a colour atlas of fundoscopy
TRANSCRIPT
A Colour Atlasof Fundoscopy
First Edition
Contents
Topics Page
Hypertensive and Atherosclerotic 01Diabetic 08Vasculitic 19Venous Occlusion 23Arterial Occlusion 33Radiation Retinopathy 44Tumour 48Acquired Macular Disease 57Angioid Streaks 60Acquired Macroaneurysm 62Infections 67Immunologic 93Haematologic 99Endocrine 106GIT 109Mesenchyme and Skeletal 111Metabolic 117
3
Diagnosis: Arteriosclerosis, of Retinal Vessels
Comment to photo: Sclerosis of arterial vessels is shown with thickening of the wall and disappearing visibility ofthe 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.
4
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 areseen and a little bit of exudate.
5
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)
6
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 whitecircumferential line may represent the edge of vitreous detachment. Proteinaceous material is deposited in theretina outside this ring. This photo was taken when the patient was lying on his right side to show the mobility ofthe 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 andthe protein outside the ring have disappeared,
7
Diagnosis: Hypertensive Retinopathy, Intraretinal Hemorrhage
Comment to photo: Centrally there is a well-defined accumulation of blood under detached internal limitinglamina. A second ring of blood has less well-defined borders and represents preretinal blood where the vitreous isdetached from the retina.
Diagnosis: Papilledema showing blurred disc margins and dilated tortuous vessels
9
Diagnosis: Diabetes mellitus, Lipemia Retinalis in Hypertriglyceridemia
Comment to photo: 16 year old patient with diabetis mellitus with hypertriglyceridemia. The vessels look creamyand 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.
10
Diagnosis: Diabetic Retinopathy, Nonproliferative, Angiogram
Comment to photo: The angiogram reveals even more bright spots than the clinical picture suggests with subtlestaining 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. Thisbecomes clearly visible in the angiogram. In addition one recognizes a subtle stain of the retina (edema) in thisarea.
11
Diagnosis: Diabetic Retinopathy, Nonproliferative
Comment to photo: Many superficial, intraretinal hemorrhages. The fluffy, white spots, cotton wool spots, aremicroinfarcts.
Diagnosis: Diabetic Retinopathy, nonproliferative
Comment to photo: The upper picture shows the fundus of a diabetic with very early changes. There is a smallcotton-wool spot and a small intraretinal hemorrhage. Some time later a lot of microaneurysms and accumulationsmicroaneurysms are found above the fovea along the supero-temporal artery in the eye of the same patient (lowerpicture). Hard exudates become visible.
12
Diagnosis: Diabetic Retinopathy, Nonproliferative, Severe
Comment to photo: The fundus shows multiple blot and dot hemorrhages. Many hard exudates are a sign ofchronic leakage. The difficulty to visualize a clear choroidal pattern also indicates diffuse edema of the retina. Thedisc 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 7years. Only with close observation microaneurysms are detected near the fovea.
13
Diagnosis: Diabetic Retinopathy, Proliferative (PDR)
Comment to photo: One has to look carefully to see the heavy neovascularization that extends along the posteriorsurface 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 showsmicroaneurysms, small dot hemorrhages, and hard intraretinal exudate. The slight discoloration of the retina in thearea of the superior arcade indicates retinal edema.
14
Diagnosis: Proliferative Diabetic Retinopathy (PDR), Severe #1
Comment to photo: Fibrovascular proliferations at the disc and along the superior and inferior arcade havecontracted and because of their intimate connections with the retina, detached the retina. Over the macula theposterior vitreous is detached since there are no vascular connectons. The elevated retina together with the flatposterior 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 belocated at the proliferation of the 6 o'clock vein and at 7 o'clock. The hemorrhagic streaks toward the maculaindicate a subhyaloid connection to this area.
15
Diagnosis: Diabetic Retinopathy, Proliferative (PDR)
Comment to photo: There are multiple signs of diabetic retinopathy: venous beading, hemorrhages, cotton-woolspots, intraretinal vascular abnormalities (IRMA), but most important for the prognosis is the neovascularization ofthe disc (NVD).
