1. 가톨릭대학교 의과대학 안과 및 시과학교실 서울성모병원 AP.박영훈 / R3박진형
Retina conference
가톨릭대학교 의과대학 안과 및 시과학교실
서울성모병원
AP.박영훈 / R3박진형

2. Case #1

3. Chief Complaint
Dec. VA (OD)
onset: 3 wks ago
Patient:
39/F
김 O 숙

4. Present Illness
30세 여자
상환 13년 전 LASIK(OU) 받은 분으로 근시퇴행 있어 6개월 전 우안 추가 LASEK 후 관찰 중 (당시 우안 시력 중간 정도까지는 보였다고 함)
내원 3~4주전부터 우안의 시력감소 있어 망막 정밀검사 위해 의뢰됨
Nyctalopia(+) : 어렸을 때부터
FHx. of nyctalopia(+) : 어머니
URI Hx.(-)
Headache(+) : 내원 2~3주전 -> 구토 동반되었음
Temporal Td(-/-)
Travel Hx.(+) : 3주전 파리 출장
Brain evaluation Hx.(-)
Syncope Hx.(+) 20대 때
VF defect(-)
EOM pain(-)

5. History DM/HBP (-/-) Ocular op/trauma(+/-) 13년 전 LASIK(OU)
6개월 전 LASEK(OD) at local
Gls (-)
Eye drop (+) 인공누액

6. Physical Examination VA OD 0.08 (N-C) OS 0.63 (1.0) IOP 29 / 15 mmHg
EOM Straight at 1' position by ACT , No LOM(OU)
Orbit No exophthalmos(OU)
Lid OU No swellling
Conj. OU not injected
Cornea OU LASIK flap scar(+)
AC OD Deep& cell(-)
OS Deep& cell(-)
Pupil OD round & 4.5mm sized, LR(+)
OS round & 2mm sized, LR(+), RAPD(+/-), NVI(-/-)
Lens OU mild cortical opacity
Fds OD tilted disc (CDR 0.8/0.8) c PPA c flat post.pole
OS tilted disc (CDR 0.8/0.8) c PPA c flat post.pole

7. Fundus photography OU

8. FAG OD

9. ICG OD

10. RNFL OCT OU

11. Disc photo & ONFL photo OU
Disc : OD sup & inf RNFLD
OS sup RNFLD
FDT : OD central defects
OS nasal defects

12. MRI Brain
T1 enhance랑 T2에서 optic nerve나 sheath는 intact 해보임

13. MRI Brain T1 enhance에서 양측 MCA가 안보임
우측은 collateral Br.가 발달되었고 좌측은 brain volume 감소로 보아 오래된 ischemia로 atrophic change가 왔음
Whitish dot들이 ischemic change site임

14. Differential diagonosis
Cue list
MRI Brain : Leptomeningeal collaterals without visible bilateral proximal MCA
Elevated IOP(OD), RNFL defect(OU)
Imp.
R/O moyamoya disease
G. suspect (OU)
Plan
NS consult
O-COST(OD), O-BMDP(OD), O-LTP(OU)
VF (OU)

15. 4-Vessel angiography – 3days later
일단 ophthalmic a.는 intact 해보임
Brain 안쪽은 다 ICA가 supply 해야하는데 4VA 소견 상 ICA에서 나오는 ACA나 MCA가 안보이고
Collateral만 보임
ECA는 원래 scalp level만 supply 해야 하는데 occipital a.와 연결되어 brain에 supply 하고있음
ACA는 ICA에서
The angiography showed 1) Not visuable beyond communicating segment of right ICA and well developed basal collateral circulation via PCA 2) Right ACA territory was supplied from anterior falx artery 3) Right PCA territory was supplied from transosseous branch of occipital artery. 4) Not visuable beyond ophthalmic segment of left ICA 5) Left ACA territory was supplied from anterior falx artery 6) Transdural anastomosis of left MMA posterior branch 7) Well developed basal collateral circulation originated from both PCA 8) Hypertrophic tentorial marginall branch of meningohypophysial trunk. both and these artery partially supply both PCA territory

