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Case Conference 서울성모병원 안과 Pf.이원기/R2최승용
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Clinical History M/49, PS Park, 26829343
C.C. Dec. VA(OU) o/s) 3 months ago P.I. M/49 pt. complaining dec. VA(OU) for 3 mo and metamorphopsia(OU) for 2mo. Diagnosed as CSC and treated by laser(x2), but he showed no improvement. Past History DM/HTN(-/+), 5yrs, PO medication Ocular trauma/op(-/-) Familial history of eye disease(-)
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Ocular Examination Fd VA 1.0/ 0.125 (n-c) IOP 9/8 mmHg by NCT
Conj. OU not injected Cornea OU clear AC OU deep & cell(-) Pupil OU round & nl .sized, LR(+/+) Lens OU mild cortical opacity Fd OD nl. disc c multiple focal SRF c RPE atrophy OS nl. disc c multiple focal SRF c RPE atrophy large bullous RD at inf. (cannot detect tear)
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Macular OCT(OU)
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B-scan (OS)
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Differential diagnosis
ERD > RRD Leopard retina in FAG, Annular peripheral choroidal detachment, Scleral thickening in B-scan Possibly elevated IOP No retinal tear and retinal folding Multifocal choroiditis Smooth detached retinal surface Accompanied by uveitis Fluid shifting depends on position Atypical CSC Exudative RD Focal leaking point in FAG Harada disease Other causes of ERD Granulomatous uveitis Hypertensive retinopathy Extraocular symptoms (Neurologic, Auditory, Dermatologic) ARMD Coat’s disease Post. Scleritis Choroidal tumor T-sign in B-scan, Ocular pain Vasculitis d/t rheumatic disease Idiopathic uveal effusion FEVR Nanophthalmos Review Previous FAG, ICG Review Previous Uveitis lab FAG(OS->OD)
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Laboratory finding CBC, BC : WNL Viral marker (-)
Toxoplasma (-), Syphilis (-) Chest PA : WNL ANA(-), Rheumatic factor(-)
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Review - 2m ago : VA 1.0/0.7
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Review – 2m ago : FAG
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Review - 1m ago : VA 1.0/0.6
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Review – 1m ago : FAG
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FAG(OS->OD)
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Cue lists A) Atypical CSC P) Large Bullous RD (unable to Tx by Laser)
Bullous RD at inf.(OS) No retinal tear, Smooth surface Middle-aged male Idiopathic disease Yellowish colored lesion, No ocular pain No chamber cell, vitreous cell B-scan : No scleral thickening OCT : Multiple PED & SRF FAG : Diffuse RPE atrophy, Focal leaking point, No disc leakage Continuous atrophic RPE lesions ICG : Choroidal vessel dilatation A) Atypical CSC P) Large Bullous RD (unable to Tx by Laser) ppV(OS) Harada disease R/O Post. scleritis R/O Typical sign of CSC
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Operation
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Progress note #1->#9 VA OS HM LP(+) Fundus OS Plan
Filled with gas Decreased gas level Increased SRF Plan pppV+Endolaser+SO inj.
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Second operation
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Progress note #18/#8 -> #25/#15
VA OS HM Fundus OS Filled with SO, well applied laser scar Position : Prone -> Free
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Review -Atypical CSC c ERD-
서울성모병원 안과 Pf.이원기/R2최승용
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CSC (Central Serous Chorioretinopathy)
Detachment of macula Accumulation of transparent fluid at post. Pole Leak from RPE level (Choroid -> Retina) Clinical features Male>Female, middle-age (30~50) Risk factor Type A personality, Stress, Increased cortisol/catecholamine
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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.
