Lesions of retina

fundus photographs of various lesions of the retina

1. LESIONS OF RETINA 1 DR SHRUTI LADDHA

2. Anatomy of the retina Extends from ora serrata anteriorly to the optic nerve posteriorly. Central retina - 5 to 6 mm in diameter centred on the fovea Clinically peripheral retina - from equator to ora serrata. Equator - vortex vein .

3. Macula (5.5 mm) Perifovea(1.5 mm) Parafovea (0.5 mm) Fovea (1.5 mm) Foveola(0.35 mm) umbo

4. Peripheral retina can be divided into – near ,mid ,far periphery. 1.5-mm ring peripheral to the temporal major vascular arcades called the near periphery. The midperiphery consists of an annular area 3 mm wide surrounding the near peripheral retina. The far peripheral retina extends in width 9 to 10 mm beyond the midperipheral retina temporally and 16 mm nasally.

5. Photograph of a normal disk Points to note : C olor Size shape margins the cup neuroretinal rim vessels Spontaneous venous pulsations peripapillary area

6. Normal arterioles and venules. The arterioles are thinner, red in color . The venules are broader, flatter, and darker red in color.

7. At arteriovenous crossings the arterioles may cross over the venules or the venules may cross over the arterioles Though the former is more common

8. The retina has an orangish color which varies with the degree of pigmentation in the pigment epithelium. Some normal variations of the pigment in the RPE can allow the orange stripes of choroidal vessels to be seen giving it a tigroid appearance. These vessels are better seen in the mid-periphery and beyond. Choroidal vessels seen as orange tape like criss-crossing bands

9. The ampulla of a vortex vein is seen as an orange bulb with choroidal veins draining into it .

10. Variations of normal optic nerve head A normal disk in which the cup cannot be appreciated A normal disk with a small cup

11. Physiologically enlarged cup - neuroretinal rim healthy pink. ISNT rule followed. No bayonetting, notching. A normal disk with blurred margins and absent cup giving a false impression of disk edema

12. H ypermetropic disc- Small elevated pink disc, small or no cup, normal vessels, crowding. Blurring of the disk margins (initially the superior and inferior) with absent cup raises suspicions of early papilledema. Presence of venous pulsations or the ability to induce them, suggests absence of it.

13. BILATERAL SWOLLEN DISC WITH HYPERAEMIA

14. P apilledema - hyperemic protuberant disk with blurred margins and obscured cup. The blood vessels on the disk appear to climb down from the elevated margins towards the surrounding retina and at places appear to be hidden by the swollen fibers. The veins of the retina are engorged. There is surrounding peripapillary edema .

15. Established papilledema with blurring of the entire margin of the optic disk with some elevation, dilated turgid and tortuous veins, soft exudates and few retinal hemorrhages . Chronic papilledema with blurring of the entire margin of an elevated optic disk with deposition of hard exudates

16. Patons line : Circumferential retinal folds due to papilloedema

17. Hypertensive optic neuropathy - optic nerve head edema is present along with other signs of hypertensive retinopathy - retinal hemorrhages, cotton-wools spots, macular edema, macular star, arteriovenous crossing changes. These changes are bilateral .

18. D iabetic papillopathy - unilateral or more commonly bilateral hyperemic swollen disks maybe present in association with mild to moderate visual loss. Other changes of diabetic retinopathy may be absent.

19. TOXIC OPTIC NEUROPATHY Hyperemic disks with blurred margins in patients, who have been addicted to alcohol and tobacco over a prolonged period of time , must be suspected to have ( tobacco-alcohol) toxic optic neuropathy . The disk swelling is less and the vascular changes of papilledema are not seen. Later in the course of the disease the disks become pale and excavated.

20. METHYL ALCOHOL POSIONING A dramatic and bilateral loss of vision with bilateral swollen hyperemic disks along with marked attenuation of the blood vessels maybe seen in methyl alcohol poisoning . These disks rapidly turn pale . In the majority, by the time the patient presents the disks are invariably pale.

21. LEBERS HEREDITERY OPTIC NEUROPATHY B ilateral hyperemic disks with blurred margins may be seen in young patients, usually male, in Leber’s hereditary optic neuropathy. The optic disks look hyperemic with tortuous and telangiectatic vessels and blurred margins. However, the disk prominence is mild. A peculiar swelling of the fibers around the disk has also been described ( pseudoedema ). The two eyes are involved in rapid succession which then proceeds to optic atrophy

22. Acute stage – hyperaemic disc swelling with blurring of disc margins Marked telangiectatic microangiopathy Late stage- atrophic appearance

23. Patients on chronic therapy with the antiarrhythmic drug Amiodarone may develop an optic neuropathy showing hyperemic disks with blurred margins. The cornea shows superficial brown deposits of the drug in characteristic whorls.

24. UNILATERAL SWOLLEN DISC WITH HYPERAEMIA

25. Optic neuritis – peripapillary flame shaped hemorhages and a macular star

26. Hyperemic swollen disks are the hallmark of optic neuritis which is usually unilateral in adults but can be bilateral in children. If it is associated with paraplegia or other neurological defects that may occur before or after the optic neuritis it is likely to be neuromyelitis optica . Bilateral optic neuritis in adults is rare and usually associated with extensive sinusitis and syphilitic meningitis.

