Review Csc ( Atypical CSC) AP.박영훈 / Ap.김규섭/ R2 유애리

Central Serous Chorioretinopathy(CSC) - Detachement of macula resulted from a leak at the level of RPE - Involving both Choroid & Retina - Accumulation of transparent fluid at the posterior pole of the fundus

Pathogenesis A. Choroidal hyperpermeability with congestion of the choriocapillaris along exudation of protein and fluid B. RPE pump decompensation(choroid 에서 retina 방향으로) occurs over time with the formation of a PED C. 결국, RPE defect가 생기고, subretina space 에 누출이 생긴다. D. 이것은 neurosensory retina elevation을 만들고 neurosensory retinal detachment 를 일으킨다.

Central Serous Chorioretinopathy(CSC) 1.Typical(classic) CSC: more common type young patients acute localized detachment of the retina mild to moderate loss of visual acuity one or a few focal leaks (FAG) 2. Chronic CSC: diffuse retinal pigment epitheliopathy -> shallow subretinal fluid 로 인한 widespread RPE alteration chronic corticosteroid usage 3. Bullous CSC : bullous retinal detachment at inf. after organ transplantation using corticosteroids Asian descent Atypical CSC Chronic -망막 색소 상피의 광범위한 변성을 동반한 형태 -ERD 가 여러군데에 만성, 재발성으로 생기는 경우 -pph->central fovea -> severe VA ↓ -bilateral 2) 수포 망막 박리를 동반한 형태 -CSC 중에서 여러 개의 장액 망막 박리가 융합되면서 급성으로 bullous RD -체위의 변화에 따른 망막 하액의 변위 -bilateral >unilateral -FAG: multiple PED & leaking & 위치변화

Demographics & Sx - predominantly male between 30-50 man vs women 8 or 9 : 1 - 생각보다 고령과 여성환자에서 흔히 발생함 (> 50 yrs old (50%) male vs female 2.6 to 1) Risk factor 1.Type A personality 2.Increased cortisol level -Cushing’s disease -organ transplantation and corticosteroid usage -pregnancy 3. 백인, 라틴계, 동양인 >> 흑인 4.Catecholamines

Symptoms first notice → minor blurring of vision (VA ranges from 20/20 to 20/200.) followed → metamorphopsia ( irregularities in the shape of retinal plane ) micropsia dyschromatopsia central scotoma loss of contrast sensitivity increasing hyperopia ( Ant. displacement of retinal plane) Dichromatopsia. :색맹 Hypermetropization :??

Examination/Clinical Findings Fundus BSCS :

FAG 1. typical angiographic findings - presence of one or several hyperfluorescent leaks in the level of the RPE 2. “smoke stack”: most common 3. “ Ink-blot” : less common 4. leakage point 1) 1-mm-wide ring-like zone immediately adjacent to the fovea 2) superonasal> inferonasal> superotemporal> inferotemporal 3) In some cases, leaking point cannot befound 5. Chronic CSC: atrophic RPE tract (mottled hyperfluorescence) Smoke-stack : early phase, small hyperF spot, dye가 RPE 빠져나옴. late venous phase, F이 subretinal space지나서 vertically ascend Ink-blot : early phase , small hyperF spot -> 점점 centrifugally하게 커짐. 리킹 포이트 (-) – 황반부 바깥쪽에 떨어져 누출점이 있는경우 누출 부위는 치유되고 장액성 박리가 흡수 도중에 있는경우 Idiopathic uvear dffusion syndrome

FAG

FAG 21초 사진으로 초기부터 여러군데의 과형광과 광범위한 망막 색소 상피 손상으로 인한 window defect 소견 관찰됩니다. 시간 지나면서 RPE defect 로 인한 과형광과 장액성 망막 박리로 인해 형광색소 차오르는것 확인할수 있습니다.

OCT RPE -OCT : serous PED under the neurosensory elevation -PED : Round or ovoid , size < ¼ DD , yellowish color 화살표 :PED OCT 상 serous PED

ICG Hyperpermeability of the choroid (best seen in the mid-phase) ICG -early phase : dilated choroidal vessels at post.pole -mid phase : multiple hyperF areas d/t choroidal hyperpermeability Hyperpermeability of the choroid (best seen in the mid-phase)

Differential diagnosis 1.female>male 2.Bilateral ERD 3.dizziness, 뇌막자극증상, 이명,탈모,백반 4.Graculoatous uveitis->AC cell(++) 5. Tx: steroid

Differential diagnosis Idiopathic uveal effusion synd. -normal, healthy individuals in middle age(male>>female) -Sx : loss of vision with a bullous exudative RD -IOP normal , AC cell (-~ rare) - 발생기전 -annular peripheral choroidal detachment -B scan

Differential diagnosis Idiopathic uveal effusion - cong. Anormaly of scleral & vortex v. ->inter.venous flow obs. Tx: Spontaneous reattachment may require weeks to months Buckle, evisceration !!! 혈관 밖으로 배출된 농도 진한 혈장단백은 공막을 통하여 확산되어 안구밖으로 배출되기도 하고, 공막 후부의 도출정맥을 통애 배출되기도 하는데, 공막이 두꺼워지고, vortex vein 이 제기능을 못하기 때문에, suprachoroid 에 혈장단백이 모여지게 되고, 이러면서 혈관을 더 압박하기떄문에 더 악화된다고 합니다.

Treatment Photocoagulation 대개 수개월내 자연호전 Only 5% : severe, permanent visual loss Photocoagulation 1) shorten the course of the disease 2) accelerating the resorption of the fluid 3) has no effect on final visual acuity 4) 3개월간 관찰  macular detachment가 지속되면 > 500µm from the center of the fovea : photocoagulation < 500µm from the center of the fovea : 6개월간 관찰 5) permanent visual loss from an untreated macular detachment in the fellow eye ICG angiographic-guided photodynamic therapy(PDT) - rationale : reduction of blood flow in hyperpermeable choriocapillaris