1. Review
Coats’ disease
AP.박영훈 / Ap.김규섭/ R2 유애리

2. Coats’ disease Definition Classification
Telangiectatic and aneurysmal retinal vessels with intraretinal and subretinal exudates
Classification
Group I : massive subretinal exudate and no vascular abnormalities
Group II : Massive subretinal exudate and multiple retinal vascular abnormalities with intraretinal hemorrhage
Group III : lacked the massive subretinal fluid -> Leber’s multiple miliary aneurysm disease
정의: 망막 모세혈관의 확장 및 혈관류의 발생으로 발생으로 인하여 망막내 및 망막하 공간에 삼출물이 축적되고 삼출 망막 박리를 일으키는 발육이상 망막 혈관질환

3. Etiology Primary vascular etiology :
Marked thickening of endothelial basement membrane of the telangiectatic vessels
Deposition of PAS-positive material
임신시 악화: 내분비 질환
감염 염증은 원인이 아님

4. Clinical manifestation
Affects males 3 times as often as females
Unilateral in 80~90% or more of cases
Age at diagnosis
as young as 4 months
2/3 of the juvenile cases present before 10 years of age
Painless
Childhood : poor vision, strabismus, or leukocoria
Adult : frequently associated with hypercholesterolemia
10세 이전: 사시 , 백색동공, 시력저하,

5. Clinical manifestation
Occurring concurrently with
Retinitis pigmentosa, Turner’s syndrome, Senior-Loken syndrome, diffuse CNS venous abnormality, etc.
No evidence to suggest genetic transmission

6. Fundus finding Localized, lipid-rich, yellow subretinal exudate
Vascular abnormalities
Sheathing
Telangiectasia
Tortuosity
Aneurysmal dilation
Zones of capillary drop-out
Neovascularization

7. Fundus finding
Macular lipid deposition Subretinal fibrosis and pigmentation Inferotemporal exudative retinal detachment

8. Fluorescein angiography
Telangiectasia and aneurysm
Capillary nonperfusion – microvascular involvement
Anomalous vascular communicating channels

9. Fluorescein angiography
Loss of blood-retinal barrier
Large telangiectatic vessels
Leaking aneurysms

10. Differential diagnosis
Retinoblastoma VS Coat’s Dz
Male
Lipid accumulation 더심함
Retinoblastoma
FAG: 종괴내의 혈관, 영양혈관 관찰
B-scan : subretinal calcification
Other conditions that cause leukocoria and strabismus
Retinoblastoma , RD, PHPV
Congenital cataract
Familial exudative vitreoretinopathy
At any age
Eales’ disease , vasculitis, collagen vascular disease
Tumor accompanied by exudation
Diabetic vasculopathies with lipid deposition
BRVO or ERM with secondary vascular leakage
Idiopathic juxtafoveal telangiectasis

11. Treatment The goal Indication
Preservation of improvement in visual acuity
Preservation of ocular integrity
Indication
Exudate is extensive and progressive
Exudate is threatening or involves the macula
Produces significant retinal detachment
목적:비정상적으로 누출되는 망막 혈관을 막아서 지방 삼출물의 흡수를 유도한다

12. Treatment In less severe cases Laser photocoagulation (TOC)
Leaking lesions are treated with moderate to large(100~500 microns) applications of moderate intensity light
Scatter photocoagulation : decrease the risk of later neovascularization
Complications : inflammation, choroidal detachment, progressive exudation, creation of virtreoretinal anastomosis, ERM formation, hemorrhage…
Intravitreal bevacizumab injections : significant reduction of macular edema and exudation

13. Treatment
Photocoagulation covers temporal aneurysms and is also placed in a scatter pattern in zones of nonperfusion

14. Treatment If lesions are too peripheral
- 삼출물이 두꺼워 레이저 에너지의
흡수가 부적절한 경우
- ERD-> SRF drainage 후
이상이 있는 혈관에 직접 시행
If lesions are too peripheral
Indirect ophthalmoscope-mounted laser
Transscleral laser
Cryotherapy
Drain SRF to obtain sufficient retinal vascular freezing
Twice-yearly follow-up is needed
Bullous RD: Intraocular infusion, drainage of SRF, and cyrotherapy
Use of vitrectomy and intraocular tamponade with gas of SO.