27. Mild optic disk edema associated with retinal edema and hard exudates deposited as and incomplete macular star in Neuroretinitis. The disk edema is relatively milder than in optic neuritis and the macular star appears early Long standing neuroretinitis showing features of chronic disk and macular edema, resolving macular star, dilated epipapillary vessels and retinal striae ( Paton’s lines )

28. Hyperemic disk edema associated with central retinal vein occlusion Papillophlebitis. There are multiple soft exudates, some hemorrhages and patches of retinal edema especially in the inferior retina

29. Hyperemic disk edema associated with posterior uveitis ( Vogt Koyanagi Harada syndrome ). Multiple patches of choroiditis are seen at the posterior pole

30. BILATERAL OPTIC NERVE HEAD SWELLING WITH PALLOR

31. Optic nerve head studded with refractile globular deposits of varying size that reflect light brightly, in a patient with optic nerve head drusen The drusen exhibit autofluorescence. This feature clinches the diagnosis .

32. A pale swollen disk in anterior ischemic optic neuropathy (the fuzzy looking swollen tissue along with pallor gives the disk a ‘milky appearance’). On FFA the disk shows patches of hypoflourescence due to nonperfusion (arrows)

33. Atrophy of the papillomacular bundle results in temporal pallor and commonly occurs following retrobulbar neuritis and bilaterally in pituitary tumors. A band or bowtie pallor with sparing of the superior and inferior parts of the disk implies optic tract involvement.

34. PRIMARY OPTIC ATROPHY • no prior optic disc edema A process affecting the visual pathways from the retrolaminar portion of the optic nerve to the lateral geniculate nucleus • White disc with a clearly delineated outline • Reduction in the number of small vessels on the disc. • Attenuation of the peripapillary vessels. • Thinning of the retinal nerve fibre layer. Optic neuritis Trauma Nutritional deficiency Toxic Pituitary tumours Optic nerve tumours

35. SECONDARY OPTIC ATROPHY Long-standing swelling of the optic nerve head. • D irty grey slightly raised disc with poorly delineated margins • Reduction in number of small vessels on the disc. • Surrounding ‘water marks’ – paton lines. • Chronic papilloedema. • Anterior ischaemic optic neuropathy. • Papillitis.

36. CONSECUTIVE: • Diseases of the inner retina or its blood supply 1. Vascular attenuation or sheathing Old CRVO or ophthalmic artery Occlusion Retinitis pigmentosa Toxic optic neuropathy Old vasculitis as in Behçet disease 2. Macular lesions giving rise to temporal disc pallor Toxoplasmosis Cone dystrophy 3. Chorioretinal Degenerative myopia Extensive pan retinal photocoagulation 4. Diffuse retinal necrosis Cytomegalovirus retinitis Acute retinal necrosis Progressive outer retinal necrosis

37. GLAUCOMATOUS OPTIC ATROPHY Large cup with notching and thinning of the neuroretinal rim, and bayoneting of blood vessels ‘ISNT’ rule is broken. Disc pallor and peripapillary atrophy. Retinal nerve fibre layer defects best seen with red-free light

38. A small, oval disk that is abnormally tilted such that the long axis of the disk lies obliquely is called a tilted disk . There is thinning and hypopigmentation of the inferonasal retinal pigment epithelium and choroid A severely tilted disk in which the disk appears to be almost horizontally placed. Obliquity in the anteroposterior axis causes the disk to look small as seen ‘end on’

39. HYPOPLASTIC DISC A congenital, unilateral or bilateral Small disc surrounded by a halo of pigmentary change (double-ring sign) Blood vessels have normal calibre although they are often tortuous Albinism and foveal hypoplasia Microphthalmos Aniridia Persistent hyaloid artery

40. LARGE DISC HIGH MYOPIA The appearance of the large disc may be exaggerated by surrounding chorioretinal atrophy Coloboma A unilateral or bilateral, congenital, condition caused by failure of fusion of the fetal fissure and contains a partial inferior excavation that has a glistening appearance

41. CONGENITAL PIT An isolated, usually unilateral, congenital, condition that is associated with mild enlargement of the optic nerve head. The pit is a round or oval depression of variable size, most frequently involving the temporal margin of the disc COMBINED COLOBOMA AND PIT Temporal pit and an inferior coloboma is an extremely rare anomaly

42. MEGALOPAPILLA A very rare usually bilateral congenital condition in which the disc diameter is 2.1 mm or more. Large cup–disc ratio with a round or horizontally oval cup without vertical notching Reduced distance between the temporal edge of the disc and the foveola by approximately one disc diameter.

43. DRAGGED’ DISC Retinopathy of prematurity A bilateral proliferative retinopathy that affects premature infants of low birth weight who have been exposed to high ambient oxygen concentrations. Temporal disc and macula dragging occurs in advanced cicatricial disease PERSISTENT HYPERPLASTIC PRIMARY VITREOUS is seen as a membrane that fans out from the optic nerve head extending anteriorly and is often associated with a fold of the retina

44. MYELINATED NERVE FIBRES- Feathery white patches seen fanning out from the disc BERGMEISTER’S PAPILLA Small grayish wisp of tissue attached to the disk surface is likely to be an embryonic remnant of the hyaloid system .

45. MELANOCYTOMA OF THE OPTIC NERVE HEAD appearing as a black mass obscuring the disk

46. SPLINTER HEMORRHAGES u ncontrolled glaucoma, anterior ischemic optic neuropathy, central retinal vein occlusion

47. NEW VESSELS ON THE DISK (NVD) appear as a network of fine vessels arborising on the surface of the disk- the rest of the retina shows features of the underlying disease such as retinal hemorrhages, exudates COLLATERALS ON THE OPTIC DISK appear as small clumps of vessels that are tortuous and curly

48. FIBROVASCULAR PROLIFERATION on the optic disk look like grayish white membranes situated on the disk surface and growing into the vitreous. Proliferating vessels are seen within the membrane. These vessels can disappear as the proliferation regresses leaving a sheet of dense fibrous tissue. The rest of the fundus shows features of the causative retinal pathology

49. LESIONS OF VITREOUS

50. SMALL VITROUS OPACITIES Dull fundal glow. Small white opacities with thread like aggregates floating in a more liquified vitreous are likely due to degenerated vitreous. Due to aging. May occur early in myopia, inflammation , trauma, surgery, hereditary vitreoretinal degeneration .

