angio conference Cystoid macular edema with retinitis pigmentosa R3 공석준/St.이미연

C/C Dec.VA(OD) onset.) several month ago case # 15375532 이 O 형 M/29 2010.11.3 C/C Dec.VA(OD) onset.) several month ago P/Hx DM/HBP (-/-) Ocular Op/trauma(-/-) Gls(+) for 15yrs Eyedrops(-)

Ocular Examination V/A OD (0.4)(n-c) OS (0.8) IOP OD 13 mmHg OS 15 mmHg Ant. Segment : non-specific Fd tilted optic disc c bony spicule-like pigment at pph c macular edema (OD>OS)

F-photo – 11/3(OU) 좌안 B-scan소견 입니다. 융기된 둥근 지붕형태의 종양이보이며 종양 앞 표면에 높은 스파이크가 나타나고 종양을 지나는 스파이크 들은 50~100%의 내반사파를 보입니다. OD OS

M-OCT – 11/3(OU) 좌안 M-OCT 사진입니다. 황반부위에 맥락막 부분이 전반적으로 융기된 소견 보이여 소량의 SSRD 소견 보이고 있습니다. OD OS

FAG – 11/5(OU) 좌안 M-OCT 사진입니다. 황반부위에 맥락막 부분이 전반적으로 융기된 소견 보이여 소량의 SSRD 소견 보이고 있습니다.

Impression Plan Cystoid macular edema c retinitis pigmentosa (OU) Acetazolamide 3T#3 for 1week KCL 1.5T#3 for 1week

f/u (2010.11.12) V/A OD (0.1) OS (0.8) IOP OD 12 mmHg OS 10 mmHg Ant. Segment : non-specific Fd tilted optic disc c bony spicule-like pigment at pph c nearly flat macula (OU) Plan Acetazolamide 2T#2 for 1week KCL 1T#2 for 1week

M-OCT – 11/12(OU) 좌안 M-OCT 사진입니다. 황반부위에 맥락막 부분이 전반적으로 융기된 소견 보이여 소량의 SSRD 소견 보이고 있습니다. OD OS

f/u (2010.11.17) V/A OD (0.63) OS (0.8) IOP OD 11 mmHg OS 10 mmHg Ant. Segment : non-specific Fd tilted optic disc c bony spicule-like pigment at pph c flat macula (OU) Plan Cut acetazolamide

M-OCT – 11/17(OU) 좌안 M-OCT 사진입니다. 황반부위에 맥락막 부분이 전반적으로 융기된 소견 보이여 소량의 SSRD 소견 보이고 있습니다. OD OS

review Cystoid macular edema with retinitis pigmentosa

Retinitis pigmentosa Group of hereditary progressive retinal disease caused by gene abnormalities on several different chromosomes Resulting in the progressive loss of photoreceptors  impaired night vision and a gradual loss of visual field

Clinical features Nyctalopia Beginning in first or second decade of life Narrowing of the visual field in the dark Getting easily disoriented on dimly lit evening or becoming accident prone at night Not pathognomonic of RP

Clinical features Visual field loss Insidious, preogressive loss of peripheral visual feld Detected in early disease with small, dim test target Earliest defect – relative scotomas in the midperiphery, between 30 and 50 degree For many types of RP, field deficit are usually found in the superior fileld

Clinical features Central vision loss Color vision defects Photopsia Can be seriously affected early in typical RP CME, diffuse retinal vascular leakage, macular periretinal fibrosis, RPE defects Color vision defects Remains good until the visual acuity is 20/40 or worse Fail early if central cones appear to be abnormal from the beginning Photopsia Occurring in the midperipheral field

Fundus findings Attenuated retinal vessels Mottling and granularity of the retinal pigment epithelium Bone-spicule intraretinal pigmentation Optic nerve pallor Increased luster, abnormal highlights, or wrinkling

CME in RP The prevalence of CME in RP Most commonly reported to be from 11% to 20% using fluorescein angiography and fundus exam Prevalence of CME in RP using OCT 13% (Hirakawa et al) 49% (Adackapara et al)

CME in RP Pathogenesis Not yet clearly understood Dysfunction of the outer blood-retinal barrier, RPE Increase vascular permeability and fluid leakage through the RPE  CME in RP CAI such as acetazolamide can be used to treat  by stimulating the pumping mechanism of the RPE

CME in RP Association with the high prevalence of antiretinal autoantibodies in patients with this condition Suggest that an inflammatory, autoimmune process may play a role  administration of steroids may be effective in treating this condition

Treatment of CME in RP Topical dorzolamide Methods Results 15 patients Topical dorzolamide, 3 times a day, for at least 4 weeks in both eyes Main outcome measure : foveal thickness measured by OCT Results 13 (87%) patients : significant decrease in retinal thickness in at least one eye 4 (31%) patients : initial improvement -> worsening with continued treatment (rebound phenomenon) Topical Dorzolamide for the Treatment of Cystoid Macular Edema in Patients With Retinitis Pigmentosa Am J Ophthalmol 2006;141:850–858

Treatment of CME in RP Intravitreal avastin injection Methods Results 13 eyes of 7 patients Inravitreal avastin 1.25mg/0.05ml was injected Main outcome measure : visual acuity, macular thickness measured by OCT F/U period : 10.28 months Results Mean macular thickness : 370.15 ㎛  142.53 ㎛ VA : 5/400~20/100  20/200~20/63 VEGF Contributes to blood retinal barrier breakdown  ME Induces vascular fenestration  increase in permeability of microvessels  fluid leakage & accumulation in the choriocapillaris Intravitreal Bevacizumab (Avastin) Injection in Retinitis Pigmentosa Current Eye Research 2009; 34: 231–237

Treatment of CME in RP Intravitreal lucentis injection Methods Results 15 eyes of 15 patients Inravitreal lucentis 0.5mg was injected Main outcome measure : BCVA, macular thickness measured by OCT F/U period : 6months Results Mean macular thickness : 478±88 ㎛  272±65 ㎛ BCVA : logMAR 0.72±0.22  0.69±0.18 Intravitreal Ranibizumab in the Treatment of Cystoid Macular Edema Associated With Retinitis Pigmentosa J Ocul Pharmacol Ther. 2009;25:545-50

Treatment of CME in RP Intravitreal triamcinolone injection Methods 20 eyes of 20 patients Inravitreal triamcinolone 4mg/0.1ml was injected Main outcome measure : BCVA, macular thickness measured by OCT F/U period : 6months Results Mean macular thickness : 456±95 ㎛  299±76 ㎛ BCVA : logMAR 0.66±0.21  0.66±0.25 Steroid Reduce levels of proinflammatory cytokines, VEGF Increase blood-retinal barrier function with edema solution Treatment of Cystoid Macular Edema in Retinitis Pigmentosa With Intravitreal Triamcinolone Arch Ophthalmol. 2007;125:759-764

Treatment of CME in RP Acetazolamide Methods Results 10 eyes of 10 patients Acetazolamide 250mg/d PO Main outcome measure : BCVA, macular thickness measured by OCT F/U period : 4-12 months Results 6 patients had significant decrease in ME by follow-up OCT 6 patients BCVA improved by ≥ 1 line in at least one eye OPTICAL COHERENCE TOMOGRAPHY IN THE DIAGNOSIS AND MONITORING OF CYSTOID MACULAR EDEMA IN PATIENTS WITH RETINITIS PIGMENTOSA RETINA 2006;26:922–927