This document discusses various retinal vascular diseases and associated findings. It covers central retinal vein occlusion and the associated findings except for neovascular glaucoma. It notes that the most common cause of neovascular glaucoma is ischemic central retinal vein occlusion. The document also discusses ophthalmic artery occlusion findings compared to central retinal artery occlusion. Additional topics covered include hypertensive retinopathy, sickle cell retinopathy, Coats disease, retinal artery macroaneurysms, and other retinal conditions like radiation retinopathy. Treatment options are provided for several of the conditions.

1. RETINAL VASCULAR DISEASES - II
By
Ahmed Alsherbeny
MD, EBO, CABOphth, MRCSEd Ophth, FRCS Ophth (Glasg), FICO, MSc

2. All the following may be associated findings after central retinal
vein occlusion, except:
A. Optic disc edema.
B. Optic disc venous–venous collateral (optociliary shunt) vessels.
C. Diffuse macular edema.
D. Neovascular glaucoma.
E. “Box-car” bloodstream in arterioles

3. Most common cause of neovascular glaucoma is :
A. Ischemic central retinal vein occlusion (CRVO).
B. Central retinal artery occlusion (CRAO).
C. Long-standing retinal detachment.
D. Uveitis.
E. Diabetes mellitus

4. Ophthalmic artery occlusion, rather than central retinal artery
occlusion is suggested by all the following findings, except:
A. Severe visual loss (bare to no light perception).
B. Marked choroidal perfusion defects on fluorescein angiography.
C. Intense ischemic retinal whitening that extends beyond the
macular area.
D. Few or no cherry-red spots.
E. Mildly decreased amplitude of the electroretinogram.

5. Hypertensive Retinopathy

6. Ocular associations and complications of hypertension:
RVO
RAO
RAM
AION
NP
Progression of DR
Posterior segment effects can be observed in the retina, choroid and
optic nerve

7. RETINOPATHY

8. • Grade 1: Mild generalized
retinal arteriolar narrowing

9. • Grade 2: Grade 1 +
arteriovenous nipping ±
‘copper wiring’ opacified
appearance of arteriolar walls

11. • Grade 3: Grade 2 + retinal
haemorrhages, exudates
‘macular star’ and cotton-wool
spots.

12. • Grade 4 ‘Malignant Hypertension’:
Grade 3 + optic disc swelling, which is a marker of ‘malignant’
hypertension Hypertensive emergency

13. CHOROIDOPATHY

14. • Siegrist streaks are flecks
arranged linearly along
choroidal vessels and are
indicative of fibrinoid necrosis
associated with malignant
hypertension

15. • Elschnig spots are focal choroidal
infarcts seen as small black spots
surrounded by yellow haloes
• Exudative retinal detachment,
sometimes bilateral, may occur in
acute severe hypertension such as
that associated with toxaemia of
pregnancy.

16.  When UNILATERAL, suspect carotid artery
obstruction on the side of the normal appearing
eye, sparing the retina from the effects of the
HTN

17. WORK-UP
 History:
• HTN, diabetes, or adnexal radiation?
 Complete ocular examination
 Check blood pressure
 Refer patient Medical internist
Emergency department
• Diastolic blood pressure of
≥110 mm hg
• Presence of chest pain
• Difficulty breathing
• Headache
• Change in mental status
• Blurred vision with optic disc
swelling

18. Sickle-cell Retinopathy

19. Sickling haemoglobinopathies are caused by one or more abnormal
haemoglobins which induce the red blood cell to adopt an anomalous
shape
1 SS (sickle-cell disease, sickle-cell anaemia) affects 0.4% of black Americans. The
disease is characterized by severe chronic haemolytic anaemia. Despite the severity of
systemic manifestations ocular complications are usually mild and asymptomatic.
2 AS (sickle-cell trait) is present in about 10% of black Americans. It is the mildest
form and usually requires severe hypoxia or other abnormal conditions to produce
sickling.
3 SC (sickle-cell C disease) is present in 0.2% of black Americans. It is
characterized by haemolytic anaemia and infarctive crises that are less severe than in
SS disease but may be associated with severe retinopathy.
4 SThal (sickle-cell thalassaemia) is characterized by mild anaemia but may be
associated with severe retinopathy.