51. MEDIUM SIZE OPACITY Weiss ring-PVD

52. Inflammatory exudates that form clumps and form snow ball like opacities, often seen over inferior retina

53. Glittering yellow deposits of synchiasis scintillans which fly around within the liquefied vitreous. At rest, they settle down only to be scattered like a ‘golden shower ‘with the next eye movement Multiple small yellowish- white deposits of Asteroid hyalosis suspended in a formed vitreous gel and hence move very little with movements of the eye

54. INTRAOCULAR FOREIGN BODY

55. INTRAVITREAL CYSTICERSOSIS A Translucent milky white cyst with dense white spot within, floating within in the vitreous cavity

56. Subhyloid or preretinal haemorrhage. Diffuse reddish haze of vitreous cavity due to fresh vitreous haemorrhage LARGE VITROUS OPACITIES

57. A large clot of blood in fresh vitreous haemorrhage

58. Large clumps of tumour cells in vitreous in patient with retinoblastoma

59. Dirty yellow exudates in vitreous cavity in patient with endophthalmitis

60. Vitreous membrane in proliferative diabetic retinopathy.

61. FUNDUS LESIONS 2

62. RED COLOURED LESIONS

63. Feathery flame shaped hemorrhages in RNFL In the retinal periphery superficial retinal hemorrhages are not flame-shaped and look rounded . This is because in the periphery the nerve fibers are separated and form a loose network. SUPERFICIAL RETINAL HAEMORRHAGE

64. DEEP RETINAL HAEMORRHAGES Punctate or rounded hemorrhages, looking like ‘dots and blots’ are hemorrhages in deeper layers of the retina

65. MICROANEURYSMS Small round punctate microaneurysms in temporal macula. There are clusters of hard exudates around the microaneurysms On FFA microaneurysms appear as hyperfluorescent punctate lesions whereas the retinal hemorrhages block the underlying fluorescence (black arrows)

66. SUBRETINAL HAEMORRHAGE Subretinal hemorrhage adjacent to a choroidal neovascular membrane. As the hemorrhage is under the retina the retinal vessels can be seen traversing over it

67. PRE RETINAL HAEMORRHAGE Preretinal hemorrhages (arrow head) and multiple ‘Roth’s spots (arrows) in anemic retinopathy Boat shaped subhyaloid haemorrhage

68. SMALL WHITE FLUFFY LESIONS

69. COTTON WOOL EXUDATES A cluster of cotton-wool spots below the disk due to an inferior hemiretinal vein occlusion Multiple cotton-wool spots, retinal hemorrhages, disk edema in the posterior pole seen in hypertensive retinopathy

70. MYELINATED NERVE FIBRES They can rarely resemble nerve fiber layer infarcts in appearance but are usually larger, often extend from the disk and show striations of the nerve fibers. They can partially obscure blood vessels. They are not associated with other microvascular anomalies that are usually found with soft exudates. These lesions are seen from birth and are present throughout life. They are usually innocuous but when extensive can cause large field defects corresponding to their shape

71. RETINITIS A focus of active retinitis looks like a fluffy white lesion as the involved retina becomes thickened, elevated and edematous Patch of retinitis adjacent to an old scar suggests the possibility of toxoplasmosis.

72. Progressive cytomegalovirus retinitis with some retinal hemorrhages and vasculitis

73. Early choroidal granulomas appear as lesions that are located deeper in the retina or choroid, larger in size and whitish-yellow in color

74. Multiple, round white spots, that are regularly spaced, in a specific pattern due to recent laser application

75. MULTIPLE YELLOW LESIONS

76. HARD EXUDATE Shiny, yellow globular clumps of hard exudates along with retinal hemorrhages and microaneurysms in diabetic retinopathy

77. DRUSEN Deposits are larger near the fovea and later coalesce to form large clumps

78. FUNDUS FLAVIMACULATUS In fundus flavimaculatus the yellowish flecks are in small curvilinear shapes resembling commas or small fish occurring bilaterally and symmetrically in the posterior pole up to the midperiphery. Patches of RPE atrophy at the macula ( Stargardt’s dystrophy) maybe present

79. Densely packed multiple yellow white spots of fundus albipunctatus in a patient complaining of night blindness. The arterioles are of normal caliber

80. In retinitis punctata albescens the arterioles are attenuated and the fundus later shows progressive degeneration with pigmentary changes. Also visual field and ERG changes are present in these patients along with progressively failing night vision. in fundus albipunctatus the fundus features and the symptoms are stationary

81. CHOROIDAL METASTASIS Choroidal metastases seen as a yellow sharply demarcated elevated lesion. These deposits are initially flat but increase in size and prominence over time. They are typically deposited in and around the posterior pole

82. CHOROIDITIS Patches of active choroiditis seen as moderate sized yellow lesions with fuzzy margins. Several pigmented healed scars are also present

83. Sudden onset of several yellow round to oval lesions, along with significant vitritis and anterior uveitis is suggestive of a group of conditions called multifocal choroiditis with panuveitis /recurrent multifocal choroiditis Common in females in their thirties, it is bilateral although frequently asymmetrical. The lesions range from 50-1000 microns in size. Old pigmented lesions are commonly present alongside new ones . As the inflammation subsides, the lesions become more punched out and atrophic with varying degrees of pigmentation. The disease tends to be chronic with recurrent bouts of inflammation. Mild edema of disc, CNVM

