coats disease. retinal vascular disorder

1. COATS DISEASE

2. Introduction
• Idiopathic
• Characterized by:
Telangiectatic aneurysmal retinal
vessels with sub-retinal exudation and fluid

3. History

4. Scottish ophthalmologist Coats
 Massive sub
retinal Exudation
 No significant
vascular
abnormalities
 Massive sub retinal
Exudation
 No significant
vascular abnormalities
 Internal
Hemorrhage
 Massive sub retinal
Exudation
 No significant
vascular abnormalities
 Internal
Hemorrhage
 Massive sub
retinal Exudation
 Frank retinal
arterioles and venous
malformation
 Massive sub
retinal Exudation
 Frank retinal
arterioles and venous
malformation
Group I Group II Group III

5. • Leber’s multiple miliary aneurysms—
only aneurysmal dilatations.(early form of
coats)
• Coats disease—multiple aneurysmal
dilatations, sub retinal exudation & intra
retinal haemorrahage.
• Von hipple’s disease -- Massive sub retinal
Exudation, Frank retinal arterioles and venous
malformation.

6. COATS DISEASE
EPIDEMIOLOGY
• M :F – 3 :1
• Age - 8-16yrs
• 2/3 rd of cases before 10 yrs
• No racial predilection

7. Presentation of Coats disease
Visual loss and strabismus White fundus reflex (leukocoria)

8. Presentation of disease
• Decrease Visual acuity
• Strabismus
• Leukocoria
• Tealangiectesia
• Intraretinal exudation
• Exudative RD
• Iris neovascularization

9. Ophthalmoscopic picture

10. Retinal telagectasia—
inferior & temporal
b/w equator and ora

11. •Retinal and subretinal
hard exudation
• Overlying vascular
dilatation and tortuosity

12. • Slow progresssion of exudation
•Exudative retinal detachment

13. •Light bulb aneurysm typical of coats disease

14. •Retro lental mass

15. FFA
•Hyper fluorescence of telangectasia in early phase
•Pooling and staining in late phases

16. Vascular malformations are highlighted

17. Histopathologic section
•marked neural retinal edema, dilated and aneurysmal vascular
channels .
•intra- and subneural retinal exudate.

18. Staging of Coats Disease
Am J Ophthalmol 2001;131:572–83

19. Ocular conditions that can
simulate
Juvenile Coats disease
 Retinoblastoma
 Retinal detachment
 Congenital cataract
 Norrie disease
 Persistent hyperplastic
primary vitreous
 Ocular toxocariasis
At any age
 Vasculitis
 Ocular toxoplasmosis
 Type 1 idiopathic
juxtafoveolar telangiectasis

20. Retinoblastoma
• Dilated vessels are
continuous with the large
vascular trunks that extend
into the tumor.
• CT and USG --pick up
calcium deposits
• FFA differentiates both.
Coats disease
• the dilated vessels do not
extend into the subretinal
mass

21. • B SCAN- Exudative , with an absence of the
calcifications.(COATS)
• CT -- characterize intraocular morphology,
quantify subretinal densities, detect calcium,
and identify vascularity within the subretinal
space-(RB)
• Examination of subretinal fluid- cholesterol
crystals and pigment-laden macrophages in
the absence of tumor cells(COATS)

22. Leber miliary aneurysms
Presents usually in early adult life
Temporal fusiform and
saccular vascular
dilatation
Hard exudates may
threaten macula
Photocoagulation may be
beneficial

23. Idiopathic juxtafoveolar retinal telangiectasia
Group 1
• Unilateral, telangiectasia
temporal to fovea
• May benefit from
photocoagulation
• Bilateral, symmetrical perifoveal
telangiectasia
• Occasionally may benefit from
photocoagulation
Group 2
Presents in middle or old age
• Good prognosis • Guarded prognosis
• Bilateral, severe, perifovea
telangiectasia and capillar
occlusion
• Photocoagulation not
beneficial
Group 3
• Poor prognosis

24. Treatment
The goal of treatment mainly is to close
telangiectesia so that further leakage will not
occur

25. Treatment
Stage I
• Documentation (CFP and FFA)
• Follow up conservatively
• Intervention (if sub-retinal fluid and
exudation develop)

26. Treatment
Stage II to IV:
• Laser photocoagulation
• Cryotherapy
• Intra vitreal anti VEGF
• Surgical Intervention

27. Ablative therapies
Laser Photocoagulation
• Less severe cases of
exudation
• With or without RD
• Vascular leakage
• Non perfusion
• NVE
Laser Photocoagulation
• Less severe cases of
exudation
• With or without RD
• Vascular leakage
• Non perfusion
• NVE
Cryotherapy
• Laser is ineffective
• Extensive sub-retinal
exudation
• RD
• Drain sub retinal
exudation

28. Intravitreal Anti VEGF

29. • Surgical Intervention
 Repair Traction
 Hemorrhage
 RRD
 End stage NVE
 Painful Blind eye

30. • Coats disease is a serious eye disease
• Repeated treatment are needed to stabilize
the affected eyes
• Lifelong and serial monitoring required.
• Careful distinction of coats disease from
retinoblastoma is important