.
Hemorrhages have spread over a larger area. The optic disc is pale. What started as a case of PORN developed into
a picture reminding one of CMV retinitis.

Diagnosis: Human Immune Deficiency Virus (HIV) Retinopathy

Comment to photo: Microangiopathy with multiple cotton-wool spots in immune suppressed patient.

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91

Diagnosis: Subacute Sclerosing Panencephalitis (SSPE)

Comment to photo: Years after a measles infection the virus can still cause an infection of the retina, and
occasionally the choroid, with lymphocytic infiltration and necrosis. This fundus shows scarring of retina. R.L. Font,
AFIP-Collection, Washington

Diagnosis: Subacute Sclerosing Panencephalitis (SSPE)

Comment to photo: Granular changes of the pigment epithelium with chorioretinal scar above the macula and
tempoal atrophz of the optic nerve.
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92

Diagnosis: Subacute Sclerosing Panencephalitis (SSPE), Gliotic Scars

Comment to photo: Mostly gliotic scars with little hyperpigmentation

Diagnosis: Subacute Sclerosing Panencephalitis (SSPE), Necrotizing Retinitis

Comment to photo: The white necrosis with fuzzy border occurs early in the disease and affects also the outer
retinal layers. Soon pigment derangements and scarring of the retina develop. Several atrophic areas are already
visible. Axons of the ganglion cells in the necrotic areas are visible as bright lines (arrows) along the normal course
of the nerve fiber layer (Bjerrum Aerea). This disease occurs preferably in children who suffered from measles. Visial
disturbances and peculiar behavior are early symptoms which lead to dementia and death.

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93

Diagnosis: Rubella Retinopathy

Comment to photo: The fundus of both eyes of this 8 year old child shows a mottled retinal pigment epithelium:
pepper and salt fundus. Vision is only slightly impaired. Other effects of the viral infection of the mother in the first
two trimesters of the congenital rubella syndrome can be heart disease, cataract, deafness, encephalitis, mental
retardation, etc. .

Diagnosis: Rubella retinopathy

Comment to photo: Extensive pigment epithelium mottling (salt and pepper fundus) after infection of mother in
first trimester of pregnancy. Vision, ERG, and dark adaptation usually normal.

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94

Diagnosis: Miliary tuberculosis

Comment to photo: The pale lesions (marked by arrow) representing tuberculous infiltrates of the choroid.

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Immunologic

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96

Diagnosis: Behcet's Disease, fundus and Optic Atrophy

Comment to photo: After prolonged disease, the media become clearer, the hyperemia of the disc gives way to
paleness, arteries and veins are ensheathed and/or occluded, neovascularization and hemorrhages occur.

Diagnosis: Behcet's Disease

Comment to photo: The view to the fundus is hazy due to vitreous cells. The inferior artery is thin and ensheathed,
temporal inferior and temporal to the disc vascular abnormalities are seen.

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97

Diagnosis: Dermatomyositis, Retinitis

Comment to photo: This is an immune disease which occurs mostly in childhood occludes precapillary arterioles,
capillaries, and postcapillary venules of sceletal muscles and connective tissue with deposits of immunoglobulins,
complement, and inflammatory cells. Here cotton-wool spots are shown in both eyes with a decrease of vision to
light perception. Similar changes occur in lupus erythematodes and rheumatoid polymyalgia.

Diagnosis: Dermatomyositis, Retinopathy

Comment to photo: After treatment with steroids there was nearly full recovery of vision. The cotton-wool spots
regressed and a few intraretinal hemorrhages remain visible.

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98

Diagnosis: Sarcoid Optic Neuropathy

Comment to photo: An elevated granuloma involving the optic disc and adjacent retina. Hard yellowish exudate is
seen radiating from the macula as it is deposited in Henle's layer.

Diagnosis: Sarcoidosis

Comment to photo: The right eye of this black patient shows so called candle-wax dripping, venous exudates and
fine venous sheathing.

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99

Diagnosis: Sarcoidosis

Comment to photo: The left eye has more advanced changes with additional atrophic preretinal proliferation and
submacular exudate.

Diagnosis: Sarcoid, Optic Neuropathy

Comment to photo: This patient reported a six-week history of gradual visual loss in her right eye associated with
persistent orbital pain on the same side. Vision was count fingers, and there was a large afferent pupillary defect.
Note the mild degree of disc swelling and the moderate degree of temporal disc pallor. Nerve fiber layer reflexes
are absent in the papillomacular region.

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100

Diagnosis: Sarcoid Optic Neuropathy

Comment to photo: Edematous disc with neovascularization. Three indistinct whitish nodules appear over the
superior aspect of the disc.

Diagnosis: Sarcoidosis

Comment to photo: Peripheral ensheathing (granulomas) of the vessels. A branch vein occlusion is indicated by the
hemorrhages.
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101

Diagnosis: Sarcoidosis

Comment to photo: Typical so called candle wax drippings, a sign of exudative periphlebitis.