84. Similar lesions seen in young myopic patients without evidence of vitreous inflammation may be a rare variant called punctate inner choroidopathy (PIC). The lesions are deeper and punched out. Multiple cream colored large ‘plaque like’ flat lesions, occurring bilaterally, in a young-middle-aged patient presenting with sudden onset of visual blurring or floaters and photopsia is likely to be lesions of acute posterior multifocal placoid pigment epitheliopathy (APMPPE)

85. PIC

86. APMPPE

87. Bilateral lesions of active APMPPE with multiple foci of plaque like epithelitis and hyperemic disk edema

88. Bilateral occurrence of small multiple round to oval hypopigmented lesions in slightly older women The cream colored lesions are located deep in the choroid and the larger choroidal vessels can be seen to traverse over them. They begin in the peripapillary area and disseminate outwards in the pattern of a spray of pellets from a shotgun. This appearance is enhanced by the fact that the shots tend to be oval with the long axis perpendicular to the optic nerve. one-fourth disk diameter in size. The arrangement of the lesions is often symmetrical in the two eyes. The margins can be well defined or slightly fuzzy but there is usually no associated pigmentation or changes in the overlying retina. As the lesions resolve they flatten out or even disappear but usually leave small scars BIRD SHOT CHORIORETINOPATHY

89. Birdshot chorioretinopathy

90. MEWDS

91. Acute, unilateral appearance of small multiple white dots, 100-200 microns in size seen in young women is likely to be part of multiple evanescent white-dot syndrome (MEWDS). The lesions are at the level of the RPE and appear at the posterior pole extending to the mid-periphery. They are more concentrated in the perifoveal region though they usually spare the fovea. Characteristically the macula shows a granular appearance due to the presence of small white dots. Blurring of the margins of the optic nerve head may be present an enlarged blind spot on visual field testing. The lesions resolve over a couple of months leaving fine RPE alterations and fairly good vision.

92. CHOROIDAL COLOBOMA A choroidal coloboma appears as a well defined oval defect in the choroid usually in the inferonasal fundus, that allows the white sclera to show. Atrophic retinal vessels can be seen traversing over the colobomatous area contrasted against the luminous sclera

93. DEGENERATIVE MYOPIA Geographical patches of chorioretinal atrophy exposing the underlying sclera in severe degenerative myopia.

94. Well circumscribed hypopigmented areas of chorioretinal atrophy in the mid-peripheral retina with posterior scalloped edges is typical of gyrate atrophy. There is associated pigmentation and the lesions gradually coalesce spreading anteriorly and posteriorly GYRATE ATROPHY

95. CHOROIDAL RUPTURE Multiple choroidal ruptures secondary to blunt trauma, seen as thin crescent-shaped scars, concentric to the disc temporally .

96. Photocoagulation scars appear as multiple, regularly arranged, circular scars scattered in a pattern in a part or all over the fundus sparing the posterior pole Multiple scars of variable size scattered randomly anywhere in the retina following multifocal choroiditis

97. White, cobweb like subretinal fibrosis secondary to long standing choroiditis

98. Acute retinal necrosis An infection caused by the varicella-zoster or herpes simplex virus which is often bilateral Peripheral infiltrates with well-defined borders – there is vitreous haze, and a few perivascular haemorrhages advanced disease reaching the posterior pole full-thickness retinal necrosis

99. Progressive outer retinal necrosis An infection caused by varicella-zoster virus which is often bilateral and predominantly affects patients with AIDS. Multifocal homogeneous yellow–white deep retinal infiltrates. The macula may be involved at an early stage Established/middle. The signs typically spread rapidly around the retina, with very extensive full-thickness necrosis Late. Scarring is plaque-like and characterized as ‘cracked mud’. Rhegmatogenous retinal detachment (RRD) is very common, as is optic atrophy.

100. Bilateral diffuse choroiditis resulting in retinal edema , serous retinal detachment and pigment epithelial detachment in Vogt- Koyanagi -Harada disease VKH

101. FFA showing multiple pinpoint leaks causing extensive areas of pooling of dye beneath the neurosensory retina and the pigment epithelium detachment

102. LARGE AREA OF GREYISH WHITE OPALSCENCE

103. CRAO Two patients that are mirror images of each other—one with central retinal artery occlusion with cilioretinal sparing and the other with cilioretinal artery occlusion

104. Emboli at the bifurcation of the retinal arteriole on the optic nerve head causing an inferior branch arteriolar occlusion. There is another embolus which has lodged further along the arteriole to lodge at a distal arteriolar bifurcation

105. PIGMENTED LESIONS

106. A welldefined , solitary gray-black flat lesion surrounded by a hypopigmented halo is likely to be a patch of congenital hypertrophy of the retinal pigment epithelium (CHRPE). The size of these lesions can vary from small to several disk diameters CHRPE Grouped pigmentation (bear tracks) are seen as clusters of small pigmented lesions arranged in patterns resembling animal tracks (‘bear tracks’)

107. Dark pigmented lesion on the optic nerve head (Melanocytoma) A choroidal nevus present inferotemporal to the disk,seen as an ill-defined, deeply located, pigmented lesion. The margins of the nevus are feathery

108. An area of subretinal hemorrhage can appear as a dark blackish-maroon lesion of the fundus mimicking pigmented lesions. The size can vary from small to large and can be quite elevated Dark subretinal blood with areas of white altered blood at the macula

109. A well defined area of blotchy pigmentation involving a sector or a large segment of the retina with well defined convex margins could also be an area of spontaneous resolution of retinal detachment . The margins are delineated by a border of granular pigmentation called demarcation lines or water marks’. The causative break is often found to be surrounded by relatively heavy pigmentation.

