Jumper 2015 WAEPS AMD MasqueradersAMD Masqueraders J. Michael Jumper, MD West Coast Retina San Francisco, CA ... • Angiography – Early blockage, late staining – Hypofluoresence

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AMD Masqueraders

J. Michael Jumper, MDWest Coast RetinaSan Francisco, CA

No DisclosuresOff label use of bevacizumabwill be discussed

Presentation Va 20/60

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47 y.o WF

7 Months Later, Va 20/30

D DystrophyDDegeneration

I InfectiousInflammatoryImmunologicIatrogenicIdiopathic

DITHCO

T TumorTraumaToxicity

H HereditaryHematologic

C Congenital

O Other

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What is AMD?

A chronic and progressive retinal disease of adults > 50 y.o. which begins with deposits of lipid protein andwith deposits of lipid, protein and inflammation in the macula. These deposits (drusen) can lead to swelling, bleeding and scarring of the retina, any of which can cause central vision loss.

Geographic Atrophy Choroidal Neovascularization

Drusen and Drusen-like Deposits

• Small/hard drusen

• Large/soft drusen

• Cuticular drusen (basal laminar deposits)laminar deposits)

• Subretinal drusenoid debris (reticular pseudodrusen)

• < 63 μm

• Common in > 40 y.o.

• Not diagnostic for AMD

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• ≥ 125 μm

• Associated with basal linear deposits

• Associated with advanced AMD

Spaide RF, Curcio CA. Retina (2010) 30(9); 1441-54.

RPE






• Once believed distinct from AMD, now thought to be part of AMD spectrum

• Small, yellow• FA: starry skySpaide RF, Curcio CA. Retina (2010) 30(9); 1441-54.






• More apparent with fundus autofluoresence

• Complications:– Vitelliform macular lesion– CNV – 21%– Geographic atrophy - 11%Spaide RF, Curcio CA. Retina (2010) 30(9); 1441-54.

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• Deposits under the retina

• Similar biochemically to the sub-RPE deposits

• FA: subtle changesSpaide RF, Curcio CA. Retina (2010) 30(9); 1441-54.






• FAF: Hypoautofluorescentspots corresponding to deposits

• OCT: conical deposits above the RPE in the subretinal space

Spaide RF, Curcio CA. Retina (2010) 30(9); 1441-54.

Adult Onset Foveal Pigment Epithelial Dystrophy (AOFPED)

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Acquired Vitelliform Lesions• Definition: “Presence of a

well-circumscribed area of yellowish subretinal material detected in patients older than 40 years”*

• TerminologyAdult onset foveal macular

1987

– Adult-onset foveal macular dystrophy

– Adult-onset foveal pigment epithelial dystrophy

– Pattern dystrophy of the RPE

– Adult vitelliform dystrophy

– Adult Best disease

*Freund KB, et al. Retina (2011) 31:13-25.

20/50

20/80

2000

• Composition– Degenerated photoreceptor

outer segments– Between the outer retina

and RPE• Genetic causes

– Best 1– PRPH2/RDS

Acquired Vitelliform Lesions

– PRPH2/RDS• Other causes

– Cuticular drusen– Reticular pseudodrusen– Traction maculopathies– CSR– AMD– Isolated

Acquired Vitelliform Lesions

• Angiography– Early blockage, late

staining

– Hypofluoresence from the lipofuscin

• Autofluorescence– Intense

hyperautofluorescence

– Helps differentiate from CNV

– Suggests Lipofuscin

FA Late

FAF

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62 yo WM, dark spot, RE

Drusen

62 yo WM, dark spot, RE

• Intravitreal injection of bevacizumab given

• Follow up 10 days later: no change in vision, OCTvision, OCT

• Observed

• Eventually required reduced fluence PDT rapid resolution

Consider a diagnostic challenge with an Anti-VEGF agent in the rare cases when you are not sure if CNV is present

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Ways that CSR Can Masquerade as AMD

• Acquired vitelliform lesion

• Diffuse ooze pattern of FA leakage

• Fibrin can mimic fibrosis• Fibrin can mimic fibrosis

• Chronic CSR– RPE atrophy

– Cytoid macular degeneration













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From Iida T, et al. Retina (2003) 23:1-7.

Ways To Differentiate CSR From AMD

• FA findings– Pinpoint leak

• Multifocal in 29%• Patterns

– Inkblot– Smokestack (7-20%)Smokestack (7 20%)

– RPE guttering

• OCT findings– Roughening

protrusions– CSR has a thickened

choroid whereas AMD has choroidal thinning





– RPE guttering




Imamura Y, et al. , Ophthalmology 2011;118:700–705.

FAF

Guttering pattern on FA is strongly suggestive of CSR

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– RPE guttering








– RPE guttering




Imamura Y, et al. , Retina (2009) 29: 1469-1473

Normal

CSR

Polypoidal ChoroidalVasculpathy AMD

Chung SE, Kang SW, Lee JH, Kim YT. Ophthalmology 2011;118:840–845

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Polypoidal Choroidal Vasculopathy

• Serosanguineousmaculopathy with…– Clinically visible orange-red

subretinal nodules– Spontaneous massive

subretinal hemorrhage– Notched or hemorrhagic

PED– Lack of response to VEGF

therapy• Of presumed AMD cases

– Caucasians 4-10%– Asians 24-55%

PCV• Suspected to be a primary

abnormality of the choroid• Indocyaine Green

Angiography (ICGA)– Less affected by blood– Identify polyps– Direct PDT– PDT + Ranibizumab was

better than Ranibizumabmonotherapy*

*Koh a, et al. Retina (2012) 32: 1453‐64.

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Polypoidal Choroidal Vasculpathy

47 y.o Healthy WF With Fluctuating Vision OS x 1 Year

Presentation 7 Months Later



DITHCO



C Congenital

O Other

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DITHCO



C Congenital

O Other


47 y.o Healthy WF With Fluctuating Vision OS x 1 Year


11 Months Later B-Cell Lymphoma

Conclusions• Phenotype of AMD is variable

- Drusen

- Atrophy

- Pigment alteration

- CNV

• Not all drusen are alike: small drusen are not diagnostic of AMD

• Acquired vitelliform lesions can be differentiated from CNV most easily by the FAF pattern- CNV dark

- AVL bright

• Choroidal thickening with EDI-OCT can differentiate CSR and PCV from AMD

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Conclusions• Consider an anti-VEGF injection challenge in cases

that may represent wet AMD- Atypical CSR

- Acquired vitelliform lesions with subretinal fluid

• Consider ICGA to rule out polypoidal choroidalvasculopathy in patients who are poorly responsive to y yanti-VEGF therapy- Especially in non-Caucasion patients

- PCV is more common than originally suspected, even in Caucasians

• When all else fails….DITHCO

Cone Dystrophy AOFPEDPolypoidal Choroidal

Vasculopathy

Chronic CSR Myopic DegenerationVitreoretinalLymphoma

Stargardt DzType 2 membranoproliferative

glomerulonephritis

North Carolina Macular Dystophy

AMD Masqueraders

J. Michael Jumper, MDWest Coast RetinaSan Francisco, CA

Documents

Jumper 2015 WAEPS AMD MasqueradersAMD Masqueraders J. Michael Jumper, MD West Coast Retina San Francisco, CA ... • Angiography – Early blockage, late staining – Hypofluoresence