Diagnosis: Diabetic Retinopathy, Proliferative (PDR)
Comment to photo: Massive neovascularization along the retinal surface (this indicates that the vitreous is notdetache
16
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: ausgeprägte kapillarfreie Zonen, am Rand davon ausgeprägte Gefäßneubildung von retinalenNetzhautgefäßen ausgehend (Neovaskularisation)
17
Diagnosis: Retinopathia diabetica, RA, zentraler Fundus, Farbbild
Comment to photo: retinale Blutungen, ausgeprägte Gefäßneubildung von papillennahen Netzhautgefäßenausgehend (Neovaskularisation oder Wundernetz)
Diagnosis: Diabetic Retinopathy (#1), Proliferative after Vitrectomy
Comment to photo: Rarely, despite successful vitreous surgery, small convoluted new vessels do develop. Theygrow very little and may bleed.
18
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 onthe fundus photograph. This may indicate that the new vessels initially are intraretinal and only later break throughthe internal lamina.
Diagnosis: Proliferative diabetic retinopathy
20
Diagnosis: Polyarteritis (Periarteritis) Nodosa
Comment to photo: As an expression of the widespread arteritis many arterial occlusions (cotton-wool spots) andhemorrhages are seen. The vessels are dilated and tortuous and leak as evidenced by the exudates in the maculararea.
Diagnosis: Lupus Erythematodes (LE)
Comment to photo: Multiple cotton-wool spots as a sign of the microvascular occlusive disease.
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. (30yofemale patient with confirmed MS)
22
Diagnosis: Multiple Sclerosis, Ensheathed Veins (M. Rucker)
Comment to photo: Peripheral veins are surrounded by a whitish inflammatory infiltrate and are thereforeobscured. 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 differentialdiagnoses includes infectious diseases (i.e. syphilis), collagenoses, sarcoid and idiopathic retinal vasculitis.
23
Diagnosis: Purtscher's-like Retinopathy
Comment to photo: In collagen vascular disease (here polyarteritis) a coagulopathy may occur with occlusion ofsmall 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.
25
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 flameshaped hemorrhages and cotton wool spots in the temporal retina with macular edema. Simultaneous superior andinferior temporal branch retinal vein occlusion is not commonly encountered. 55 year old hypertensive lady withend 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 thevein occlusion
26
Diagnosis: Branch Vein and Artery Occlusion, Old
Comment to photo: There are corkscew vessels near the temporal raphe. They indicate connections between theinferior 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 aswell as the artery. The artery may have been occluded at the kink. Loss of capillary network is seen in the area ofprevious combined occclusion.
27
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 hardexudates.
28
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 tortuousin some areas. Small retinal hemorrhages and some hard exudates near the fovea are seen in the retina. The viewto the retina is obscured by membranes that are in front of the retina. A barely visible traction band pulls on thevein in the direction of the disc where one sees neovascular tissue. The retinal neovascularization is easilyoverlooked but becomes very obvious in the angiogram.
29
Diagnosis: Branch Vein Occlusion (BVO) (#4)
Comment to photo: Nine months later the hemorrhage is absorbed. There are several corkscrew vessels as a sign ofslowed-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 andfollows the nerve fibers along their course.
30
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 upto and including the foveal area and cotton-wool spots. The angiogram demonstrates leakage of the parafovealvessels. 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 onlydiscrete retinal hemorrhages.
31
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 blowncentral retinal vein occlusion with elevated disc and blurred margins, semicircular retinal folds and massiveintraretinal hemorrhages
Diagnosis: Impending Vein Occlusion
Comment to photo: This 27 year old female patient on examination of the right eye with normal VA showedconsiderable tortuosity of the vessels with enlargement of the veins and slight hyperemia and blurring of the discmargin: signs of an impending vein occlusion.
32
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.
33
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 badprognosis for visual recovery.
35
Diagnosis: Branch Artery Occlusion, acute
Comment to photo: Multiple cotton-wool spots indicate many micro-infarcts in the area of the temporal superiorartery.
Diagnosis: Branch Artery Occlusion, acute
Comment to photo: White material (calcified?) occludes the superior artery and causes edema of the retina in itsarea of distribution.
36
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 toderive 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.