110. classic ‘bone corpuscle’ shaped pigmentation in r etinitis pigmentosa Attenuation of the retinal vessels along with a fine mottling of the RPE in the mid-peripheral retina but no discernible pigment clumping referred to as retinitis pigmentosa sine pigmento

111. Scattered white spots deep in the retina with arteriolar attenuation and an atrophic macula in retinitis punctata albescens

112. Pigment clumping seen in the posterior pole, bilaterally, with minimal pigment disturbance in the peripheral retina in inverse RP or pericentral retinitis pigmentosa. The arterioles are attenuated and the disk shows a waxy pallor. The features are present bilaterally

113. A diffuse dark red discoloration of the posterior pole where the normal choroidal vascular pattern due to a diffuse choroidal hemangioma

114. In Oguchi’s disease the fundi have a striking grayish pink metallic color. The retinal vessels including the finer vessels stand out contrasted against this background and it is difficult to differentiate the arteries from the veins. The choroidal vessels are less visible

115. Fundi of patient with Oguchi’s disease after staying in a dark room for one hour. The appearance has changed dramatically and the fundi look normal

116. HYPOPIGMENTED FUNDUS

117. A total lack of chorioretinal pigment in albinism resulting in a completely hypopigmented fundus. All levels of the choroidal vessels are visible. The fovea is indistinct.

118. Almost complete atrophy of the choroid with only small patches of intact choroid, seen in an elderly male is likely to be the end stages of choroideremia FFA highlights the small island of normal choroidal tissue remaining at the macula

119. End-stage retinopathy with extensive areas of both pigment clumping and depigmentation

120. Diffusely hypopigmented fundus (sunset glow) in a patient recovered from Harada’s disease

121. FUNDUS LESION 3 ABNORMALITIES WITH RETINAL VASCULATURE

122. Normally at an AV crossing the venule crosses the arteriole obliquely but with arteriolar sclerosis the venule is seen to be deflected in such a manner that it crosses the arteriole at right angles ( Salus sign) Compression of the vein (arrow) results in an apparent impediment to the flow of blood in the vein across the crossing resulting in ‘banking’ and swelling of the peripheral part of the vein (Gunn’s sign)

123. All caliber changes occurring at arteriovenous crossings are collectively referred to as ‘AV nicking’ seen here at multiple sites (arrows) Grade 4 hypertensive retinopathy with disc edema , retinal hemorrhages, soft exudates and vessel caliber changes

124. Dilated veins in papillitis seen along with disk edema and peripapillary soft exudates

125. Venous reduplication (S shaped looping or winding of vessels) is a sign of increasing retinal ischemia as seen in severe NPDR along with other signs such as soft exudates and retinal hemorrhages in multiple quadrants

126. Sheathing of a vessel with an adjacent patch of retinal hemorrhage due to active vasculitis A sheathed occluded vessel as a result of old vasculitis

127. Resolving vasculitis with decreasing perivascular infiltration, retinal hemorrhages and retinal edema

128. New vessels initially look like small red bulbs that grow to spread out as multiple radial vessels like a fan Radial arrangement of new vessels also called ‘sea fan’ neovascularization

129. Vasculitis in Eales’ disease is often associated with patches of chorioretinal atrophy

130. Frosted branch angiitis is a severe form of vasculitis that affects almost the entire vasculature. The profuse perivascular infiltration causes the blood vessels to look like frosted branches

131. Retinal edema, crystalline deposits and dilated ectatic vessels seen in the temporal parafoveal region suggestive of idiopathic macular telangiectasias (formally referred to as idiopathic juxtafoveal telangiectasias/parafoveal telangiectasias)

132. Extensive malformations of the retinal capillaries with subretinal and intraretinal exudates in Coats’ disease

133. Presence of a red vascular growth with a pair of markedly dilated feeder vessels, i.e. an arteriole and venule extending from the mass for a variable distance is highly suggestive of peripheral capillary hemangioma A vascular malformation that looks like a cluster of dark grapes (saccular aneurysms) filled with blood occupying one to two disk diameters in size is the unmistakable appearance of a retinal cavernous hemangioma

134. A pair of dilated and tortuous vessels emerging from a tumor is likely to be the efferent and afferent vessels of a peripheral capillary hemangioma Highly dilated and tortuous vessels, secondary to peripheral arteriovenous communications in Wyburn-Mason syndrome

135. A network of arborising or broom like vessels lying superficially on the disk or on the retinal surface is suggestive of neovascularisation Neovascularization is initially very subtle and visible only with high magnification

136. NVE form at the junction of ischemic and non-ischemic retina

137. Retinal edema, crystalline deposits and dilated ectatic vessels seen in the temporal parafoveal region suggestive of idiopathic macular telangiectasias (formally referred to as idiopathic juxtafoveal telangiectasias/parafoveal telangiectasias)

138. Extensive malformations of the retinal capillaries with subretinal and intraretinal exudates in Coats’ disease

139. Retinal macroaneurysm seen along the inferotemporal arteriole surrounded by retinal edema and deposition of hard exudates The macroaneurysm fills up with dye on FFA surrounded by a thin rim of blocked fluorescence due to hemorrhage

140. Avascular retina of a premature neonate where the vessels are still to reach the retinal periphery which looks pale Stage 1 retinopathy of prematurity: Abrupt cessation of retinal vascularization in a premature neonate creating a ‘demarcation line’ between the vascular and avascular retina

141. Stage 2 retinopathy of prematurity: The demarcation line acquires a three-dimensional element forming a well defined ‘ridge’ between the vascular and avascular retina Stage 3 retinopathy of prematurity: Small tufts of bright red blood vessels grow behind the ridge. Later they extend into the vitreous and proliferate as if trying to grow towards the lens. This is referred to as ‘extraretinal proliferation’

