1. 망막 CONFERENCE
R2 이지영/St.전소희

2. M/47 조 O 영 History C/C : floater(OD) for 1 week P/I DM/HBP (-/-)
상기 환자 severe aplastic anemia로 본원 혈액종양내과에서 골수이식예정인 환자로 내원 1주일 전부터 우안 비닐막이 씌운 것 같고 물방울이 보이는 증상 발생하여 내원
History
DM/HBP (-/-)
Severe Aplastic anemia: 2011년 10월 진단
ATG 3.5mg/kg for 5days : ~ 12.17
G-CSF : ~ (9 times)
Cyclosporin 300mg /d : ~
Oral candidiasis, Herpes zoster 진단
Ocular op/trauma(-/-)
Eye drop (-)

3. Ocular exam VA OD 0.4(N-C) / OS 1.0 IOP OD 9 / OS 13 mmHg
Conj OU mild injected
Cornea OD mild edematous, KP’s
AC OD deep & cell(rare)
OS deep & cell (-)
Pupil OD round & dilated, LR(+, slugguish)
Lens OD mild cortical opaticy, iris pigment on ant. surface
Fd OD nl optic disc c dot & blot Hm. c multiple whitish-yellow lesion at inf.
some exudates c multiple sheathed vessels
OS CDR 0.6 c flat post pole

4. Fundus photo
retinal vascular sheathing : neutrophilic perivascular infiltration
Perivascular hemorrhage
granular patches
Confluent areas of retinal whitening associated with venous sheathing and hemorrhages along the major retinal vascular arcades
irregular granular borders with small, isolated satellite lesions
adjacent to retinal vessels with hemorrhage

5. Lab findings(2012.2.27) CBC WBC: 940 /µl seg neu: 53% lymph: 42%
Mono : 5%
Eosino: 0
Baso: 0
ANC 500
Plt
Hb/Hct 6.9/19.8

6. Impression CMV retitis ? R/O ARN (OD) R/O CMV retinitis (OD)
Immunocomprimised pt.
Mild AC reaction
Fd : granular patches, confluent areas of retinal whitening associated with venous sheathing and hemorrhages

7. Fundus photo
retinal vascular sheathing : neutrophilic perivascular infiltration
Perivascular hemorrhage
granular patches
Confluent areas of retinal whitening associated with venous sheathing and hemorrhages along the major retinal vascular arcades
irregular granular borders with small, isolated satellite lesions
adjacent to retinal vessels with hemorrhage

8. Impression ARN>CMV retinitis R/O ARN (OD) R/O CMV retinitis (OD)
Peripheral retinal necrosis
Vessel 따라서 spread 되는 양상이 아님.

9. DDx. Progressive outer retinal necrosis (PORN)
toxoplasmosis chorioretinitis
syphilitic retinitis
acute multifocal hemorrhagic retinal vasculitis
toxocariasis
fungal or bacterial retinitis
Behcet’s disease
sarcoidosis
commotio retinae
central retinal artery or ophthalmic artery occlusion
ischemic ocular syndrome
collagen-vascular disases
intraouclar leukemia / lymphoma
retinoblastoma

10. Plan Diagnositc test Therapeutic plan Daily f/u
CMV, HSV, VZV, EBV IgM/G
CMV, HSV, VZV, EBV RQ PCR
Therapeutic plan
IV acyclovir 1800mg #3
O-1AT x2, O-LON xq2, O-MOX x4, O-GC x5 / OD
Intravit. ganciclovir inj.(OD)
Daily f/u

11. Viral titer CMV RQ PCR <500 VZV RQ PCR (+) HSV type I PCR (-)
CMV RQ PCR (-) at blood
EBV RQ PCR 6393 copy/mL at blood
CMV IgM (-) IgG (+) at serum
HSV IgM (-), IgG (+) at serum
VZV IgM (-), IgG (+) at serum

12. O-1AT x2, O-LON xq2, O-MOX x4, O-GC x5(OD)
Progression note
trace ++ + -
AC cell
Vit cell
intravitreal ganciclovir inj.(OD)
O-1AT x2, O-LON xq2, O-MOX x4, O-GC x5
IV acyclovir 1800mg
Po acy 4000mg
H-LON 60mg
H-LON 50mg
O-1AT x2, O-LON xq2, O-MOX x4, O-GC x5(OD)