16. 4-Vessel angiography – 3days later
양측 다 아지랑이 모양의 collateral Br.만 잔뜩 보임 – moyamoya의 전형적인 소견
ACA는 ICA에서
The angiography showed 1) Not visuable beyond communicating segment of right ICA and well developed basal collateral circulation via PCA 2) Right ACA territory was supplied from anterior falx artery 3) Right PCA territory was supplied from transosseous branch of occipital artery. 4) Not visuable beyond ophthalmic segment of left ICA 5) Left ACA territory was supplied from anterior falx artery 6) Transdural anastomosis of left MMA posterior branch 7) Well developed basal collateral circulation originated from both PCA 8) Hypertrophic tentorial marginall branch of meningohypophysial trunk. both and these artery partially supply both PCA territory
Plan : encephaloduroarteriogaleasynangiosis
STA(ECA) – MCA(ICA) anastomosis

17. Visual field OU – 1wk later

18. Diagnosis Plan Moyamoya Dz. POAG(OD) NTG(OS)
Keep O-COST, O-BMDP, O-LTP
TAGF PO
F/U 4mo.

19. Brain SPECT – after anastomosis
시술 후 Perfusion을 보기 위한 SPECT
좌측은 안정 시, 우측은 혈관을 dilation 시키는 약물 투여 시
좌측이 우측에 비해 덜 빨감 – 덜 perfusion 됨

20. Case #2

21. Chief Complaint
Dec. VA (OD)
onset: 1 wk ago
Patient:
33/M
최 O 환

22. Present Illness
33세 남자
일주일 전 갑자기 우안 시력 저하 생겼으며 local에서 당뇨병성망막병증일 가능성 높다고 하여 further evaluation 위해 내원함

23. History BP 200/100 mmHg DM/HBP (-/+) for 5yrs, no medi.
Ocular op/trauma(-/-)
Gls (+)
Eye drop (-)
BP 200/100 mmHg

24. Physical Examination VA OD 0.02 (0.4) OS 0.1 (1.0) IOP 16 / 17 mmHg
EOM Straight at 1' position by ACT , No LOM(OU)
Orbit No exophthalmos(OU)
Lid OU No swellling
Conj. OU not injected
Cornea OU clear
AC OU Deep& cell(-)
Pupil OU round & nl sized, LR(+), RAPD(-/-), NVI(-/-)
Lens OU mild cortical opacity
Fds OU blurred disc margin c papilledema
flame shaped Hm. c tortous peripapillary vv.
exudative lesions c multiple CWS
OD ME(+)

25. Fundus photography OU

26. mOCT OU

27. Differential diagonosis
Cue list
Slit lamp exam : ME(OD) disc swelling(OU)
retinal Hm. c hard exudates, CWS (OU)
Imp.
R/O DM retinopathy(OU)
R/O hypertensive retinopahty(OU)
Plan
ME consultation for lab.
Intravit. Avastin inj.(OD)
BUN/Cr 30.2 / 2.40
Blood glucose 107
HbA1c 5.4
Urine glucose (-)

28. Differential diagonosis
Cue list
Slit lamp exam : ME(OD) pseudopapilledema(OU)
retinal Hm. c hard exudates, CWP (OU)
Imp.
R/O DM retinopathy(OU)
R/O hypertensive retinopahty(OU)
Plan
ME consultation for lab.
Intravit. Avastin inj.(OD)
Done after 1wk
BUN/Cr 30.2 / 2.40
Blood glucose 107
HbA1c 5.4
Urine glucose (-)

29. 3mo. F/U Fundus photography OU

30. 3mo. F/U mOCT OD – VA (1.0)

