1. Case presentation
AP.박영훈 / St. 이승범 / R2 이용은

2. 33/M C.C.) Decreased VA (OS) for 6 months
P.I.) 33세 남자 환자는 약 10년 전 좌안 Coats’ disease 진단 받고 당시 레이저 치료 시행 받았으며 0.2 정도의 시력 유지되었음.
약 6개월 전 좌안 시력 저하 발생하여 타 병원 방문하여 유리체 출혈 진단 받고 본원 내원함.
Past history)
DM/HBP(-/-)
Ocular op / trauma (+/-) : ‘00, ‘03 laser treatment(OS) x2
Eye drop(-)

3. Ocular exam VA IOP Ant. Segment : N-S Fundus exam OD 1.0 OS HM (n-c)
OD 16 mmHg
OS 12 mmHg at 3PM
Ant. Segment : N-S
Fundus exam
OD normal optic disc c flat post pole
OS vitreous hemorrhage

4. B-scan (OS)

5. Fundus photography

6. Impression & plan Impression Plan
Vitreous hemorrhage due to Coats’ disease (OS)
Plan
Phaco + ppV + IOL insertion (OS)

7. Op title : phaco+ppV+intravit. TRI inj
Op title : phaco+ppV+intravit.TRI inj.+tractional membrane removal+int.SRFD+endolaser+FAE+SO inj.(OS)
Op finding
Total RD c thick & old SRF, sub-retinal hemorrhage
Retinal tear at 4o/c , 5 o/c

8. Progress note <POD#1>
VA OS LP(+)
IOP OS 17 mmHg
Lens : A. aphakia
Fundus : total RD c fibrotic SRF at entire retina
Supine position, head elevation 30°
Eye drop : O-MOX x4, O-LON q2hrs, O-1AT x2 (OS)

9. Progress note <POD#1>
Macular lipid deposition with multiple retinal vascular abnormalities with intraretinal hemorrhage,
혈관이상 : Telangiectasia, Tortuosity, Aneurysmal dilation

10. Progress note <POD#6>
VA OS LP(+)
IOP OS 10 mmHg
Fundus OS Subretinal exudate

11. Review
Coats’ disease
AP.박영훈 / St.이승범 / R2 이용은

12. 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

13. Etiology Primary vascular etiology :
Marked thickening of endothelial basement membrane of the telangiectatic vessels
Deposition of PAS-positive material

14. 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

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

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

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

18. Fundus finding
Thick lipid accumulation in the macula Numerous vascular anomalies in the inf. & temporal area

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

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

21. Differential diagnosis
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

22. Ancillary testing
Sonography : Subretinal calcification & detecting extraocular extension(between Coats’ disease and retinoblastoma)
Exam of SRF : cholesterol crystal and pigment-laden macrophages in the absence of tumor cells

23. 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

24. 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

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

26. Treatment 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.

27. Reference Retina, 4th edition. Stephen J. Ryan
Combined intravitreal bevacizumab and triamcinolone injection in a child with Coats disease.
; J AAPOS Jun;12(3):