ReTINA conference 서울성모병원 안센터 R2 김은영

Central scotoma “오른 눈에 동전크기 검은 점 보여요.” - Onset : 1 wk ago F/24 26388726 권 O 비 Chief Complaint Central scotoma “오른 눈에 동전크기 검은 점 보여요.” - Onset : 1 wk ago Present Illness 상기 24세 여환, 기저질환 없는자로 1주전부터 우안 중심부 동전 크기의 암점 보여 개인 안과 의원 방문, 큰병원 권유 받고 본원 의뢰됨.

History Past History Surgical History (-) Medication History (-) DM/HTN/TBc/HBV/CRD(-/-/-/-) : 최근 1달전 방광염 치료 받음 Allergic History (-) Surgical History (-) Medication History (-) Ocular trauma Hx(-) Eye drop use (-) Gls(+) for 10 yrs

Review of System General Fever (-) Chilling (-) Sweating (-) Headache (-) Dizziness (-) Weight loss (-) Poor oral intake (-) Weakness (-) Decreased urination (-) Cardiovascular Cyanosis (-) Chest pain (-) Respiratory Cough (-) Sputum (-) Rhinorrhea (-) Dyspnea (-) Chest wall retraction (-) Sore throat (-) Aspiration history (-) Gastrointestinal Nausea (-) Vomiting (-) Diarrhea (-) Constipation(-) Abdominal pain (-) Genitourinary Hematuria (-) Dysuria (-) Frequency (-) Skin Skin eruption (-)

Ocular Examination VA OD 0.16(1.0 x -3.75Ds) OS 0.25(1.0 x -4.00 -0.50 155) MR OD -3.75 -0.75 35 OS -4.50 -0.75 5 IOP(11:00, AP) OD 12 mmHg / OS 13 mmHg EOM straight at primary position/OU Orbit & adnexa no exophthalmos/OU Conj. not injected/OU Cornea & sclerae clear/OU Ant. Chamber deep & cell(-)/OU Iris & pupil round, nl. Sized, LR(+), NVI(-)/OU Lens clear/OU myopic

Fundus photography OD nl optic disc flat post pole, pigment stippling c foveolar yellowish hypopigmented lesion above macula pigment stippling associated with a foveolar yellowish hypopigmented lesion. 1m f/u에서는 stain 줄었음.

Macular OCT Disrupted RPE Infl. Deposit at RPE level

Initial plan Impression Diagnostic plan R/O ARPE(OD) - Acute Retinal Pigment Epithelitis R/O MEWDS(OD) - Multiple Evanescent White Dot Syndrome R/O PIC(OD) - Punctate inner choroidopathy Diagnostic plan FAG(OD  OS) VF 24-2 F/U 2 days later Initial plan Difficult to DDx. <White dot synd.> -> exclusion diagnosis MEWS - u/l, multiple, hypofluorescent lesions in ICG PIC – b/l, may represent an autoimmune type of uveitis The dot appears as a small granuloma which is composed of lymphocytes and macrophages.

VF24-2

FAG & ICG(OD) – early phase FAG transmission hyperfluorescence(WD) At the midphase of ICGA, a foveolar patchy hyperfluorescence, which slowly faded, was observed. On ICGA midframe to late frame, a central halolike hyperfluorescence, giving a cockadelike appearance to the macular area, was seen.

FAG & ICG(OD) – late phase FAG transmission hyperfluorescence(WD) Generally FAG : mild stain(OD) / ICG : no NV At the midphase of ICGA, a foveolar patchy hyperfluorescence, which slowly faded, was observed. On ICGA midframe to late frame, a central halolike hyperfluorescence, giving a cockadelike appearance to the macular area, was seen.

Follow-up plan Diagnosis R/O ARPE(OD) Therapeutic plan 1. F/U 1 month later 2. Patient education : good prognosis, self-limiting Visit ASAP if sudden VA change occurs Follow-up plan

Macular OCT(F/U 1m) F/U 1m : Fd -> pigmentary change 호전됨

Case Review ‘ACUTE RETINAL PIGMENT EPITHELIITIS’ (ARPE)

ACUTE RETINAL PIGMENT EPITHELITIS(ARPE) - Introduction First described in 1972 by Krill and Deutman a rare macular disorder affecting healthy young adults acute, painless, unilateral ‘blurred vision’ or ‘central scotoma’

Anatomy(10 Retinal layers)

Interpretation of Spectral Domain OCT

Natural course of ARPE Self-limited almost all patients regaining good visual acuity within 6 to 12 weeks Recurrence is unusual

Inflammation on and around RPE !? Pathogenesis Inflammation on and around RPE !? Unknown May be an inflammatory disorder of the RPE Transient dysfunction in the RPE/photoreceptor outer segments interface Not been associated with any systemic conditions. cf.) preceding viral illness has been reported the foveal photoreceptors may be the primary site affected in ARPE, with a secondary postinflammatory response in the RPE.

