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Meet The Choroid

Joe Pizzimenti, OD, FAAO

allthingsoct@gmail.com

Financial Disclosureso Honoraria

n Review of Optometry

n Optometric Management

o Scientific Advisory Boards

n Zeiss

n Zeavision

n Thrombogenics

n Genentech

Financial Disclosures

o Consulting Fees

n Zeiss

n Zeavision

n Maculogix

o Proprietary Interests

n None

o Stockholder: Zeavision

Goals for This Courseo Functional anatomy

review

n Choroid

o Choroid examination and evaluation

o Case examples

o Interactive

Questions?

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The Choroid: Structure, Function, and Evaluation

The Choroido Located between the

sclera and the RPEn Extends from ora serrata

to optic nerveo Pigmented/vascular

tissue .75mm thicko Nourishes the RPE

n Choroiocapillaris designed to leak

o Absorbs light that passes through retina

The Choriodo Loose connective tissueo Melanocyteso Choriocapillaris

n Fenestrated endothelium allows diffusion of proteins

n S__________ regulationn High blood flow n Very little O-2 extracted,

so high venous O-2

BM

CC

Mel.

thicknessRPE

sclera

Bruch’s Membrane o Basal lamina of RPEo Anterior collagenous

layero Elastic layero Posterior collagenous

layero Basal lamina of CC

endotheliumo Contamination of

Bruch’s can result in d________, CNVM

Nourishing the Retinao 2 main sources of blood

supply to retina:o Choroidal BVs

n Supplies outer retinal layers, including PRs

o CRAn 4 branches nourish inner

retinan Run radially toward fovea

• Choriocapillaris

• Sattler’s layer

• Haller’s layer

• Supra - choroid

Choroid Microstructure

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Imaging the Vascular Layers of the Choroid

Imaging the Choroid

Imaging the Choroid-EDI WHAT IS ENHANCED DEPTH OCT IMAGING?

• EDI-OCT• Enhanced-depth imaging (EDI) OCT modifies the standard technique of image acquisition to better reveal the structural details of the choroid.

EDI HOW IS EDI ACHIEVED?

• SD-OCT has a coherence gate of about 2 mm. • Coherence gate is the tissue depth at which

the interference image can be obtained.• An interference signal can be obtained when

the tissue being examined enters the coherence gate.

• However, the signal intensity attenuates in the depth direction, from superficial (retinal) to deep layers (choroid).

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HOW IS EDI ACHIEVED?• Consequently, to obtain high-quality

images in standard SD-OCT, it is important to bring the retinal tissue (B-scan) to the upper aspect of the imaging range.

• In contrast, EDI-OCT creates an inverted mirror image. The reference surface of the inverted mirror image is on the choroidal side.

EDI SHOWS DEEPER INTRAORBITALON, LAMINA, C/S JXN

EDI Indocyanine Green Angiography (ICGA)

o Uses digital imaging systems

o Dye properties

o “Sees” through blood

o Delineates choroidal circulation better than fluorescein angiography

o Boundaries of occult membranes imaged

Questions? Uveal Melanoma

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Manypatients with uvealmelanomahavenosymptoms.

Theirtumorsarefoundduringa"routine"eyeexamination.

Uveal melanoma is the second-most common form of melanoma (skin) and the most common primaryintraocular malignancy.

Up to 50% of patients are at risk for fatal metastatic disease.

Uveal Melanoma

Choroidal melanoma hasanannual incidence of5-6cases per

million people peryear.

Otherthanhavingblueorgreeneyesandalightcomplexion,studieshavenotidentifiedanydefiniteriskfactorsorexposuresthat

predisposepatientstodevelopingthiscancer.

Amelanotic Choroidal

Melanoma

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Presentation ofMelanoma

Asymptomatic

Flashes

Scotoma

VF

defectVitreous

hemorrhage

Pain is rare

Systemically

well

Q: Where does Choroidal melanoma come from?

A: Choroidal Nevus

• Ch Nevusisthemostcommonintraoculartumor

• Proliferationofchoroidalmelanocytes

• Presentin~7.9%ofCaucasians

• Growthisrareafterpuberty?

As we age:

• Nevi increasein number andthickness

• Pigment changes

• Metaplasia

•Drusen/lipofuscinChoroidal Nevus?