Diagnosis: Sarcoidosis, Papilledema

Comment to photo: Hyperemia of the disc and multiple small yellowish lesions in the choroid. (Biopsy proven case
of sarcoid).
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Haematological

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103

Diagnosis: Anemia

Comment to photo: Cotton-wool spots developed in this woman after prolonged menstrual bleedings with
Hemoglobin of 6.0.

Diagnosis: Anemia, Aplastic

Comment to photo: Intraretinal (sub internal membrane) hemorrhage. Note the well-defined area of hemorrhage
with horizontal (positional) level.

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104

Diagnosis: Anemic Retinopathy, Iron Deficiency

Comment to photo: Multiple intraretinal and preretinal hemorrhages and Roth's spots. 30 year old male with
dimness of vision. Anemia with a Hb of 5.6 gm/dl due to iron deficiency.

Diagnosis: Polycythemia

Comment to photo: Dilated, tortuous arteries and veins and papillary edema are frequently found with
Polycythemia. Often there are also multiple intraretinal hemorrhages .

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105

Diagnosis: Sickle Cell SC Disease

Comment to photo: In the periphery aprupt stop of vessels with proliferation and vascular abnormalities.

Diagnosis: Sichle-Cell SC-Disease

Comment to photo: At the border of vascular to avascular retina new vessels proliferate. Here is a non-perfused
sea-fan of vessels. Traction has caused hemorrhages. The yellow patches are bleached blood. Stage II-IV

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106

Diagnosis: Sickle Cell Retinopathy, Black Sunburst

Comment to photo: In sickle cell retinopathy one often finds jetblack hyperpigmentations near the equator, usually
in the vicinity of an arteriole. These develop after a deep intraretinal hemorrhage which caused a reactive
proliferation of the retinal pigment epithelium.

Diagnosis: Sickle Cell SC Disease

Comment to photo: Severe disease with traction detachment and accumulation of exudate and subretinal strands.

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107

Diagnosis: Sickle Cell Thalassemia Disease (S-thal)

Comment to photo: 12 hours after buckling procedure: arterial occlusions of the retina above the midline with
acute loss of vision.

Diagnosis: Sickle Cell Thalassemia Disease (S-thal), choroidal infarcts

Comment to photo: The same patient has wedge-shaped areas of choroidal and RPE atrophy in the fundus
periphery. They are a sign of choroidal infarcts from previous attacks.

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108

Diagnosis: Sickle Cell Thalassemia Disease (S-thal), Siegrist's Spots

Comment to photo: In the same eye there are Siegrist's spots, a line of pigment clumps. They are a sign of previous
choroidal vascular occlusion.

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Endocrine

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110

Diagnosis: Graves Ophthalmopathy (Thyroid Eye Disease, Graves' Disease), Choroidal Folds

Comment to photo: The fundus shows striae (folds) of the choroid and retina from the pressure of the edematous
orbital tissue.

Diagnosis: Optic Disc in Hypoparathyroidism

Comment to photo: The left optic disc is no longer swollen, but does exhibit mild disc pallor, greater superiorly. An
inferonasal step was present on visual field testing. A lumbar puncture revealed normal intracranial pressure (ICP).
In patients with disc swelling in the setting of hypoparathyroidism, ICP may be either normal or elevated.
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111

Diagnosis: Optic Disc in Hypoparathyroidism

Comment to photo: The right optic disc exhibits chronic disc swelling with hyperemia and venous dilation. Visual
parameters were normal.

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GIT

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113

Diagnosis: Crohn's Disease

Comment to photo :The posterior segment is rarely involved in Crohn's disease. Here are cotton-wool spots in the
fundus periphery as a sign of a peripheral vasculitis.

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Mesenchymal and Skeletal

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115

Diagnosis: Tuberous Sclerosis, (Bourneville's Disease)

Comment to photo: Nine year old girl. The large retinal tumor shows white calcification, the small one is still
somewhat transparent. The tumors are astrocytic hamartomas.

Diagnosis: Tuberous Sclerosis (Bourneville disease), Retina

Comment to photo: Flat hamartoma of the retina, which is difficult to see, because it is semitransparent.
drsuman_chowdhury@yahoo.com
116

Diagnosis: Capillary Hemangioma of the Retina (von Hippel's Disease)

Comment to photo: Several pink hemangiomas of varying sizes are seen. They are often recognized by the
enlarged feeder vessels. New tumors can occur. It is important to recognize them for treatment while they are very
small.

Diagnosis: Capillary Hemangioma of the Retina, (von Hippel's Disease)

Comment to photo: Multiple small pink tumors (capillary hemangiomas) leak extensively causing yellowish
exudates under the retina. Feeder vessels are not yet dilated. An intra- and sub-retinal hemorrhage surrounds
several tumors.