Pathogenesis of ARPE Initial lesion? RPE vs outer neurosensory retina (with a secondary subacute post- inflammatory response in the RPE) increased reflectivity at IS/OS interface the RPE inner band is disrupted a disruption of the IS/OS interface associated with a wider disruption of the RPE inner band The RPE inner band is considered to correspond either to the Verhoeff’s membrane constituted by tight junctions of RPE cells or to basal infoldings and apical processes that enclose the photoreceptor outer segments

Diagnostic clues the presence of a characteristic fine pigment stippling in the macular area at the level of the retinal pigment epithelium(RPE) surrounded by yellow-white haloes of hypopigmentation At the midphase of ICGA, a foveolar patchy hyperfluorescence, which slowly faded, was observed. On ICGA midframe to late frame, a central halolike hyperfluorescence, giving a cockadelike appearance to the macular area, was seen.

Discrete area of pigment stippling associated with a foveolar yellowish hypopigmented lesion Fundus photography Fundus photograph demonstrating a discrete area of pigment stippling associated with a foveolar yellowish hypopigmented lesion.

Angiographic findings(ICG) At the mid-frame, Foveolar patchy hyperfluorescence From Mid to late frame, Central halo-like hyperfluorescence ; Cockadelike app. to the macular area (At the late-frame, ) numerous hyperfluorescent spots of RPE staining scattered throughout the posterior pole and mid-periphery Indocyanine green angiography(4 and 16 minutes) showing a patchy hyperfluorescence in the macular area. A hyperfluorescent macular halo with a cockadelike appearance of the macula is also seen. The intermediate and late hyperfluorescence observed with ICGA, and the associated normal choroidal vasculature, suggest that the RPE may always be involved in ARPE. Indeed, the described hyperfluorescence appears to be related to RPE staining with indocyanine green accumulation within the inflamed RPE cells

Angiographic findings(FAG) Transmission hyperfluorescence (FAG 3 min) ‘transmission hyperfluorescence’ corresponding to the hypopigmented yellowish macular lesions. This hyperfluorescence was observed in a part of and not in the whole area of the hypopigmented area. No leakage of dye into the surrounding retinal tissue was observed in any phase of examination.

Management Prognosis Observation F/U imaging studies to confirm the diagnosis Prognosis Excellent 치료는 일반적인 AMD의 치료를 유지하는 것이 현재의 방침입니다.

White dot syndromes Acute Posterior Multifocal Placoid Pigment Epitheliopathy Birdshot choroidopathy Multiple Evanescent White Dot Syndrome Acute Zonal Occult Outer Retinopathy Multifocal Choroiditis and panuveitis Punctate Inner Choroiditis Serpiginous choroiditis Acute Posterior Multifocal Placoid Pigment Epitheliopathy 건강한 젊은 남녀. 양안대칭 . 1/3 감기증상 선행. 갑자기 발병급격한 시력저하.I 1차 병변부위 맥락막 모세혈관 혈류장애. ICG 지속적인 저형광 Multiple Evanescent White Dot Syndrome : 젊고 건강한 여자 . 단안. Flu like Sx. 선행 급격히 발병. 수주내 자연치유. Fd: 다발성 !! 황반부 주변 몰린 다발성 !! 백색반점, Acute Zonal Occult Outer Retinopathy 젊고 건강한 백인. 일측성증상. 광시증동반 갑작스런 암점 발생. 초기 Fd .특이소견 관찰 안됨. Birdshot choroidopathy :양안성 , 만성 염증. 다발성 저색소 반점 Multifocal Choroiditis and panuveitis : 30대중반 근시인 여자 흔하나. 주로 양안성. 대부분 vit. 심한 염증소견 Punctate Inner Choroiditis : 근시 젊고 건강한 여성, 주로 양안 침범. 증상은 주로 단안 중심암점 시력저하. 앞에 것과 달리 Ant.Uveitis. Viteritis 드물다. CNV 25 % 관찰 . Tx: 스테로이드 PO. Or subtenon . Px. Good.

REference Retinal fluorescein and indocyanine green angiography and spectral-domain optical coherence tomography findings in acute retinal pigment epitheliitis. - Retina. 2011 Jun;31(6):1156-63. . Retina. 2011 Jun;31(6):1156-63. doi: 10.1097/IAE.0b013e3181fbcea5. Retinal fluorescein and indocyanine green angiography and spectral-domain optical coherence tomography findings in acute retinal pigment epitheliitis.