• Nevi < 2 mmin thickness (A-scan)

• No known relationshipto sunlight exposure

• Indistinct borders

• May undergo malignantchange into melanoma

Choroidal Nevus?

ChoroidalNevus WorkupandManagement

• Baselinefundusphotography,FAF• OCTiflocationpermits,OCTA?• A/B-Scan• IVFA?

• Yearlydilatedfundusexamination–Ormorefrequent

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qCaucasian

qLightcoloredeyes(blue)

qFairskin

qPropensitytoburnwhenexposedtoUVlight

qCutaneousnevior freckles

q Irisnevi

qWelders

RiskFactorsforMelanoma “Nevoma”

Follow or Co-manage?• T= thickness (>2mm)

• F= subretinal fluid

• S= symptoms

• O= orange pigment

• M= margin touches disk

• No risk factors (<4%)

• 1 risk factor (36%)

• 3 risk factors (50%)

• 5 risk factors (70%)

UsingHelpfulHintsDaily=Ultrasoundhollow,haloabsent,drusenabsent

DOCUMENTEDGROWTH - MEANSEVERYTHING

To Find Small Ocular MelanomaUsing Helpful Hints Daily

7/29/2016

OcularmelanomaCalculator.com

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Echography of Small Choroidal Melanoma

B-Scan EchogramAssess topographic features, including tumor shape, surface contour and boundaries

A-Scan EchogramInternal structure, reflectivity, tumor height (elevation)

EDI-OCT

o Mean small melanoma thickness was 1025 microns on EDI-OCT compared to 2300 microns on ultrasonography.

Enhanced Depth OCT (EDI) of a small melanoma

Shields, 2012

OrangePigment =Lipofuscin

Fundus Autofluorescence (FAF)ofaSmallChoroidalMass

Melanoma

Fundus Autofluorescence (FAF) inChoroidalMelanoma

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Echography ofLargeMelanoma Nevusw/Drusen =Chronicity

HaloNevus = Chronicity NoDrusen,Nohalo

NoDrusen, noHalo Questions?

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Differential Diagnoses

CongenitalHypertrophyofthe

RetinalPigmentEpithelium

• Common, benign lesion

• Focal area in which RPE cells are taller and more densely packed with melanosomes

Familial Adenomatous

Polyposis(FAP)&CHRPE

– ADinheritance

– Adenomatouspolypsthroughoutrectum&colon

– Startstodevelopin

adolescence(15-40yrs)

– Ifuntreated,allpts

willdevelopcolorectalCA >80%ofpatientswithFAPhaveatypicalCHRPElesions

• Breast cancer is the most common CA type to

metastasize to the eye - followed by lung CA

• 85% of patients with breast CA metastases will have a known history of breast CA

• Breast CA metastases tend to be bilateral and multifocal (multiple)

• 40% of these patients have a brain metastasis

MetastaticTumorstotheChoroid

Metastasis from lungto choroid

MetastaticProstateCancer

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METS Melanocytoma• Benignmelanocyticuvealtumor

• Composedoflarge,plumpnevuscellsthatareheavilypigmented

• Canpresentinallagegroupsandraces,thoughmorecommonin• African-Americans• Females

• Patientsareusuallyasymptomatic

• Adjacenttoorwithintheopticnerve

• Blackincolorwithfeatherymargins

• Visualfielddefectmaybepresent

• APDmaybepresent

Melanocytoma CombinedHamartoma oftheRetina andRPE• Greypigmentationwithsuperficialgliosis• Secondaryretinalwrinklingandvesseltortuosity• Lesionscanbejuxtapapillary,peripapillary orwithintheposteriorpole

Absenceofretinaldetachment,hemorrhage,exudationorvitreousinflammation

GeneticTestingonSmallMelanoma

(Shields, 2015)

Treatment &Management

• Enucleation• Radioactiveplaques

• Protonbeamradiotherapy

Mostwidelyaccepted

• Transpupillarythermotherapy

• Localresection

Lesscommon

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Co-manageUvealMelanoma with:

Retina/Ocular Oncology

PCP

General Oncology

Collaborative OcularMelanoma Study

• Organized and funded in 1985 to address issues related to management of choroidal melanoma.