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117

Diagnosis: Capillary Hemangioma of the Disc, von Hippel Syndrome

Comment to photo: Pink, defined tumor over the disc surrounded by areas of light hemorrhage, hard exudate as
sign of chronic leakage into the macula.

Diagnosis: Capillary Hemangioma of the Retina, (von Hippel's disease)

Comment to photo: Yellowish (partially scarred) vascular tumor with tortuous arterial and venous feeder vessels,
which indicate increased blood flow.
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118

Diagnosis: Capillary Hemangioma of the Retina, (von Hippel's Disease)

Comment to photo: A large pink well defined mound is seen in the periphery of the fundus

Diagnosis: Capillary Hemangioma of the Retina, (von Hippel's Disease)

Comment to photo: Dilated tortuous artery and vein are the feeder vessels of the peripheral tumor. Note the
proliferative tissue over the vessel

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119

Diagnosis: Congenital Macrovessels, Wyburn-Mason Syndrome

Comment to photo: Convoluted and anastomosed enlarged vessels. It is very difficult to separate arteries from
veins. Visual acuity is usually decreased. Hemorrhages and exudates may develop. When together with orbital or
cerebral involvement it is called Wyburn-Mason syndrome

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Metabolic

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121

Diagnosis: Primary Oxalosis

Comment to photo: 2-year-old child after kidney transplantation for oxalosis. Multiple patches of
hyperpigmentation and fibrotic appearing geographic areas represent cells containng calcium oxalate. ( G.O.H.
Naumann)

Diagnosis: Anemia, Pernicious (Vitamin B12 deficiency)

Comment to photo: Retinal hemorrhages develop with Vitamin B12 deficiency. The hemorrhage in the fovea is
underneath the internal limiting lamina and has sharp edges, whereas the other ones are in slightly deeper retinal
layers and have diffuse edges.
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122

Diagnosis: Hyper(prebeta) lipoproteinemia, Type 5

Comment to photo: The tortuous vessels have a creamy-white appearance with occasional intraretinal
hemorrhages. Massive extravasation of lipid material into the retina and even into the vitreous. The fundus photo
of this left eye was taken when the patient was severely ill.

Diagnosis: Bassen-Kornzweig Syndrome, (A--Lipoproteinemia)

Comment to photo: Retinitis pigmentosa-like fundus picture: waxy disc. pigmentary changes (bone spicule-like),
narrow vessels.A-beta lipoproteinemia, steatorrhoea.
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123

Diagnosis: Bassen-Kornzweig Syndrome

Comment to photo: Atypical pigmentary changes and loss of mostly rod function. Nightblindness, ataxia,
abetalipoproteinemia and Vitamin A absorbtion deficiency. 23 year old male with good visual acuity.

Diagnosis: Glycolipidosis (Gaucher)

Comment to photo: This retina has a suggestion of grayness .

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124

Diagnosis: Neuronal ceroid lipofuscinosis, Batten-Mayou, Juvenile Amaurotic Idiocy

Comment to photo: Bull's eye maculopathy and optic atrophy. Autosomal recessive, storage of lipopigments.

Diagnosis: Sphingomyelin Lipidosis Type A-D, Niemann-Pick

Comment to photo: The macula shows a cherry red spot surrounded by slowly fading whitish retina (accumulation
of lipid). Eight year old girl. Occurs in 50% of Type A.

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125

Diagnosis: Sphingomyelin Lipidosis Type A-D, Niemann-Pick

Comment to photo: Macular halo occurs in type B as a punctate crystalloid ring. (Type A has in 50% a cherry red
spot of the macula).

Diagnosis: Gangliosidosis GM2, Tay-Sachs Disease, Cherry Red Spot

Comment to photo: 1 1/2 year old child. Cherry red spot surrounded by whitened retina. Similar to Niemann-Pick
disease, except for early visual loss.

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126

Diagnosis: Homocysteinemia (hereditary), Branch Vein Occlusion

Comment to photo: Homocysteinemia occurs with several autosomal recessive diseases involving the metabolism
of homocystein. About 1/2 of the patients are mentally retarded. Osteoporosis and lens luxation are frequent.
Strong tendency of thrombosis. Here one sees branch vein occlusions of the temporal veins in a patient with high
serum homocysteine levels.

Diagnosis: Protein-S Deficiency, Primary Thrombophilia, Retina

Comment to photo: Autosomal dominant enzymopathy. with reduced growth and increased tendency for
thrombosis. The peripheral vascular occlusions have caused development of shunt vessels and hemorrhages.
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127

Diagnosis: Cystinosis

Comment to photo: Patchy depigmentation of the pigment epithelium, progressive from periphery to posterior
pole.

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The End

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