142. Stage 4 retinopathy of prematurity: A sub-total retinal involvement with macula spared Stage 5 retinopathy of prematurity: Total retinal detachment assuming a partial or closed funnel configuration. The detached retina and proliferative tissue in the vitreous give a white appearance to the pupil (leukocoria)

143. Plus disease is characterized by engorgement and tortuosity of the blood vessels in the retina. Additional findings include retinal hemorrhages, poorly dilating pupil, abnormal dilated vessels on the iris and hazy media

144. Bilateral presence of extensive hemorrhages in several layers of the retina along with tortuous and dilated veins and white centered Roth’s spots in anemic retinopathy

145. In patients with hyperviscosity syndromes the hemorrhages are dense and seen adjacent to the blood vessels White centered retinal hemorrhages called Roth’s spots, though thought to be characteristic of leukemic retinopathy can be found in many blood dyscrasias

146. Retinal hemorrhages and a partially absorbed premacular hemorrhage in a patient with Terson’s syndrome Multiple cotton-wool spots and ischemic white retinal patches seen in the central fundus in Putscher’s retinopathy

147. Large premacular hemorrhage as a result of Valsalva’s retinopathy Multiple retinal hemorrhages and retinal infarcts in HIV retinopathy

148. Central retinal vein occlusion is often associated with significant macular edema and deposits of hard exudates Sheathed retinal veins, macular scar and laser marks in an old central retinal vein occlusion

149. Dense retinal hemorrhages limited to one quadrant of the retina are suggestive of branch retinal vein occlusion Dense retinal hemorrhages involving the superior hemisphere due to hemicentral retinal vein occlusion

150. A microaneurysm and a retinal haemorrhage classified as mild NPDR without CSME Presence of microaneurysms, retinal hemorrhages, hard exudates in the posterior pole but not within 500 microns of the center of the fovea classified as moderate NPDR without CSME

151. Multiple retinal hemorrhages and soft exudates in all quadrants and venous reduplication (arrow) classified as Very Severe NPDR with CSME New vessels elsewhere less than half disk diameter and no vitreous hemorrhage classified as non-high risk PDR

152. Neovascularization of the optic disk occupying more than half the disk area classified as high risk PDR Neovascularization elsewhere with subhyaloid hemorrhage classified as high risk PDR

153. MACULA

154. Appearance of a normal macula which has a central dark area with a small circular yellow light reflex in the centre FFA of normal macula in which the central foveal avascular zone appears dark and is surrounded by a wreath of end capillaries. All larger vessels terminate before the FAZ

155. Glassy appearance of an edematous macula in a patient with small branch vein occlusion. There are several retinal hemorrhages and the edematous area is bordered by deposits of hard exudates Grayish looking thickened macula (except the central cherry red spot) due to ischemic macular edema in a patient with central retinal artery occlusion—the patient also had advanced glaucomatous cupping of the optic nerve

156. Multiple, small, ‘red colored’ cysts arranged in a rosette around the fovea is the characteristic appearance of cystoid macular edema

157. A star shaped radial arrangement of linear yellow hard exudates centered on the fovea called a ‘macular star’ seen in a patient with neuroretinitis A complete macular star along with retinal hemorrhages , soft exudates and disk edema in Grade 4 hypertensive retinopathy

158. A well circumscribed round to oval area of neurosensory elevation centered at the fovea in central serous chorioretinopathy (CSCR) An area of neurosensory detachment associated with a small optic disk pit (arrow) in the temporal edge of the disk

159. A small, well defined area of retinal elevation looking like an ‘orange blister’ is the characteristic appearance of a pigment epithelial detachment A larger pigment epithelium detachment with multiple drusen in age related macular degeneration

160. Thickened macula along with subretinal hemorrhage due to an underlying choroidal neovascular membrane Dirty gray subretinal membrane gives the macula a thickened appearance. In the absence of subretinal hemorrhage or exudates, a subtle membrane may not be noticed

161. Wrinkles (fine retinal folds) across the macula in a patient with a shallow retinal detachment Folds in the retina due to macular edema

162. A better defined ERM causing tortuosity of the blood vessels and ILM folds A contracting epiretinal membrane can cause the clivus to be drawn towards the center deepening the foveal depression. This causes the fovea to look darker red and when seen in contrast to the whitish membrane it can falsely look like a macular hole, actually a pseudohole

163. Bilaterally present delicate pleats arranged radially around the fovea of a young male are likely to be seen in X-linked juvenile retinoschisis

164. Fluid that seeps in through a full thickness macular hole appears as a cuff of subretinal fluid around the hole Subtle thinning at the fovea in a patient with a lamellar macular hole

165. Macular thinning can be mistaken for a macular hole

166. occlusion of a cilioretinal arteriole causing a ‘cherry red spot’ as the ischemic retina extends to the fovea Reddish perfused patch in the papillomacular area supplied by a cilioretinal artery in a patient with central retinal artery occlusion

167. Cherry red spot secondary to traumatic macular edema (Berlin’s edema ). The trauma causes diffuse gray white opacification of the retina and often involves large areas . This type of opacification is usually at a deeper level of the retina and is better defined and more glistening than the cloudy edema seen with vascular occlusion

168. Bulls eye maculopathy seen in a patient with advanced cone dystrophy. A pigmented area in the center is surrounded by a complete or incomplete parafoveal ring of depigmentation Bulls eye maculopathy secondary to chloroquine toxicity—early changes include irregularity (mild stippling or mottling) in the macular pigmentation and blunting of the foveal reflex. Later there is central irregular pigmentation surrounded by a concentric zone of hypopigmentation