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Classification Typical (classic) CSC Atypical CSC older age
Acute localized SRF, One or a few focal leaks in FAG, mild to mod. loss of VA Atypical CSC older age Ex. Corticosteroid usage, After organ transplantation, etc. Chronic CSC (Recurrent) Diffuse retinal pigment epitheliopathy (Wide spread RPE alteration) Severe loss of VA, Bilateral Bullous CSC Bullous RD at inf. -> Fluid shifting Bilateral > Unilateral Multiple PED & leaking -> Fusion (in FAG)
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Bullous CSC Rare condition Yellow-white SRF in the post. Pole
Inf. Bullow RD extending up to sup. arcade in the supine position “Teardrop” RD Localized post. pole RD connected to a large inf. bullous RD Leakage point Can be hidden by SRF Leakage from PED : Larger than typical CSC, From the edges Leakage without PED
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Diagnosis of Bullous CSC
Misdiagnosis – Complex and Confusing Complicated during corticosteroid usage, After Organ transplantation, During pregnancy, Hemodialysis Spontaneous aggravation and resolution DDx RRD RD d/t other causes Harada disease, Posterior scleritis, Multifocal chorioretinitis, Choroidal tumor, Uveal effusion syndrome Some reports -> S.buckling, Steroid Tx.
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Differential diagnosis
ERD > RRD Leopard retina in FAG, Annular peripheral choroidal detachment, Scleral thickening in B-scan Possibly elevated IOP No retinal tear and retinal folding Multifocal choroiditis Smooth detached retinal surface Accompanied by uveitis Fluid shifting depends on position Atypical CSC Exudative RD Focal leaking point in FAG Harada disease Other causes of ERD Granulomatous uveitis Hypertensive retinopathy Extraocular symptoms (Neurologic, Auditory, Dermatologic) ARMD Coat’s disease Post. Scleritis Choroidal tumor T-sign in B-scan, Ocular pain Vasculitis d/t rheumatic disease Idiopathic uveal effusion FEVR Nanophthalmos
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Findings of Bullous CSC
Ophthalmoscopic / Angiographic findings Yellowish colored lesion (d/t Xantophyll) Confinement of yellow-white lesion to the outer retinal layers Absence of vitreous cells With single or multiple PEDs Shifting nature of the SRF with fluctuating VA Absence of retinal break Typical FAG findings of CSC
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Typical Angiographic findings
FAG findings Presence of one or several hyperfluorescent leaks in the level of the RPE “Smoke stack”(common), “Ink-blot” Leakage point 1mm wide ring-like zone immediately adjacent to the fovea Superonasal>Inferonasal>Superotemporal>Inferotemporal In some cases, leaking point cannot be found Chronic CSC : Mottled hyperfluorescence (Atrophic RPE tract) ICG findings Hyperpermeability of the choroid (mid-phase)
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Angiographic findings
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Treatment of CSC Spontaneous resolution 5%, Severe visual loss
대부분 수개월 내, 시력 20/25 이상 회복 5%, Severe visual loss Photocoagulation Shorten course of the disease (Acclerating resoption of the fluid) No effect of final VA Severe form, permanent VA loss를 일으킨 반대안 Photodynamic therapy reduction of blood flow in hyperpermeable choriocapillaris Anti-VEGF Decrease endothelial permeability by changing tight junctions of RPE and fenestration of choriocapillary
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Treatment of Bullous CSC
Spontaneous resolution 악화 요인 제거 : Steroid, Pregnancy 등 Fibrin in exudation captured in SRF Fibroblast ingrowth를 통해 Partial resolusion d/t subretinal fibrotic membrane Laser Treatment Efficacy has not been well extablished No significant difference (?) Resolution of the disease, Final visual outcome Surgery
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Surgical drainage for SRF
Drainage of SRF When Laser is unavailable d/t SRF Ext. drainage Need buckling Can be assisted by intraocular surgery (gas injection or ppV) Can avoid retinotomy, risk of intraocular surgery Complete transscleral drainage is very difficult d/t Fluid shifting Risk of subretinal, choroidal hemorrhage
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Surgical drainage for SRF
Int. drainage Need ppV ppV, FAE , int. SRFD, Endolaser Usage of PFCL Can be assisted by buckling Risk of RRD Re-opening of retinotomy site Fluid accumulation around the retinotomy site Silicone oil tamponade for prevention of RRD
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