37
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 embolusfrom vessels with arterosclerotic changes (Carotid). It is composed of cholesterol and lipid. It may or may notocclude 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 arteryoriginates near the emerging central vessels as a very early branch of the inferior arcade. The retina is edematousin its area of distribution. Careful examination reveals what looks like a yellowish obstruction near the nasal marginof the disc at 4 o'clock. Multiple yellowish particles are seen in the vessel above the fovea.
38
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.
39
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-venouscrossing. At the biforcation of an artery a yellowish plaque (Hollenhorst Plaque) is thought to be an embolus fromvessels with arterosclerotic changes (Carotid). It is composed of cholesterol and lipid. It may or may not occlude thevessel.
40
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 discnot 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 socalled box-car formation in the vessels.
41
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 thepapillo-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.
42
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-carformation 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.
43
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 seethe 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 slowperfusion.
44
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 recentartery occlusion. Box-carring of the blood collumn in some vein cause by stagnant blood flow. A patent cilioretinalvessel 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 ofvessels.
46
Diagnosis: Radiation Retinopathy
Comment to photo: Small hemorrhages, vascular dilatations, macular edema and exudates have developed afterirradiation.
Diagnosis: Radiation Retinopathy
Comment to photo: Radiation changes are progressive: one year later there are more vascular abnormalities andthe edema and exudates have increased.
47
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.
48
Diagnosis: Radiation Retinopathy
Comment to photo: This retinopathy usually occurs 12-18 months after damage to the retina following eitherorbital or ocular radiotherapy. It is a microangiopathy characterized by vascular occlusion and altered vascularpermeability. The fundus findings are similar to diabetic retinopathy. Cotton wool spots indicate infarction of thenerve fiber layer and there are commonly noted in the posterior pole as the nerve fiber layer is thickest there. This37 years old gentleman had radiotherapy a year ago for nasopharyngeal carcinoma. He presented with blurring ofvision of 2 weeks duration. VA of both eyes were 0.6. Right eye: multiple cotton wool spots in all 4 quadrants of theposterior pole. Dot-blot hemorrhages, flame shaped hemorrhages and sub-RPE hemorrhage. No hard exudate orneovascularization.
Diagnosis: Retinale angiomatöse Proliferation (LA, Farbbild)
Comment to photo: paramakuläres retinales Ödem, retinale Blutungen, harte Exsudate, Abhebung derneurosensorischen Netzhaut, Drusen und Pigmentepithelveränderungen
50
Diagnosis: Astrocytic (Glial) Hamartoma
Comment to photo: Congenital glial tumor that is sharply delineated, originally dome shaped, later flat, andchalky-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 asisolated findings as here in a healthy 74 year old man. The tumor seems to be composed of many globules.
51
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 vascularizationwith rapid leakage.
52
Diagnosis: Reactive Astrocytic Hyperplasia
Comment to photo: Rapidly growing vascularized benign retinal tumor (thought to be caused by traction frompreretinal 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 canbe appreciated at the periphery of the lesion superonasally and inferonasally. Fine superficial radial folds indicatepreretinal proliferation. A single flame hemorrhage is seen at 11 o'clock.
53
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.
54
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.
55
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 adenomatouspolyposis, 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 familialadenomatous polyposis (which often develops into cancer) and other hamartomas of the skeleton and soft tissues.
56
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 ofprevious child
57
Diagnosis:RetinoblastomaComment to photo:Small spontaneously regressing tumor beginning to calcify in 4 yearold child
Diagnosis: Retinoblastoma, After Irradiation
Comment to photo: Calcified remnant of tumor after radiation therapy
59
Diagnosis: Cystoid Macular Edema
Comment to photo: The macula of this aphakic patient looks normal at first glance. The slight swelling and thecystoid spaces of the foveal area are often beyond the resolution of a photograph but can be seenbiomicroscopically.
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.
60
Diagnosis: Cystoid Macular Edema
Comment to photo: One has to look very carfully to detect the two large cystoid spaces in the fovea. There aremany more smaller ones.
62
Diagnosis: Angioid Streaks
Comment to photo: Irregular radiating lines that extend around the disc and from the disc margin into themidperipheral fundus. The lines represent cracks in the collagenous and elastic parts of Bruch's membrane. Parts ofthe cracked lines are hyperpigmented. In this case, despite the extensive lesions, no neovascularization has occuredand 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
63
Diagnosis: Angioid Streaks
Comment to photo : The right eye shows prominent cracks in Bruch's membrane and macular scars, an indicationof previous neovascularization and hemorrhage.
