1. Case conference Localized RNFL defect
3 cases of localized RN리 defect and a review of the literature
Pf.안명덕/R3 정윤혜

2. 72/F typical findings of a patient with glaucoma
Large CDR, notching, thinning of neural rim at inf. of right eye and RN리 defect

3. 1yr later, disc hm at inferotemporal area

4. Worsened RN리
Disc morphology, VFD, progressing

5. Glaucoma Optic neuropathy High C/D ratio Neural rim thinning Notching
Bayonetting
Laminar dot sign
Peripapillary atrophy
Progressive loss of the visual field
High CDR, neural rim thinning, notching, bayonetting, laminar dot sign

6. Case 1 김O구(62/M) 16777542 c/c: for elevated IOP(OD)
PI : mod. NPDR(OU)로 f/u 하던 자로, IOP AP 23/22mmHg로 녹내장파트에 의뢰됨. PH
DM/HTN (+/-) for 12yrs, PO medi
Ocular trauma/ op(-/-)
High IOP, RNFL defect, no field defect, nl. optic disc
OHT: high IOP, nl optic disc,

7. Ocular examination VA(OD/OS) 0.8/0.8 IOP AP 21/21 mmHg CCT 533/537 μm
Lid No swelling
Conj. Not injected
Cornea clear
AC deep & cell(-)
Pupil round & nl sized, LR(+/+), RAPD(-/-)
Lens clear
Fds nl. optic disc c multiple dot blot hm at 2Q /(OU)

8. Differential diagnosis
Cue list
High IOP history
Deep angle
Normal optic disc (OU)
Differential diagnosis
R/O Ocular hypertension
R/O POAG
Plan
IOP f/u VF
Thick CCT

9. Progress note (3m later)
O: IOP AP 23/22 mmHg
SWAP : WNL (OU)
A: Ocular hypertension(OU)
P: Start O-TVP x hs (OU)
WNL

10. Progress note (IOP) SWAP RNFL OCT, SWAP, Disc photo O-TVP x hs (OU)

12. O-TVP x hs (OU)
O-TVP x hs (OU)
RNFL OCT,
SWAP,
Disc photo
SWAP

13. Sup. RNFL defect, no neural rim thinning(OU)
Normal CDR no neural rim thinning
Sup. RNFL defect, no neural rim thinning(OU)

14. Sup. RNFL defect(OU)

15. WNL

16. Normal optic disc No field defect RNFL defect
A: Ocular hypertension : RNFL defect
POAG(OU) - Preperimetric glaucoma : normal optic disc
P: Keep O-TVP x hs (OU)
F/U 4m
High IOP, RNFL defect, no field defect, nl. optic disc
OHT: high IOP, nl optic disc, RNFL defect 있어서?
Preperimetric glaucoma: nl. Optic disc이어서 low probability?
Normal optic disc
No field defect
RNFL defect

17. Case 2 정O승 (47/M) 13884923 CC: for DMR evaluation
PI : Type 2 DM 10년 된 분으로, DMR evaluation 위해 내원. PH
DM/HTN (+/-) for 10yrs , po med
Ocular trauma/op(-/-)
Nl. Optic disc,
high IOP RN리 defect, VF defect
중간에 #21 우안 SWAP?
Optic disc는 안 변했는데 RN리 defect 저명해짐-CWP 있었던 곳

18. Ocular examination VA(OD/OS) 0.8/1.0 IOP(OD/OS) 23/27 mmHg
CCT OD 614/ OS 620 μm 17/20mmHg?
Lid No swellling
Conj. Not injected
Cornea clear
AC deep & cell(-)
Pupil round & nl sized, LR(+/+), RAPD(-/-)
Lens clear /(OU)
520microm average error is 0.7mmHg per 10microm of deviation
For the possible overestimation d/t his thick CCT

19. Fundus photo
Nl optic disc
Fds OU normal optic disc c flat post. pole OD flame shape hm below the disc 1DD away, cotton wool patch near the hm

20. Differential diagnosis
Cue list
High IOP?
Deep angle
Normal optic disc (OU)
Cotton wool patch c flame shaped hm(OD)
Differential diagnosis
Mild NPDR(OD)
R/O Ocular hypertension
R/O POAG
Plan
IOP f/u VF
Thick CCT

21. Progress note (6m later)
IOP AP 20/22 mmHg
VF 24-2 (OU) :WNL
F/U 6m
WNL

22. Progress note (1yr later)
IOP AP 24/24 mmHg
SWAP, RNFL/Disc photo(OU)

23. Inf. RNFL defect Sup. RNFL defect
RNFL defect 는 50% of the thickness of RNFL loss 있을때,
Disc 만 확대한 사진을 넣고 이전과 현재를 비교한번 해줄것
Disc 는 크게 변한 모습이 아닌데 RNFLdefect는 저명해짐
Inf. RNFL defect
Sup. RNFL defect

