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

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(-)

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

B-scan (OS)

Fundus photography

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

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

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)

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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.

Reference Retina, 4th edition. Stephen J. Ryan Combined intravitreal bevacizumab and triamcinolone injection in a child with Coats disease. ; J AAPOS. 2008 Jun;12(3):309-11.