Diagnosis: Angioid Streaks
Comment to photo: The left eye has excessive concentrically and radially arranged cracks in Bruch's membrane.
65
Diagnosis: Acquired Macroaneurysm
Comment to photo: Two aneurysms are visible, one large and one small, and are surrounded by circinate materialas an indication of chronic leakage
Diagnosis: Acquired Macroaneurysm, Angiogram
Comment to photo: The angiogram reveals three aneurysms
66
Diagnosis: Acquired Macroaneurysm (#1)
Comment to photo: Typically, there are subretinal and intraretinal hemorrhages from the burst aneurysm (herehidden behind the blood).Note the blood level behind the detached internal limiting membrane (this is not apreretinal 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 nowlarger pool below.
67
Diagnosis: Acquired Macroaneurysm
Comment to photo: Intraretinal hemorrhage underneath the internal limiting membrane. The blood is partiallybleached.
Diagnosis: Acquired Macroaneurysm
Comment to photo: Five weeks later the hemorrhage is absorbed, the aneurysm is visible. Note the arterial shuntvessels.
68
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 arterysuggests an arterial aneurysm.
70
Diagnosis: Retinitis, Meningococcus
Comment to photo: The other eye reveals a larger white lesion surrounded by blood. Here one has to assume abacterial 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.
71
Diagnosis: Retinitis, Septic
Comment to photo: Twelve year old child with staphylococcal septicemia: papilledema, macular edema, andhemorrhages.
Diagnosis: Retinitis, Septic
Comment to photo: Peripheral septic retinitis and Roth's spots (whitish center surrounded by hemorrhage).
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 tuberculosisbut compliance to HAART and anti-tuberculous therapy was very poor. He developed cryptococcal meningitis andcomplained to blurring of vision bilaterally after 1 month. CD4 count was 171. BCVA was 6/12 OU. Pupils reactedbriskly to direct and consensual light reflexes and without relative afferent papillary defect (RAPD). There wasbilateral optic disk swelling, macular starfolds and increased venous filling, peripapillary flame shapedhemorrhages. The right retina shows cotton wool spots. Densely white infiltrates of the retina are thought to befoci of cryptococcal infection. The patient succumbed 2 months later due to fulminant pulmonary tuberculosis.
73
Diagnosis: Periphlebitis, Frosted
Comment to photo: Frosted periphlebitis can occor as an early sign of a bacterial endophthalmitis (in this casecaused by streptococci). Branches of major veins appear ensheathed and are surrounded by intraretinalhemorrhages. 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 tothe right of the optic nerve represent early foci of retinitis, they later enlarged as the retinitis progressed.
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) andactive retinitis (abutting the optic nerve to the right).
75
Diagnosis: Cytomegalo-Virus(CMV) Retinitis
Comment to photo: Shows the frosted-branch-variant of CMV retinitis. Note the sheathing of venules and area withretinitis 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 withHodgkin's disease.
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 fromAIDS
77
Diagnosis: Retinitis (Candida Albicans)
Comment to photo: The media are hazy but one sees clearly a whitish lesion between disc and fovea whichrepresents a retinitis caused by candida
Diagnosis: Retinitis (Candida albicans)
Comment to photo: Several white, preretinal infiltrates
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 showsthat 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 changedposition as documented by the changes in the pigment epithelium.
79
Diagnosis: Cysticercosis
Comment to photo: Same patient. Intravitreal cyst with evaginated scolex (head). The cysticercus presumablyinvaded 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 subretinalmaterial. 7-year-old girl. Differential diagnosis: retinoblastoma
80
Diagnosis: Cysticercosis, Subretinal
Comment to photo: In an area of retinal detachment one sees the Cysticercus. The invaginated white head orscolex 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 pigmentepithelium and outer retina from the subretinal space (see insert)resulting in optic atrophie.
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 pigmentepithelium 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 andsubretinal lesions.
