Case conference Localized RNFL defect 3 cases of localized RN리 defect and a review of the literature 2011.6.23 Pf.안명덕/R3 정윤혜

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

1yr later, disc hm at inferotemporal area

Worsened RN리 Disc morphology, VFD, progressing

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

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,

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)

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

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

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

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

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

Sup. RNFL defect(OU)

WNL

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

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

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

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

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

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

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

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

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

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

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

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)

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

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??

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

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

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

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

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

Review Localized RNFL defect

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

? RNFL defect = glaucoma

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):1762-7. 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):485-90. Localized RNFL defects can be an early sign of glaucomatous damage; however, their presence is not pathognomic for glaucoma.

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

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

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.

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

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

Sequelae of CWS

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

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

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.

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

? RNFL defect = glaucoma

RNFL defect ≠ glaucoma 대부분

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