15. O-1AT x2, O-LON xq2, O-MOX x4, O-GC x5(OD)
Progression note
trace ++ + -
AC cell
Vit cell
intravitreal ganciclovir inj.(OD)
intravitreal foscarnet inj.(OD)
O-1AT x2, O-LON xq2, O-MOX x4, O-GC x5
IV acyclovir 1800mg
Po acy 4000mg
H-LON 60mg
H-LON 50mg
O-1AT x2, O-LON xq2, O-MOX x4, O-GC x5(OD)

17. 2012.3.12 Plan - acyclovir 4000mg#5, H-LON 50 mg - keep eye drops
VA OD (0.5)
IOP OD mmHg
Conj OD not injected
Cornea OD clear
AC OD deep & cell(+)
Pupil OD round & mild dilated d/t cycloplegics
Lens OD mild cortical opacity c mod. NS
Fds OD nl. optic disc c perifoveal whitish confluent lesion - decreased
Vit cell (+) vitreous opacity (+)
multiple retinal Hm. c multiple sheathed vessels at inf. arcade
multiple whitish-yellow lesion at inf. - decreased
Plan
- acyclovir 4000mg#5, H-LON 50 mg
- keep eye drops
- discharge 후 OPD f/u , intravit. foscarnet inj

18. Acute Retinal Necrosis
Review
Acute Retinal Necrosis

19. Introduction Definition Epidemiology
characterised by confluent, peripheral, necrotizing retinitis, peripheral occlusive arteritis and moderate-to-severe vitritis
Epidemiology
No association between gender, race, age
Typically occur in healthy, immunocompetent pts
But also be found in immunocompromised pts.

20. Etiology multiple members of the Herpes virus family
Varicella-zoster virus(m/c)
Herpes simplex virus 1
Herpes simplex virus 2
Cytomegalovirus
Epstein-Barr virus
HLA haplotypes
HLA-DQw7, HLA-Bw62, DR4
Bilateral involvement occurs in 2/3 of the patients
frequently in the first six weeks, but sometimes months to years later.
no correlation between the severity of ARN in the first eye

21. Clinical presentation
Symptom
mild to moderate ocular or periorbital pain
red eye
visual symptom
floaters, blurred vision
decreased vision
vitreous opacification, necrotizing retinitis, optic neuritis or neuropathy

22. Clinical presentation
Episcleritis or scleritis
Anterior uveitis
Mild to moderate anterior segment cellular reaction
granulomatous or fine keratic precipitates
Hypopyon, dendritic or stromal keratitis(rare)
Acute necrotiziong retinitis
Deep, multifocal, yellow-white patches
typically beginning in the peripheral fundus(360 degrees)
spreading toward the posterior pole
the macula is frequently spared
Active vasculitis
perivascular hemorrhages, vascular sheathing
terminal obliteration of arterioles by thrombi
In early ARN, multifocal white patches of retinal necrosis occur in the peripheral retina
With progression of ARN, the areas of retinal whitening begin to coalesce. Mild perivascular retinal hemorrhages are noted
The peripheral retinal whitening becomes confluent with vascular obliteration.

23. Clinical presentation
Vitritis (severe)
Choroiditis
Optic nerve involvement
Optic nerve swelling
Dyschromatopsia
Relative afferent papillary defect
Visual field defect
Bilateral involvement occurs in 2/3 of the patients
frequently in the first 6 weeks, but sometimes months to years later.
no correlation between the severity of ARN in the first eye
Bilateral involvement occurs in 2/3 of the patients
frequently in the first six weeks, but sometimes months to years later.
no correlation between the severity of ARN in the first eye

24. Clinical presentation
Resolusion phase (4~6 weeks)
the retinal areas appear thinned and atrophic
underlying retinal pigment epithelial alterations (distinct scalloped pattern)
Full-thickness retinal breaks
large, irregular, and multiple
at the junction of normal and affected retina
accompanying vitreous fibrosis and traction
Rhegmatogenous RD
major cause of visual loss
occurs in 50% to 75% of cases not treated with prophylactic laser photocoagulation
high grade PVR, posterior located multiple break
6 days~ several months (4~6 weeks)

25. Clinical presentation
Initial reports of ARN in healthy patients with normal systemic immunity
no extraocular manifestations of herpetic infection
Secondary to reactivation of a latent herpetic infection
dermatomal VZV (herpes zoster)
primary VZV (chickenpox)
perioral blisters (HSV)
reactivation of the viral genome from the trigeminal ganglion may result in the development of ARN after transneural spread
uncommon for healthy individuals to develop ARN simultaneously with cutaneous VZV or HSV infection