82
Diagnosis: Echinococcosis
Comment to photo: Parasite, Echinococcus granulosus (dog tapeworm) in hydatoid cyst, can be found in manytissues, 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. Theserepresent most likely the course a sub retinal fly larva took. In this case larvae could not be identified. ( G.O.H.Naumann)
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. Thediagnosis is presumptive based on history of contact with dogs and serological tests for toxocara canis.
84
Diagnosis: Toxocariasis
Comment to photo: The scar tissue of parasite has pulled on retina at the disc and dragged it nasally. The fovea isnow 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
85
Diagnosis: Toxocariasis
Comment to photo: Large subretinal granuloma with contraction of the retina and preretinal strand formation. Theperipheral retina is detached.
Diagnosis: Toxocariasis
Comment to photo: Parasitosis, the disc is dragged towards nasally. A fine vitreous strand extends from the disc tothe periphery.
86
Diagnosis: Toxocariasis
Comment to photo: Scarred down granuloma in the periphery with localized traction detachment. Note thepigmented 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 retinaespecially in midperiphery and optociliary vessels of the disc. This patient was under treatment for AIDS, had herpeszoster several months ago. and experienced sudden loss of vision in the left eye one week ago.
87
Diagnosis: Progressive Outer Retinal Necrosis (PORN), With Optic Neuritis
Comment to photo: Eight weeks later, despite antiviral therapy increased retinal whitening and retinal vasculitiswith 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.
88
Diagnosis: Herpes Zoster Retinitis
Comment to photo: The media are hazy. The retina shows whitening in multiple areas and hemorrhages due to anocclusive vasculitis with retinal necrosis.
Diagnosis: Herpes Zoster Retinitis
Comment to photo: Advanced stage with vitreous haze, optic atrophy, ensheathed and occluded vessels and retinalnecrosis. Clinically it is not possible to differentiate between herpes simplex and herpes zoster. Herpes zoster provenby in situ hybridization of the retina in the enucleated eye.
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 theouter 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.
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 intoa 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.
91
Diagnosis: Subacute Sclerosing Panencephalitis (SSPE)
Comment to photo: Years after a measles infection the virus can still cause an infection of the retina, andoccasionally 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 andtempoal atrophz of the optic nerve.
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 outerretinal layers. Soon pigment derangements and scarring of the retina develop. Several atrophic areas are alreadyvisible. Axons of the ganglion cells in the necrotic areas are visible as bright lines (arrows) along the normal courseof the nerve fiber layer (Bjerrum Aerea). This disease occurs preferably in children who suffered from measles. Visialdisturbances and peculiar behavior are early symptoms which lead to dementia and death.
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 firsttwo trimesters of the congenital rubella syndrome can be heart disease, cataract, deafness, encephalitis, mentalretardation, etc. .
Diagnosis: Rubella retinopathy
Comment to photo: Extensive pigment epithelium mottling (salt and pepper fundus) after infection of mother infirst trimester of pregnancy. Vision, ERG, and dark adaptation usually normal.
94
Diagnosis: Miliary tuberculosis
Comment to photo: The pale lesions (marked by arrow) representing tuberculous infiltrates of the choroid.
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 topaleness, 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.
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 tolight 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 spotsregressed and a few intraretinal hemorrhages remain visible.
98
Diagnosis: Sarcoid Optic Neuropathy
Comment to photo: An elevated granuloma involving the optic disc and adjacent retina. Hard yellowish exudate isseen 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 andfine venous sheathing.
99
Diagnosis: Sarcoidosis
Comment to photo: The left eye has more advanced changes with additional atrophic preretinal proliferation andsubmacular exudate.
Diagnosis: Sarcoid, Optic Neuropathy
Comment to photo: This patient reported a six-week history of gradual visual loss in her right eye associated withpersistent 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 reflexesare absent in the papillomacular region.
100
Diagnosis: Sarcoid Optic Neuropathy
Comment to photo: Edematous disc with neovascularization. Three indistinct whitish nodules appear over thesuperior aspect of the disc.
Diagnosis: Sarcoidosis
Comment to photo: Peripheral ensheathing (granulomas) of the vessels. A branch vein occlusion is indicated by thehemorrhages.
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 caseof sarcoid).
103
Diagnosis: Anemia
Comment to photo: Cotton-wool spots developed in this woman after prolonged menstrual bleedings withHemoglobin of 6.0.