31. Review : Disc swelling

32. Disc swelling?

33. Optic neuropathy DDx Pseudopapilledema ? Systemic dz 동반
Brain MRI 및 CSF 이상소견
FAG 이상소견 VA acute loss
Pseudopapilledema ?
Leber hereditary optic neuropathy
Methanol poisoning
Tiled optic disc
Peripapillary myelinated nerve fibers
Crowded disc in hypermetropia

34. Optic neuropathy DDx

35. Disc edema Key features Blurring of the optic disc margins
Anterior extension of the nerve head
Venous congestion of arcuate and peripapillary vessels
Hyperemia of the optic nerve head

36. Disc edema Pathophysiology blockage of optic nerve axoplasmic flow
stasis of axoplasmic flow
increases venous pressure at or near the lamina cribrosa

37. Disc edema OCULAR MANIFESTATIONS Mechanical clinical signs
Blurring of the optic disc margins
Filling in of the optic disc cup
Anterior extension of the nerve head (3D = 1mm of elevation)
Edema of the nerve fiber layer
Retinal or choroidal folds, or both
Vascular clinical signs
Venous congestion of arcuate and peripapillary vessels
Papillary and retinal peripapillary hemorrhages
Nerve fiber layer infarcts (cotton-wool spots)
Hyperemia of the optic nerve head
Hard exudates of the optic disc.

38. Disc edema early – fully developed – chronic - late Disc hyperemia
Blurred margin
NFL blurring
gross elevation of the optic nerve head
engorged and dusky veins appear
peripapillary splinter hemorrhages
Hemorrhages occur
disc cup is obliterated completely
less disc hyperemia
hard exudates occur within the nerve head
secondary optic atrophy
disc swelling subsides
retinal arterioles are narrowed or sheathed
optic disc appears dirty gray and blurred

39. Disc edema SYSTEMIC ASSOCIATIONS acute optic disc edema
axoplasmic stasis, edema, and vascular congestion

40. Pseudopapilledema
papilledema is disc edema secondary to increased intracranial pressure
pseudopapilledema is apparent optic disc swelling that simulates some features of papilledema but is secondary to an underlying process(usually benign)
Most patients with pseudopapilledema lack visual symptoms
no obscuration of the peripapillary vessels by the nerve fiber layer edema
may be unilateral or bilateral

41. Patholphysiology Tilted disc hyperopic eye
the optic nerve enters the eye at an extremely oblique angle, giving a portion a more elevated aspect (usually nasally)
hyperopic eye
smaller optic cup -> crowding of the axons
Myelinated nerve fiber layer
can lead to the appearance of a large cup with blurring of the disc margins.
disc drusen
small conglomerates of mucopolysaccharides and proteinaceous material that become calcified with advancing age

42. Patholphysiology
remnants of the congenital hyaloid system and localized gliosis
optic nerve tumors
Papillitis vs papilledema??
inflammatory, infiltrative, and infectious conditions
syphilis, Lyme disease, and cat-scratch disease
anterior ischemic optic neuropathy, optic neuritis, diabetes, sarcoidosis, and leukemic infiltration

43. Presentation Visual field loss VA Fundus
In many patients with disc drusen, visual field defects eventually develop along with afferent pupil defect, though patients usually remain symptom free. VA
A minority of patients with disc drusen experience transient visual flickering or graying out that is similar to transient visual obscurations that are sometimes seen in patients with papilledema.
Fundus
Edema of the nerve fiber layer that blurs the disc margins and the peripapillary vasculature is a hallmark of true papilledema.
Usually, the peripapillary vessels are clearly seen in pseudopapilledema, except in such cases as myelinated nerve fibers.
the disc is yellow, the cup may be small or absent, venous congestion is not present, spontaneous venous pulsations are often present, congenitally anomalous vessels may be seen, and the disc abnormality may be familial.

44. Follow up With disc drusen, The visual prognosis is generally good.
gradual loss of the peripheral visual field may occur and, rarely, loss of central vision
The visual prognosis is generally good.