24. Initial 1 yr later 1년전과 비교한 사진
CWP 있던 부위에 RNFL, no change in disc morphology
Disc 만 확대한 사진을 넣고 이전과 현재를 비교한번 해줄것
Disc 는 크게 변한 모습이 아닌데 RNFLdefect는 저명해짐

25. Normal optic disc RNFL defect (CWP) Field defect
A: POAG : normal optic disc
P: Start O-COSTx2 (OU)
F/U 3m
DMR로 인한?
Normal optic disc
RNFL defect (CWP)
Field defect

26. Case 3 최영옥(60/F) 21630754 c/c: for evaluation of glaucoma
PI : 4년전 당뇨 진단 받고 주기적으로 안과 진료 받던 자로, 1년전 안압이 높다는 이야기 듣고 예방적으로 O-LTP x hs (OU) 사용하고 있던 중 본원 진료 원하여 내원. PH
DM/HBP(+/+) for 4yrs/ 10yrs po med
Ocular trauma / OP (-/-)
1달 전 AML 진단 받음
High CDR, LDS, RN리 defect
CWP

27. Ocular examination VA(OD/OS) 0.8/0.8 IOP AP 13/15 mmHg O-LTP x hs (OU)
CCT 563/533 μm
Lid No swellling
Conj. Not injected
Cornea clear
AC deep & cell(-)
Pupil round & nl sized, LR(+/+), RAPD(-/-)
Lens clear /(OU)

28. High CDR, RNFL defect c cotton wool patches(OD>OS)

30. Cue list Impression Plan History of high IOP
High C/D ratio, RNFL defect
Impression
R/O POAG(OU)
Plan
Keep O-LTP x hs (OU)
SWAP 1m later -> BMT 시행으로 f/u loss
Thick CCT
POAG??

31. Progress note (18m later)
IOP AP 17/17 mmHg O-LTP x hs (OU)
SWAP, RNFL/Disc photo(OU)
OD suspicious of inf. arcuate defect
OS suspicious of inf. paracentral scotoma

32. Progress note (1yr 5m later)
Initial
18m later
IOP AP 17/16 mmHg
CWP -> RNFL defect severe
No definite change in optic disc
Satisfy ISN”T rule

33. High C/D ratio RNFL defect (CWP) Field defect A: R/O POAG
P: Keep O-LTP x hs(OU)
F/U 5m
DMR로 인한?
High C/D ratio
RNFL defect (CWP)
Field defect

34. RNFL defect (progressed after CWP) Field defect
Case 1
Normal optic disc
RNFL defect
No field defect
Case 2
Normal optic disc
RNFL defect (progressed after CWP)
Field defect
Case 3
High C/D ratio
RNFL defect (progressed after CWP)
Field defect

35. BRVO with hemorrhage and CWP
Localized RNFL defect with disc hemorrhage
Localized RNFL defect with CWP
Disc hemorrage 후 RNFLdefect는 확실한 녹내장 증거인데 반해( highly specific)
CWP후 RNFLdefect도 CWP가 BRVO에서 보듯 ischemia에 의한 NFLinfarction이니까
당연히 녹내장의 ischemic aspect로 나타나는 증거일 가능성이 있다고 얘기해주고
하지만 보고들을 보면 CWP에 동반된 RNFLdefect는 녹내장이 아니고 진행하지않더라는 얘기가 더 많더라…로 결론 내리면 될듯
그리고 review에 녹내장의 vascular therory에 대해 좀 review 넣는 것이 좋을 듯
Ischemic aspects of glaucoma?
Ischemia

36. Review Localized RNFL defect

37. RNFL defect
Loss of axonal bundles -> neural rim changes of glaucomatous optic atrophy -> visible defects in the retinal nerve fiber layer
Correlate highly with visual field changes, neural rim area

38. ?
RNFL defect = glaucoma

39. Localized Retinal Nerve Fiber Layer Defect
Normal
POAG
NTG
OHT
Reported rates
Nearly 0 %
19 ~ 25 %
47 %
14 %
Sugiyama K, Uchida H, Tomita G, et al. Localized wedge-shaped defects of retinal nerve fiber layer and disc hemorrhage in glaucoma. Ophthalmology 1999;106(9):
Woo SH, Park KH, Kim DH. Comparison of localised nerve fibre layer defects in normal tension glaucoma and primary open angle glaucoma. Br J Ophthalmol 2003;87(6):695-8.
Tuulonen A, Airaksinen PJ. Initial glaucomatous optic disk and retinal nerve fiber layer abnormalities and their progression.. Am J Ophthalmol 1991;111(4):
Localized RNFL defects can be an early sign of glaucomatous damage; however, their presence is not pathognomic for glaucoma.

