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

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

3. 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)

4. 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

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

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

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

8. f/u ( )
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

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

10. f/u ( )
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

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

12. review Cystoid macular edema with retinitis pigmentosa

13. 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

14. 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

15. 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

16. 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

17. 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

18. 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)

19. 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

20. 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

21. 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

22. 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 : months
Results
Mean macular thickness : ㎛  ㎛
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

23. 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

24. 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:

25. 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