169. Stargardt’s dystrophy associated with fundus flavimaculatus Early Stargardts disease

170. Patch of pigmentation just temporal to the fovea bilaterally, seen in late stages of Idiopathic macular telangiectasias (also called parafoveal or juxtafoveal telangiectasias)

171. Angioid streaks (breaks in the Bruch’s membrane) seenas dark red lines radiating from the optic disk in all directions that lie deep in the retina and resemble cracks

172. Myopic maculopathy—rounded, sharply demarcated,yellowish white areas with varying amounts of pigmentation seen in a myopic eye are focal areas of chorioretinal atrophy allowing the sclera to show through Criss-crossing yellow colored choroidal vessels at the macula due to choroidal sclerosis. A ‘ laquer crack’ seen as a fine linear reddish line is also present

173. Sharply demarcated, round to oval area of depigmentation, showing the underlying choroidal vessels is an area of geographic atrophy

174. A sharply demarcated hypopigmented spot as a result of exposure to ultraviolet rays called photic retinopathy

175. Areas of linear or serpentine shaped mottling at or around the macula in healed geographic helicoid peripapillary choroidopathy (GHPC) Polypoidal or serpentine shaped fuzzy, grayish yellow lesions at the macula in active macular GHPC (Geographic Helicoid Peripapillary Choroidopathy)

176. Scar consisting mainly of white retinal gliosis with no pigmentation Scar consisting of subretinal gliosis, pigmentation and chorioretinal anastamoses secondary to a choroidal neovascular membrane

177. Characteristic punched out macular scar of congenital toxoplasmosis Multiple linear curved scars concentric to the disk margin are a result of choroidal tears

178. Multiple yellow, pale or white punctate round deposits of varying size at the posterior pole ( drusen) Small (less than 63 microns) flat drusen with well defined margins are called hard drusen

179. Large drusen (greater than 125 microns) with less distinct, fuzzy margins that are elevated or dome shaped are called soft drusen . They tend to become confluent adopting various shapes as they coalesce Still larger confluent, soft drusen (500 microns) may have a pool of serous fluid around them and are referred to as drusenoid PEDs ( Drusenoid pigment epithelial defects)

180. Regressing drusen are whiter and refractile in appearance. Their margins become irregular and areas of calcification start to appear. Associated RPE atrophy becomes prominent. Drusen in dry age related macular degeneration in which they are associated with areas of hyperpigmentation and RPE changes

181. Subretinal dirty gray membranous lesion due to a choroidal neovascular membrane Often a CNVM is associated with adjacent subretinal hemorrhage

182. A partially scarred CNVM with a ring of subretinal hard exudates CNVM along with drusen in age related macular degeneration

183. CNVM seen adjacent to an old scars Myopic CNVM

184. Bilateral yellow colored cyst like lesion under the macula is the unmistakable appearance of vitelliform macular dystrophy

185. A subretinal cystic lesion that is translucent white in color along with a characteristic white dot within the lesion (scolex) is the characteristic appearance of a subretinal cysticercus

186. Bilateral glistening yellow crystals scattered in the retina along with atrophy of the RPE and choroid is suggestive of Bietti’s crystalline dystrophy

187. Drug related crystalline deposits in the paramacular region

188. Polyps of choroidal vasculature in idiopathic polypoidal choroidal vasculopathy (IPCV) can result in multiple areas of serous and hemorrhagic detachments of the RPE and the neurosensory retina in the posterior pole along with altered heme

189. PERIPHERAL RETINAL DEGENERATION

190. LATTICE DEGENERATION 8 % population, moderate myopes Linear, spindle/cigar shaped lesions parallel to the ora made up of yellow spots with white lines of sclerotic vessels crisscrossing are likely to be areas of typical lattice palisade degeneration or dystrophy. Radial or circumferential . Radial lie along the blood vessel, posteriorly – Stickler and Wagner syndrome

191. Pathology. There is discontinuity of the internal limiting membrane with variable atrophy of the underlying NSR. The vitreous overlying an area of lattice is synchytic but the vitreous attachments around the margins are exaggerated Complications Tears Atrophic holes

192. • Management. Asymptomatic areas of lattice are generally not treated prophylactically, even if retinal breaks are seen, unless particular risk factors are present, perhaps including RD in the fellow eye; Treatment of the fellow eye when extensive lattice (more than 6 clock hours) is present, or there is high myopia, or higher risk of detachment.

193. A pigmented lattice with prominent sclerosed vessels running across A radial lattices running along a blood vessels and relatively posterior in location

194. Multiple rows of lattices A lattice harboring a retinal hole

195. A large tear present at the edge of a pigmented lattice. Such tears can sometimes unzip the entire lattice resulting in a giant tear

196. Snail-track degeneration Snailtrack degeneration is characterized by sharply demarcated bands of tightly packed ‘snowflakes’ that give the peripheral retina a white frost-like appearance . The vitreous is usually liquefied and retinal holes are common.

197. Retinal tears are retinal breaks that occur secondary to retinal traction. The traction creates a ‘v’ shaped retinal flap which may be elevated in the presence of continuing traction. Sometimes the flap may tear off completely forming an operculum floating in the vitreous. Inferotemporal quadrant U-tears (horseshoe) consist of a flap, its apex pulled anteriorly by the vitreous, the base remaining attached to the retina RETINAL BREAK

198. Retinal holes : Retinal breaks secondary to trophic changes occur in the form of retinal holes. They have no tractional element in their pathogenesis. They are usually round in shape with smooth edges. There is no flap or operculum and little pigmentation. They can occur in isolation or within lattices. Retinal holes are found in 0.2-0.4% of adult eyes. They can be unilateral and have no predilection for any particular quadrant.