40. Ischemic theory in Glaucoma
Decreased perfusion pressure-> ischemia of optic disc -> obstruction of axoplasmic flow
Impaired perfusion of the optic nerve head
Impaired circulation in peripapillary retinal arteries
Optic disc hemorrhage
Localized constriction of retinal arteries
The superficial layer of the ONH receives its blood supply via small
branches of the central retinal artery. The prelaminar
region, a small area anterior to the laminar cribrosa, is
mainly supplied by branches from recurrent choroid
arterioles and the short posterior ciliary arteries

41. BRVO with hemorrhage and CWP
Localized RNFL defect with disc hemorrhage
Localized RNFL defect with CWP
Disc hemorrage 후 RNFLdefect는 확실한 녹내장 증거인데 반해( highly specific)
CWP후 RNFLdefect도 CWP가 BRVO에서 보듯 ischemia에 의한 NFLinfarction이니까
당연히 녹내장의 ischemic aspect로 나타나는 증거일 가능성이 있다고 얘기해주고
하지만 보고들을 보면 CWP에 동반된 RNFLdefect는 녹내장이 아니고 진행하지않더라는 얘기가 더 많더라…로 결론 내리면 될듯
그리고 review에 녹내장의 vascular therory에 대해 좀 review 넣는 것이 좋을 듯
Ischemic aspects of glaucoma?
Ischemia

42. Cotton Wool Spots Whitish gray, fluffy deposits
Discrete opaque swellings of the superficial retina
Localized accumulation of axoplasmic debris in the retinal ganglion cell axons due to focal axonal ischemia caused by interruption of orthograde(from the retina to the LGB) or retrograde(from the lateral geniculate body to the retina) axoplasmic transport in the unmyelinated axons
Aggregates of cytoid bodies(mitochondria and other organelles) : distended stumps of RGC axons: light scattering properties-> dense whiteness
Represents areas of focal retinal ischemia from terminal arteriolar occlusion
Cytoid bodies
Terminal swelling of a disrupted ganglion cell axon that has expanded up to 10 fold(5-25 micrometer,) while becoming crammed with mitochondria and other subcellular material as a result of obstruction of axoplasmic transport.

43. Cotton Wool Spots Frequently a manifestation of systemic disorders
Diabetes, hypertension, collagen vascular disease
HIV and other infections
Hematologic disorders and coagulopathies
Heavy PRP 며칠 후에, grossly distended axon end bulbs – silver stain

44. Nonprogressive RNFL defects followed up from 3 to 19 yrs after initial event
Case
73/M, HBP for 17 yrs, IOP 12mmHg (OD)
1yr:CWS resolved and RN리 defect formed in the area of the CWS
RN리 defect did not reach the disc margin
VF: nl
RN리 thickness by OCT was thinned (91microm, 반대안 131microm)
Serial red-free fundus photo, VF, CDR measurements every 6 m for 6 yrs: no progress, no IOP fluctuation
1yr
6yr

45. Sequelae of CWS

46. 69/M
DM type 2/ HBP
IOP 17/19 mmHg

47. Sup temporal localized 구리 defect
No neuroretinal rim thinning or excavation
CDR 0.3/0.4
Inf. Arcuate scotoma DMII/ HBP
IOP 17/19 : NTG로 진단 , f/u c no treatment
1 yr later: isolated CWS at inf. Temporal region -> 1 yr later: new localized RN리 defect & new sup. Arcuate defect

48. Diagnosis of glaucoma cannot be definitely excluded, but spatial and temporal association between the inferior RNFL defect and the CWS suggest ischemic injury from DM and/or systemic hypertension as more likely cause for RNFL loss.

49. RNFL defect Glaucoma vs after CWS
Similarities
orthograde and retrograde axoplasmic flow disruptions in the RGC level
Visual field defects at corresponding areas
Differences
Glaucoma: lamina cribrosa and posterior sclera foramen
CWS: terminal arteriole and venule

50. ?
RNFL defect = glaucoma

51. RNFL defect ≠ glaucoma
대부분

52. Localized RNFL defect ≠ glaucoma
대부분
Does not reach the disc margin
Is not accompanied by neuroretinal rim changes
does not progress for several years