20. SIGNS
 Anterior Segment:
• Conjunctiva: Dark red corkscrew- or comma-shaped vessels
• Iris: Patches of ischaemic atrophy
• Hyphaema may be spontaneous or follow minor trauma. Careful IOP
control (avoiding CAI) is critical

21. SIGNS
 Non- proliferative retinopathy:
• Venous changes. Tortuosity is very common and is thought to be due to peripheral arteriovenous shunting.
• Arteriolar changes. Occlusions can involve branch, central or macular vessels. ‘Silver wiring’ of arterioles in
the peripheral retina signifies previously occluded vessels. Corkscrewing of peripheral vessels may be seen.
• Optic disc ‘sign of sickling’. Dark red blots on the disc surface due to small vessel occlusion.
• ‘Salmon patches’. Orange–red mid-peripheral superficial intraretinal haemorrhages that may break through
to become preretinal or subretinal. The initiating event is thought to be a vascular occlusion.
• Black sunbursts. Patches of peripheral RPE hyperplasia and chorioretinal atrophy that evolve from some
salmon patches. The extent and morphology of pigmentation is variable, but an outer pale band is generally
present.
• Macular (retinal) depression sign. An oval depression in the temporal macular retina due to retinal thinning
following arteriolar occlusion, with irregularity of the light reflex.
• Peripheral areas of whitening or darkening.
• Angioid streaks occur in up to 6%.

24. SIGNS
 Proliferative retinopathy:
Goldberg staging of proliferative retinopathy
Stage 1 Peripheral arteriolar occlusions
Stage 2 AV anastomoses
Stage 3 Neovascular proliferation (‘sea- fans’)
Stage 4 Vitreous haemorrhage
Stage 5 Retinal detachment

25. Stage 1

26. Stage 2

27. Stage 3

28. Stage 4

29. Stage 5

30. TREATMENT
 Management of hyphema ‘High risk of optic nerve damage compared to
normal person’ IOP >24 mmHg, >24 hours AC wash
 There are no well-established indications or guidelines for retinopathy
treatment
 No treatment required for small peripheral lesions, as high probability of
spontaneous regression following autoinfarction

31. TREATMENT
 Scatter laser photocoagulation:
• Severe visual loss from the disease in the fellow eye
• Rapid growth of large elevated sea-fans with spontaneous haemorrhage
 Vitrectomy:
• Vitreous hemorrhage
• Retinal detatchment
Results are generally disappointing
Scleral buckling is not preferred to prevent anterior segment ischemia

32. TREATMENT
 Anti- VEGF therapy:
• May have a role, although evidence to date is limited
• Caution should be used in cases with significant traction

33. FOLLOW-UP
 No retinopathy: Annual dilated fundus examinations.
 Retinopathy present: Repeat dilated fundus examination every 3 to 6
months, depending on severity.

34. Retinal Artery Macroaneurysm

35. A retinal artery macroaneurysm is a localized
dilatation of a retinal arteriole and has a
predilection for older hypertensive women.
Symptoms
• Often asymptomatic
• Decreased vision
• Sudden loss of vision
• Unilateral 90%

36.  Focal dilatation of retinal arteriole
 Usually 100– 250 microns in size
 Within first three orders of the arterial
tree
 Exudation ± circinate exudates
 Consider the diagnosis in any presentation
of ‘hourglass’ haemorrhage, i.e.
simultaneous preretinal and subretinal
haemorrhage
SIGNS

37. SIGNS
Hourglass haemorrhage Exudation

38. WORK-UP
 History:
• Systemic disease, particularly HTN or diabetes
 Complete ocular examination:
• Look for concurrent retinal venous obstruction (present in 1/3 of cases) and
signs of hypertensive retinopathy
 FFA:
• Immediate complete filling (partial filling suggests thrombosis) with late
leakage
 OCT
• Helpful in demonstrating and following any ME that may be present

39. FFA
Photograph and fluorescein
angiogram showing MA with
haemorrhage at the subretinal
and prehyaloid levels
˃ MA at the bifurcation of a second-
order artery of the superior
temporal arcade
Fluorescence lamp in the dark

40. OCT
Hemorrhagic retinal arterial macroaneurysm
(RAM) visible on the inferior temporal branch,
surrounded by a dense subretinal hemorrhage,
and responsible for retrohyaloidal hematoma
and serohematic fluid
Horizontal OCT passing through the
macula. Retrohyaloidal
hyperreflectivity, dense in the lower
area, with visible serohematic fluid

41. CAUSES OF MULTILEVEL HAEMORRHAGES
(SUBRETINAL, RETINAL, SUBHYALOID, VITREOUS)
Retinal Artery Macroaneurysm
Hemorrhagic CNV
Trauma
Choroidal Melanoma
Valsalva retinopathy
Idiopathic polypoidal choroidal vasculopathy
Terson syndrome

42. TREATMENT
 Observation is indicated in eyes with good VA in which the macula is not threatened and in
those with mild retinal haemorrhage without significant oedema. In many cases
macroaneurysms will spontaneously involute
 Laser treatment may be considered if oedema or exudates threaten or involve the fovea,
particularly if there is documented visual deterioration.
 Intravitreal bevacizumab closed 95% of macroaneurysms in a case series, with resolution of
macular oedema.
 Nd:YAG laser hyaloidotomy may be considered for a persistent premacular haemorrhage in
order to disperse the blood into the vitreous cavity (Fig. 13.55C and D), from where it may be
absorbed more quickly.