• Main Outcome: overall survival of patient following treatment

• > 4000 patients. 65% pts eligible

Small melanomas < 2.5 mm in height

Medium melanomas 2.5 – 10.0 mm

Large melanomas > 10.0 mm

• Secondary outcomes: metastasis-free

survival, years of useful vision

Plaque left in place for 4 days to provide 8,000 centigray of radiation to entire tumor. The remainder of the body receives a small amount of radiation, about the equivalent of a chest x-ray.

BrachytherapyforUvealMelanoma Treatment SideEffects

• Mainsideeffectoffocaloculartreatmentis… • Radiation retinopathy!

• NVD/NVE• Exudativechanges• Macularedema

• Occursseveralweekstomonthsaftertherapy

Choroidalmelanoma-pre-Radiotherapy

Melanoma pre-Tx echography (left),post-radiotherapy (right)

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RadiationRetinopathy:exudate,NVD

Choroidal Melanoma Pre-TxAcknowledgement: SherrolReynolds, OD, FAAO

S/PRadiotherapy Management ofRR

• Avastin/Lucentis/Eylea• Laser• SiliconeoilattimeofBrachytherapy

–attenuatesradiationdose,andmayprotectagainstradiationretinopathy

Riskfactorsformetastasisfromthechoroid

• Thickness>2mm

• Symptoms– Flashes,floaters,lossofvision

• Proximitytotheopticnerve

• Documentedgrowth

Shields Cl Shields JA. Riskfactors for metastas is of smallchoriodalmelanocytic Les ions .Ophthalmology 1995

MelanomaMetastasis

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OcularMelanoma Quiz

• Whatisthe10-yearmortalityrateforpatientsdiagnosedwithalargeuvealmelanoma?

a.1%b.7%c.9%d.50%

OcularMelanoma Quiz

• Whatisthe10-yearmortalityrateforpatientsdiagnosedwithalargeuvealmelanoma?

a.1%b.7%c.9%d.50%

QuestionsCENTRAL SEROUS

CHORIORETINOPATHY

OCT W/EDI IN CSCPACHYCHOROIDAND SUBRETINAL FLUID IN CSC

CSC MANAGEMENT

• Due to the high likelihood of spontaneous resolution, first line therapy for first time CSC remains risk factor modification (reduce stress, d/c steroids) and observation.

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CSC MANAGEMENT • For CSC that persists or returns:

• In eyes with focal lesions not involving the fovea, focal argon laser treatment may be suitable.

• In eyes with foveal involvement, photodynamic therapy or micropulse diode laser would spare central vision.

• As our understanding of the mechanism of CSC grows, new therapies, such as a spironolactone or eplerenone, may prove to be beneficial.

• Pichi F, Carrai P, Ciardella A, Behar-Cohen F, Nucci P. "Comparison of two mineralcorticosteroids receptor antagonists for the treatment of central serous chorioretinopathy." Int Ophthalmol (2016)

Common Causes of CNV

o Exudative AMDo Ocular Histoplasmosis

o High Myopia

o Angioid Streaks

o Choroidal Rupture

o Chronic CSC (less common)

Fluorescein Angiography (FA)

o FA answers the question: is the blood-retinal barrier intact?

The Fluorescein Angiogramo Stages

n Choroidal phasen Arterial phasen Laminar venous phasen Venous phasen Recirculatory phasen Late phase

CNV in Wet AMD FV Scar

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Causes of CNV

o OHS

48 y/o WM, -12.00D

Concave fundus, CNV, schisis

Causes of CNVo High Myopia in a

52 y/o WM• CNV w/heme

Choroidal Rupture

ANGIOID STREAKS

o Note Angioid Streaks radiating from the optic discs and macular laser scarring

Differential Dx. of Angioid Streaks: PEPSI

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CNV VariantsPolypoidal Choroidal Vasculopathy (PCV)

Retinal Angiomatous Proliferation (RAP)

PCV

PCV RAP

RAP Conclusionso The choroid is among the eye’s most

important, yet ignored tissues.

o It is a high-flow vascular structure that provides nourishment to the outer retina.

o Clinicians should be familiar with the various conditions that affect the choroid, as these can lead to blindness or even

death.

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Thank you!Joe