199. A retinal hole appears as a well demarcated, round, bright red colored lesion bordered by grayish, slightly raised margins The base appears bright red as the choroid shines through – as seen in this large hole i

200. Horseshoe retinal tear appears as a red colored ‘V’ shaped break in the retina Retinal hole with surrounding subretinal fluid

201. Difficult to see large horseshoe tear that can easily be missed on cursory examination. Such tears are better visualized by indentation .

202. GIANT RETINAL TEAR Retinal tears greater than 90 degrees circumference are called giant retinal tears. They occur near the ora and a thin piece of retina can be seen between the posterior edge of the break and the ora differentiating it from a dialysis. The flap of the tear hangs back and tends to roll inwards or scroll up and is prone to develop PVR changes.

203. Twenty percent of GRTs result from blunt trauma . The force of trauma is transmitted to the posterior margin of the vitreous base which may also get avulsed and hang like a thread or clothes line. Sometimes direct trauma to the temporal side of the globe may cause a giant tear.

204. A giant retinal tear with retinal detachment and a ‘rolled out’ edge

205. The edge of an avulsed vitreous base is seen hanging like a rope in the vitreous cavity

206. Disinsertion of the retina from the ora results in a retinal dialysis. In a retinal dialysis there is no tendency for the flap to roll up. Dialyses commonly occur after blunt trauma or are spontaneous. Traumatic dialyses most commonly occur in the superonasal and inferotemporal quadrants. RETINAL DIALYSIS

207. Spontaneous retinal dialyses are often asymptomatic and bilateral. They occur in young males in the second to third decade. There may be a genetic predisposition. The detachment associated with retinal dialyses are usually slowly progressive as the patients are usually young and the vitreous is well formed. The detachment shows signs of chronicity such as demarcation lines and retinal cysts. The patients are not symptomatic till the macula is involved which takes some time.

208. In a retinal dialysis the retina appears to be disinserted from the ora and there is no retinal tissue between the break and the ora

209. Rows of multiple small cysts arranged as a band running along the ora is likely to be peripheral microcystoid degeneration. These are the most common degenerations of the peripheral retina and are seen in all adult eyes. The cysts look like tiny, often red colored vesicles on a grayish white background, which makes the retina, look thickened and opaque

210. A cystic retinal tuft (CRT) Also known as a granular patch or retinal rosette congenital abnormality consisting of a small, round or oval, discrete elevated whitish lesion typically in the equatorial or peripheral retina, more commonly temporally ; there may be associated pigmentation at its base. It is comprised principally of glial tissue; strong vitreoretinal adhesion is commonly present and both small round holes and horseshoe tears can occur. though the risk of RD in a given eye with CRT is probably well under 1%.

211. They sometimes give a nodular appearance due to altered retinal and glial cells . Often the base of the lesion is whitish, although occasionally there may be some retinal pigment epithelial hypertrophy at the base. Vitreous strands are attached to the tip and avulsion of the tip by these strands can cause a retinal tear to occur

212. A ‘ white without pressure’ area appears as a well defined region of opalescent white retina in the periphery (like the retina does when indented) tears, occasionally develop along the posterior border of white without pressure White without pressure

213. Sharply demarcated, oval, white patches of chorioretinal atrophy called Paving stone degeneration scattered in the peripheral retina. Some of these lesions can coalesce forming larger ones with convex scalloped margins. As the retinal and choroidal layers are atrophic the underlying white sclera shines through with mild pigmentation of the margins. Pavement stone degeneration

214. Snowflake degeneration appears as tightly packed small white specks looking like snowflakes scattered in the peripheral retina SNOW FLAKE DEGENERATION

215. Degenerative retinoschisis (RS) is present in about 5% of the population over the age of 20 years hypermetropia. RS is believed to develop from microcystoid degeneration by a process of gradual coalescence of degenerative cavities , resulting in separation or splitting of the NSR into inner and outer layers , with severing of neurones and complete loss of visual function in the affected area. In typical retinoschisis the split occurs in the outer plexiform layer, and in the less common reticular retinoschisis at the level of the nerve fibre layer. Retinoschisis :

216. Early retinoschisis usually involves the extreme inferotemporal periphery of both fundi, appearing as an exaggeration of microcystoid degeneration with a smooth immobile dome-shaped elevation of the retina. The elevation is convex, smooth, thin and relatively immobile , unlike the opaque and corrugated appearance of a rhegmatogenous RD.

217. The thin inner leaf of the schisis cavity may be mistaken, on cursory examination, for an atrophic long-standing rhegmatogenous RD but demarcation lines and secondary cysts in the inner leaf are absent. The lesion may progress circumferentially until it has involved the entire periphery. The typical form usually remains anterior to the equator; the reticular type is more likely to spread posteriorly.

218. Gray dome like elevation of the retina in the peripheral retina commonly in the inferotemporal quadrant and bilateral is likely to be peripheral retinoschisis

219. Diffuse choroidal/ chorioretinal atrophy in myopia is characterized by diffuse or circumscribed choroidal depigmentation, commonly associated with thinning of the overlying retina occurs typically in the posterior pole and equatorial area of highly myopic eyes. Retinal holes developing in the atrophic retina may occasionally lead to RD. Because of lack of contrast, small holes may be very difficult to visualize. CHORIORETINAL ATROPHY

220. Pars plana cysts appear as small translucent cysts in the pars plana region PARS PLANA CYST

221. RETICULAR (HONEYCOMB) DEGENERATION: It is an age-related change consisting of a fine network of perivascular pigmentation that sometimes extends posterior to the equator