43. TREATMENT

44. TREATMENT
 Intravitreal gas injection with face-down positioning may shift subretinal haemorrhage
away from the macula. Adjunctive intravitreal recombinant tissue plasminogen activator
(rtPA) may be used.
 Vitrectomy may be necessary for persistent vitreous haemorrhage.

45. FOLLOW-UP
 Based on the amount and location of exudate and hemorrhage

46. Coats Disease

47. • Idiopathic retinal telangiectasia
• ♂:♀ 3:1
• Young, although up to 1/3 may be asymptomatic until their 30s
• Unilateral, 10% of cases are bilateral
• No family history

48. Symptoms
• Asymptomatic
• V/A
• Strabismus
• Leucocoria

49. Signs
 Telangiectasia and fusiform focal aneurysmal arteriolar
dilatations:
• ‘Light bulb’ aneurysms
• Often in the inferior and temporal quadrant
 Intra- and subretinal exudates:
• Remote from the vascular abnormalities
• Progression to extensive exudative retinal detachment

50. ‘LIGHT BULB’ ANEURYSMS

51. INTRA- AND SUBRETINAL EXUDATES

52. COATS DISEASE - LEUCOCORIA

53. EXTENSIVE EXUDATIVE RD

54. Investigations
 FFA:
 Early hyperfluorescence of
telangiectasis and aneurysmal
dilatations
 Late staining and leakage
 Ultrasound: Why ?!

55. DIFFERENTIAL DIAGNOSIS OF LEUKOCORIA
PREDICT
Persistent hyperplastic primary vitreous
Retinoblastoma / Retinopthy of prematurity
Endophthalmitis
Dysplasia of the retina
Inflammatory cyclitic membrane
Congenital cataract / Coat’s Disease
Toxocaiasis

56. TREATMENT
 Observation in patients with mild, non-vision threatening disease and in those with a
comfortable eye with total retinal detachment for whom there is no potential for
restoration of useful vision.
 Laser ablation of points of leakage should be considered if progressive exudation is
documented
 Anti-VEGF therapy. Limited studies of anti-VEGF therapy have been carried out, but
initial results are promising.
 Cryotherapy
 Vitrectomy may be considered in eyes with significant tractional preretinal fibrosis or
total exudative detachment.
 Enucleation may be required in painful eyes with neovascular glaucoma.

57. Other Retinopathies

58. Radiation retinopathy
○ Capillary occlusion with the
development of telangiectasis and
microaneurysms.
○ Retinal oedema, exudate, cotton-
wool spots and haemorrhages .
○ The changes are best seen on FA.
○ Proliferative retinopathy.
○ Papillopathy. Radiation optic
neuropathy
may occur but is less common as the
nerve seems to be less sensitive than
retinal vessels.

59. Purtscher retinopathy
Purtscher retinopathy is
caused by microvascular
damage with occlusion and
ischaemia associated with
severe trauma, especially to
the head and in chest
compressive injury

60. Valsalva retinopathy
The Valsalva manoeuvre consists of
forcible exhalation against a closed
glottis, thereby creating a sudden
increase in intrathoracic and intra-
abdominal pressure (e.g. weight-lifting,
blowing up balloons).
The associated sudden rise in venous
pressure may rupture perifoveal
capillaries leading to premacular
haemorrhage of varying severity

61. Retinopathy in Blood Disorders

62. Anaemia
• Retinopathy:
○ Retinal venous tortuosity is related to the severity of anaemia but may occur in
isolation.
○ Dot, blot and flame haemorrhages, cotton-wool spots and Roth spots are more
common with co-existing thrombocytopenia.
• Optic neuropathy may occur in pernicious anaemia.

63. Leukaemia
○ Retinal haemorrhages and cotton-wool spots are
common.
○ Roth spots are retinal haemorrhages with white
centres
○ Peripheral retinal neovascularization is an
occasional feature of chronic myeloid leukaemia
(A).
○ Retinal and choroidal infiltrates may occur, most
commonly posterior to the equator (B) and may
masquerade as posterior uveitis. In chronic
leukaemia they may give rise to a ‘leopard skin’
appearance (C).
○ Scattered haemorrhages may occur in patients
with thrombocytopenia (D).
○ Optic nerve infiltration may cause swelling and
visual loss. (DD of CNS Infiltration Vs Local)

64. Hyperviscosity
The hyperviscosity states are a
diverse group of rare disorders
characterized by increased blood
viscosity due to polycythaemia
or abnormal plasma proteins.
• Ocular features include
retinal haemorrhages and
venous changes and occasionally
RVO and conjunctival
telangiectasia.

65. Thank You