Diagnosis: Anemia, Aplastic
Comment to photo: Intraretinal (sub internal membrane) hemorrhage. Note the well-defined area of hemorrhagewith horizontal (positional) level.
104
Diagnosis: Anemic Retinopathy, Iron Deficiency
Comment to photo: Multiple intraretinal and preretinal hemorrhages and Roth's spots. 30 year old male withdimness 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.
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-perfusedsea-fan of vessels. Traction has caused hemorrhages. The yellow patches are bleached blood. Stage II-IV
106
Diagnosis: Sickle Cell Retinopathy, Black Sunburst
Comment to photo: In sickle cell retinopathy one often finds jetblack hyperpigmentations near the equator, usuallyin the vicinity of an arteriole. These develop after a deep intraretinal hemorrhage which caused a reactiveproliferation 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.
107
Diagnosis: Sickle Cell Thalassemia Disease (S-thal)
Comment to photo: 12 hours after buckling procedure: arterial occlusions of the retina above the midline withacute 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 fundusperiphery. They are a sign of choroidal infarcts from previous attacks.
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 previouschoroidal vascular occlusion.
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 edematousorbital 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. Aninferonasal 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.
111
Diagnosis: Optic Disc in Hypoparathyroidism
Comment to photo: The right optic disc exhibits chronic disc swelling with hyperemia and venous dilation. Visualparameters were normal.
113
Diagnosis: Crohn's Disease
Comment to photo :The posterior segment is rarely involved in Crohn's disease. Here are cotton-wool spots in thefundus periphery as a sign of a peripheral vasculitis.
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 stillsomewhat 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.
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 theenlarged feeder vessels. New tumors can occur. It is important to recognize them for treatment while they are verysmall.
Diagnosis: Capillary Hemangioma of the Retina, (von Hippel's Disease)
Comment to photo: Multiple small pink tumors (capillary hemangiomas) leak extensively causing yellowishexudates under the retina. Feeder vessels are not yet dilated. An intra- and sub-retinal hemorrhage surroundsseveral tumors.
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 assign 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.
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 theproliferative tissue over the vessel
119
Diagnosis: Congenital Macrovessels, Wyburn-Mason Syndrome
Comment to photo: Convoluted and anastomosed enlarged vessels. It is very difficult to separate arteries fromveins. Visual acuity is usually decreased. Hemorrhages and exudates may develop. When together with orbital orcerebral involvement it is called Wyburn-Mason syndrome
121
Diagnosis: Primary Oxalosis
Comment to photo: 2-year-old child after kidney transplantation for oxalosis. Multiple patches ofhyperpigmentation 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 isunderneath the internal limiting lamina and has sharp edges, whereas the other ones are in slightly deeper retinallayers and have diffuse edges.
122
Diagnosis: Hyper(prebeta) lipoproteinemia, Type 5
Comment to photo: The tortuous vessels have a creamy-white appearance with occasional intraretinalhemorrhages. Massive extravasation of lipid material into the retina and even into the vitreous. The fundus photoof this left eye was taken when the patient was severely ill.
Diagnosis: Bassen-Kornzweig Syndrome, (A-β-Lipoproteinemia)
Comment to photo: Retinitis pigmentosa-like fundus picture: waxy disc. pigmentary changes (bone spicule-like),narrow vessels.A-beta lipoproteinemia, steatorrhoea.
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 .
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 (accumulationof lipid). Eight year old girl. Occurs in 50% of Type A.
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 redspot 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-Pickdisease, except for early visual loss.
126
Diagnosis: Homocysteinemia (hereditary), Branch Vein Occlusion
Comment to photo: Homocysteinemia occurs with several autosomal recessive diseases involving the metabolismof 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 highserum homocysteine levels.
Diagnosis: Protein-S Deficiency, Primary Thrombophilia, Retina
Comment to photo: Autosomal dominant enzymopathy. with reduced growth and increased tendency forthrombosis. The peripheral vascular occlusions have caused development of shunt vessels and hemorrhages.
127
Diagnosis: Cystinosis
Comment to photo: Patchy depigmentation of the pigment epithelium, progressive from periphery to posteriorpole.