26. Immunocompromised patients
ARN subsequently has been reported in immunocompromised patients with HIV infection, autoimmune disorders, cancer, and organ transplants
rapidly progressive necrotizing retinitis
Bilateral involvement in 90%
mild anterior segment cellular reaction
decreased Involvement of retinal and choroidal vessels
More commonly have retinal detachment
poor responsive to acyclovir treatment
worse visual outcome
More recurrence
Bilateral involvement in 90%
More commonly have retinal detachment(80%)
Worse response to the antiviral treatment
More recurrence

27. Diagnosis American Uveitis Society diagnostic criteria
1. 진단기준 (한글판 망막)
1) 주변 망막에 하나 이상의 경계가 분명한 망막괴사 병소
2) 치료하지 않으면 급속히 진행
3) 병변의 진행방향이 측면으로 확산
4) 세동맥의 폐쇄혈관염
5) 유리체와 전방에 염증소견
(시신경증이나 공막염, 안구통은 필수요건이 아님)
위의 다섯 가지 소견이 모두 보여야 ARN
그렇지 않으면 necrotizing herpetic retinopathy
The diagnosis of ARN is based on
clinical examination
characteristic funduscopic appearance

28. Laboratory evaluation
Aqueous humor and/or vitreous fluid
Qualitative PCR
Quantitative PCR
Presence of viral Ab in the pph. Blood
not specific finding
Serum Ab
not necessarily elevated

29. FAG Not diagnostic arterial phase venous phase recirculation phase
focal choroidal perfusion defects
venous phase
abrupt “cut off” of the intravascular F pattern
recirculation phase
staining of the optic disc
staining of both retinal a & v d/t obliterative vasculitis

30. Diagnostic vitrectomy or retinal biopsy
atypical cases
evaluation of intraocular samples with various techniques, such as viral culture, serology, polymerase chain reaction, and histopathologic examination
The viral cause of ARN often is difficult to determine in an end-stage atrophic or detached retinal specimens
more readily identified in ocular specimens obtained during acute stages of the disease

31. Differential diagnosis
Progressive outer retinal necrosis(PORN)
Immunocompromised patients
Varicella-zoster retinitis
Extensive, rapidly progressing full-thickness necrosis of the retina involving posterior pole
No associated AC or vitreous reaction, rare vascular inflammation
Poor response of antiviral therapy

32. Differential diagnosis
CMV retinitis
immunocompromised individuals
the clinical course is chronic and indolent
minimal or absent vitreous inflammation
initially with 1 or 2 patches of white granular retinal lesions at post. pole
Hemorrhage is more common
Hematogenous spread
no response acyclovir

33. Differential diagnosis
Behcet disease
FAG : broom-shaped dye leakage from capillary wall
Sarcoidosis
Nodular periphlebitis
Toxoplasmosis
Elevated lesion in chorioretinitis
dense yellow-white lesions with better defined borders
Inflammation is usually more intense
peripheral retina is usually less involved
Syphilis
Diffuse, patchy necrotizing retinitis, prominent vitritis and vaculitis

34. Treatment Anti-viral treatment Anti-inflammatory treatment
Anti-thrombotic treatment
Vitreous surgery

35. Treatment Anti-viral treatment Acyclovir Induction therapy
IV Acyclovir : standard Tx.
maintenance therapy
Oral acyclovir : after IV acyclovir ( fellow eye에 대한 prophylaxis)
Immunocomprimised pt. – more long term maintenance
Ganciclovir
first-choice of CMV retinitis
acyclovir 에 효과 없는 경우에 효과가 있을 수 있음.
BM suppression -> pancytopenia
Valacyclovir : pro-drug of acyclovir, useful for the initial Tx.
- maintenance therapy
Alternative antivirals should be considered if the retinitis does not respond in a timely fashion to acyclovir within 3-5 days

37. Treatment Anti-inflammatory treatment Anti-thrombotic treatment
for intraocular and vitreous inflammation
IV acyclovir 투여 후 시간 뒤에 시작
for optic nerve dysfunction(바로 시작)
Anti-thrombotic treatment
d/t occlusive retinal arteritis and phlebitis
Low dosage aspirin(100mg /day)
for ischemic optic neuropathy
IV acyclovir 투여 후 시간 뒤에 시작

38. Treatment Prophylactic Laser Photocoagulation
The use of confluent laser photocoagulation posterior to active retinitis has been recommended to prevent retinal detachment.
Retinal Detachment Surgery

39. 감사합니다.