Retinal Artery Macro aneurysm, Primary Retinal Telangiectasia

1. Retinal Artery Macroaneurysm,
Primary Retinal Telangiectasia
By Dr Wasim Ullah Shah
Resident Ophthalmology Eye B ward
KTH Peshawar

2. Retina
• The retina is a thin,
semitransparent,
multilayered sheet of
neural tissue that lines
the inner aspect of the
posterior two-thirds of
the wall of the globe

3. Retinal Circulation
• Arterial System:
– Central Retinal Artery
• Enters the globe approx 1cm behind the
globe
– Intima – single layer of endothelium
– Media – smooth muscle
– Adventitia – loose connective tissue
– Arterioles
• Arise from CRA
• Walls contain smooth muscle

4. Arterial System contd.
• Capillaries
– Retinal capillaries (RC) supply inner 2/3rd of
retina
– Choriocapillaries  outer third
– Inner Plexus (capillary network)Ganglion
cell layer
– Outer Plexus Inner Nuclear layer
– RC lack smooth muscle and elastic tissue

6. Retinal Capillaries
• Wall consists of
– Endothelial cells: Inner Blood-Retinal Barrier
– The basement membrane
– Pericytes
• Its pseudopodial processes envelop the arteries
• Have contractile properties
• Thought to participate in autoregulation of
microvascular circulation

7. Venous system
• Small venules
– larger than capillaries
– Similar structure
• Large venules
– Contain smooth muscle
– Merge to form veins
• Veins
– Smooth muscle + elastic tissue
– distensible

8. Retinal Artery Macroaneurysm
Introduction
• Localized dilatation of retinal arterioles
• Predilectation for older hypertention (75%) women
• dyslipidemia is also associated
• 90% involve only one eye
Diagnosis
Symptoms :
 insidious impairment of vision due to leakage involving macula
 Sudden visual loss due to haemorrhage is less common

9. Retinal Artery Macroaneurysm Diagnosis Cont...
Fundus
 Saccular arteriolar dilatation is typical often at bifurcation or
arteriovenous crossing on a temporal vascular arcade.
 The aneurysm may enlarge to several times the diameter of the
vessel
 There is associated retinal haemorrhage in 50%
Course
 Chronic leakage
 Haemorrhage
 Spontanous involution
 Other complications like epiretinal membrane, choroidal
neovascularization

10. Retinal Artery Macroaneurysm Diagnosis Cont...
FA
 Uniform filling with macroaneurysm is typical with late leakage
 Incomplete filling is due to thrombosis
OCT
 Demonstrate the lesion itself
 Main role is to monitor macular oedema
 Or subhyaloid haemorrhage
Vascular Risk Factors
 Blood pressure
 Serum lipids

12. Treatment
Observation
 With good visual acuity
 If macula is not threatened
 With mild retinal haemorrhage
 In many cases macroaneurysms
will spontaneously involute following retinal or vitrous haemorrhage
Laser
 If oedema or exudates threaten or involve the fovea with
documented visual deterioration
 Burns may be applied to the lesion itself the surrounding area or
both
 It may take several months for oedema and exudate to fully absorb

13. Treatment cont ...
• Intravitreal bevacizumab 95% of macroaneurysm
with resolution of macular oedema
• Yag laser hyaloidectomy for persistent premacular
haemorrhage in order to disperse the blood into
vitrous cavity
• Intravitreal gas injection in face down position may
shift the sub retinal haemorrahge away from the
macula
• Vitrectomy for persistent vitrous haemorrhage

15. Primary Retinal Telangiectasia
Intrduction
 comprises a group of rare, idiopathic, congenital or
acquired retinal vascular anomalies characterized by
dilatation and tortuosity of retinal blood vessels,
multiple aneurysms, vascular leakage and the
deposition of hard exudates.
 it involves mainly capillary bed although the
arterioles and venules may also be involved

16. Types
Ideopathic Macular Telangiectasia
 Type 1 Aneurysmal Telangiectasia
 Type 2 perifoveal Telangiectasia
 Occlusive Telangiectasia
Coats Disease
Eales Disease

17. Ideopathic Macular Telangiectasia
• Condition of unknown pathogenesis
• Family history is present in small proportion of cases
Type 1 Aneurysmal Telangiectasia
 May be closely related to coats disease
 Involves only one eye
 Both the peripheral retina and macula can be effected
 Patients are typically of middle aged males
Symptoms
 Mild to moderate blurring of vision in one eye

18. Ideopathic Macular Telangiectasia cont...
Signs
 Early signs more readily detected on red free photographs
 Larger aneurysms form as the condition progress
 Macular oedema including cystoid changes
 Chronic leakage and lipid deposition
OCT
 Demonstrates retinal thickening, CMO and localized exudative
retinal detachement
FA
 Shows telangiectasia and multiple capillary, venular and
arteriolar aneurysms with late leakage and CMO
Treatment
 Treatment is with laser to points and areas of leakage and
with
 Intravitreal VEGF inhibitors

20. Type 2 Perifoveal Telangiectasia
 Bilateral form
 Worse visual prognosis
 Males and females equally affected
 Onset in middle age
 Findings are limited to perifoveal area
 Degeneration of muller cells is thought to be an important
pathogenic mechanism
Symptoms
 Blurring in one or both eyes
 Distortion may be a feature

21. Type 2 Perifoveal Telangiectasia cont...
Signs
 Greyish loss of parafoveal retinal transparency extending up
to one disc diameter from the foveola.
 Fine superficial crystalline retinal deposits may be seen
 Parafoveal telangiectasia can be seen more readily with red
free photography.
 Right angled venules are characteristics
 Foveal atrophy may simulate a lamellar hole
 Small RPE plaques develop in many patients
 Aneurysms are uncommon but have been reported
 Visual acuity generally does not deteriorate to less than 6/60

22. Type 2 Perifoveal Telangiectasia cont...
OCT
 Formation of hyporeflective inner retinal spaces of variable
size
 Inner lamellar cyst that enlarges with progressive disease is
commonly seen underlying the fovea
 thinning and disruption of photoreceptor layer is also very
common
 Pigment clumps are seen as intraretinal hyperreflective
plaques with posterior shadowing
 Foveal thinning is common

23. Type 2 Perifoveal Telangiectasia cont...
FAF
 Central foveal hyperautoflourescence is a common early
finding
 In more advance disease an area of well demarcated central
hypoautoflourescence develops
 Retinal crystals and pigment clumping give
hypoautoflourescence
FA
 Shows bilateral perifoveal telangiectasia
 It is also used to confirm CNV

24. Type 2 Perifoveal Telangiectasia cont...
Macular pigment optical density(MPOD)
 Imaging shows a possibly pathognomonic pattern of oval
reduction in density
Treatment
 Intravitreal Anti VEGF agents decrease leakage but are not
helpful visually

26. Occlusive Telangiectasia
 Extremely rare condition
 Presents in late midddle age
 Poor visual prognosis
 It relates to capillary occlusion rather than telangiectasia
 Progressive occlusion of parafoveal capillaries with marked
aneurysmal dilatation of terminal capillaries

27. Coats Disease
Introduction
 Ideopathic retinal telangiectasia
 Onset in early childhood
 Associated with intraretinal and sub retinal exudation and
frequently exudative retinal detachement without signs of
vitroretinal traction
 75% of patients are males
 95% have involvement of only one eye
 Genetic predisposition may be involved as somatic mutation in NDP
gene in some patients
 Leber miliary aneurysm is milder form of the same disease with
more localized pattern and carrying a better visuall prognosis

28. Coats Disease cont...
Diagnosis
Symptoms :
 Unilateral visual loss
 Strabismus
 Leukocoria
Fundus
 Telangiectasia and fusiform focal aneurysmal arteriolar
dilatation
 Intra and sub retinal exudates progression to extensive
exudative retinal detachment

29. Coats Disease cont...
Complications
 Rubiosis iridis
 Glaucoma
 Uveitis
 Cataract
 Phthisis bulbi
FA
 Mild cases shows early hyperflourescenece of telangiectasia and
aneurysmal dilatations and late staining and leakage
OCT
 May be helpful in assessment of the macula in cooperative older
children

31. Treatment
Observation
 In patients with mild, non vision threatning disease
 And in total retinal detachment for whom there is no potential for restoration of useful
vision
Laser ablation
 Points of leakage should be considered
 Multiple repeated treatments over an extended term are commonly required
Anti VEGF
 Therapy should be used as an adjunct to laser
Intravitreal Triamcinolone
Cryotherapy
Vitroretinal surgery
Enucleation
 May be required in painful eyes with neovascular glaucoma

33. Eales Disease
Introduction
 Ideopathic occlusive peripheral periphlebitis
 Rare in caucasians but important cause of visual morbidity in
young males from india
 Characterized by three stages: inflamatory, occlusive and
retinal neovascular
 Diagnosed principally by clinical examination
 Visual prognosis is good in majority of cases
 Tubercular protein hypersensitivity may be important in the
aetiology

34. Eales Disease cont...
Diagnosis
Symptoms:
 Floaters or sudden visual reduction due to vitrous
haemorrhage
Signs
 Systemic neurological features have been reported
 Mild anterior uveitis
Fundus
 Typically bilateral though often asymmetrical
 Peripheral periphlebitis, sheathing superficial retinal
haemorrhages and sometimes cotton wool spots
 Pigment chorioretinal scars may be seen
 Branch retinal vein occlusion
 Peripheral capillary non perfussion, microaneurysms,
tortousity, vascular shunts and neovascularization with
recurrent vitrous haemorrhage
 Macular oedema can develop

35. Eales Disease cont...
Complications
 Tractional retinal detachment
 Macular epiretinal membrane
 Neovascular glaucoma
 Cataract
FA
 Identify vasculitis and areas of non perfusion
Investigations
 Should be performed to rule out other csuses of vasculitis e.g
sarcoidosis, tuberculosis and peripheral retinal
neovascularization (e.g. Haemoglobinopathies)

36. Eales Disease cont...
Treatment
 Steroids
 Antitubercular treatment
 Scatter photocoagulation or cryotherapy
 Intravitreal VEGF inhibitors
 Vitrectomy : for persistent vitrous haemorrhage, tractional
detachment and macular epiretinal membrane

38. Thank you