Acquired Macular Disease

1. AMD

2. angioid streaks

3. choroidal osteoma

4. fundus flavimaculatus

5. multifocal choroiditis

6. ocular histoplasmosis syndrome

7. optic disc drusen

8. ONH pits

9. pathological myopia

10. pattern dystrophies

11. photocoagulation

12. polypoidal choroidal vasculopathy

13. sarcoidosis

14. serpiginous or geographic choroiditis

15. toxoplasmic retinochoroiditis

16. traumatic choroidal rupture

what are common ophthalmic conditions associated with macular neovascularization?

1. demographics

2. age

3. male vs female

4. race/ethnicity

what are ways to differentiate between the various causes of macular neovascularization?

angioid streaks

• irregular, radiating, jagged, tapering line that extends from the peripapillary area into the peripheral fundus

• results from changes in the collagenous & elastic portion of Bruch’s which renders it weak & predisposed to breaks

• reddish orange to dark red or brown

• CNVM can grow through into the sub-RPE or subretinal space in/near papillomacular region

• may have significant underlying systemic disease

1. pseudoxanthoma elasticum

2. Ehler’s Danlos

3. Paget’s

4. sickle cell

5. idiopathic

6. homocysteinuria

7. acromegaly

8. Marfan’s syndrome

what systemic conditions are angioid streaks associated with?

pathological myopia (myopic macular degeneration)

• in myopes greater than -6.00D

• slow progressive vision loss usually in 5th decade or later

• clinical appearance:

  • scleral crescents

  • oblique nerve insertion

  • PPA

  • macular pigment mottling

  • posterior staphyloma

  • well circumscribed area of atrophy

  • lacquer cracks

  • risk of CNVM

  • Fuch’s spot

Fuch’s spot

raised, circular pigmented lesion ~1/2 DD in size, consisting of localized ingrowth of fibrovascular tissue, associated w/ pathological myopia

lacquer cracks

• spontaneous focal linear breaks in Bruch’s secondary to high or progressive/pathological myopia

• may have associated small, subretinal hemorrhage unassociated w/ evidence of CNVM

• felt to be caused by stretching of retina & choroid, pathological myopia

• begins as thinning of choroid & RPE in macular area

choroidal ruptures

contusion & rupture of the choroid & retina caused by high-velocity trauma, usually from blunt trauma to the globe, sclera may be visible w/in the break

pattern macular dystrophy

• adult onset

• BILATERAL

• autosomal dominant

• typically begins midlife w/ visual disturbance

  • typically retain good vision in 1 eye until late adulthood

• appearance:

  • deposits can range in color

  • patterns can vary widely & be asymmetric

• good visual prognosis for all groups

• rarely does CNVM develop

adult vitelliform pattern macular dystrophy

• group of pattern macular dystrophies

• can be asymmetrical

• lesions can fade & pigment

butterfly shaped

• group of pattern macular dystrophies

• triradiate pattern

• zone of depigmentation around deposits

multifocal pattern macular dystrophy

• group of pattern macular dystrophies

• yellow scattered flecks

• normal choroidal fluorescence

• better prognosis for vision

1. later onset than Stargardt’s

2. normal choroidal fluorescence

3. better prognosis

how do you differentiate between multifocal pattern simulating Stargardt’s macular dystrophy & Stargardt’s?

reticular pattern macular dystrophy

• group of pattern macular dystrophies

• fishnet pattern centered on fovea extending in all directions

• better viewed w/ IVFA

• can fade over time

angioid streaks

angioid streaks

angioid streaks

angioid streaks

large CNVM & leaking

angioid streaks

CNVM causing leaking of fluid

angioid streaks

pigmented angioid streaks

angioid streaks, CNV & subretinal hemorrhage associated

angioid streaks

pathological myopia

lacquer cracks

lacquer cracks

lacquer cracks

lacquer cracks & subretinal hemorrhage

lacquer cracks

lacquer cracks

lacquer cracks

choroidal ruptures

choroidal rupture & CNV

choroidal rupture

choroidal rupture

adult vitelliform pattern macular dystrophy

butterfly pattern macular dystrophy

butterfly pattern macular dystrophy

butterfly pattern macular dystrophy

Stargardt’s like pattern dystrophy

polypoidal choroidal vasculopathy

• idiopathic, primary disorder of inner choroidal vasculature

• likely represents a form of CNV

• inner choroidal vascular network w/ aneurysmal bulge

• appearance:

  • visible orange, spheroid, polyp-like structure

  • multiple, recurrent serosanguineous detachments of the RPE & NS retina

  • usually bilateral

• better visual prognosis

• preponderance in heavily pigmented races

• age of onset is much younger than in AMD

pachychoroid

• attenuation of choriocapillaris overlying dilated choroidal veins

• associated w/ progressive RPE dysfunction & neovascularization

• thickened vessels that do not taper toward posterior pole, may be diffusely present or focal in nature

• choriocapillaris thinning observed over thick vessels

greater than 300microns subfoveal

what constitutes a thick choroid?

Haller’s

in pachychoroid, the choroid is thick due to dilation of choroidal vessels in _____ layer

choroidal hyperpermeability → choriocapillaris attenuation, RPE complications, & neovascularization

what is the pathogenesis of pachychoroid?

idiopathic central serous chorioretinopathy (ICSC)

• localized serous detachment of the retina in macular area

  • sloping margins

  • small RPE detachment

• no blood or exudates

• age of onset: mid 30s

  • range: 20-50yo

• men > women

• whites > non-whites

• seen in type A personalities

• relatively good prognosis (majority spontaneously recover in 4-8wks)

idiopathic but part of pachychoroid disease spectrum

what is the pathophysiology of ICSC?

1. unilateral, central vision loss or distortion or blur to moderate degree

2. VA may be near normal w/ a hyperopic shift (20/20 - 20/80)

3. micropsia

4. color vision complaints (blue-yellow)

5. decreased contrast sensitivity

6. poorer macular photostress recovery time

7. positive central scotoma or dark spot on Amsler testing

what are the signs/sx of ICSC?

3rd

if a pregnant pt has an ICSC, typically there is recovery in the ___ trimester

1/3 to 1/2

_______ of pts w/ ICSC have a recurrence

50

____% of pts that have an ICSC recurrence have it w/in the 1st year

10

__% of ICSC pts have a third episode

20

__% of ICSC pts become bilateral

5

less than __% of ICSC pts develop a CNVM

smoke stack

what is the characteristic appearance of ICSC on IVFA?

laser photocoagulation

what is the tx for ICSC?

RPE defect is >1/4 DD from foveal center &

1. greater than 4-6mo duration

2. pt has occupational considerations

3. turbid sub-retinal fluid

4. severe bouts of recurrence

5. bilateral cases w/ a defect in the fellow eye

when should laser tx be considered for ICSC?

no

can you tx ICSC w/ steroids?

1. aldosterone antagonist agents (spironolactone, eplerenone)

2. CAIs

3. tx of H. pylori (questionable)

what drugs can you use to manage ICSC?

vitreal macular traction

• traction on retinal surface & macular distortion

  • incomplete PVD

  • macular cysts

  • can progress to macular hole

• central vision complaint

• may spontaneously resolve/detach

• vitrectomy indicated if persistent & symptomatic

idiopathic age-related macular hole

• circular or oval depression in the central macula

• varying in size

• round, well-defined red area w/ surrounding gray halo of detached retinal elevation

• 50% have yellow nodular deposits at the level of the RPE (bread-crumb)

• 10-20% of pts have ERM

• operculum structure often visible

• age of onset: 6th-8th decade

• females > males

1. blur/metamorphopsia when other eye is covered

2. mean VA: 20/200

3. + Watzke sign

what are the signs/sx of idiopathic macular hole?

stage 1

• idiopathic macular hole stage

• loss of normal foveolar depression & FLR

• localized contraction of prefoveal vitreous

• yellow spot or ring may be apparent

• no tx recommended at this stage

  • 50% resolve spontaneously

stage 2

• idiopathic macular hole stage

• partial/early macular hole formation w/ a full thickness opening

stage 3

• idiopathic macular hole stage

• full development of macular hole

stage 4

• idiopathic macular hole stage

• macular hole & PVD

10

there is a ___% chance of bilaterality (usually w/in 2y) if PVD is not present in fellow eye

0

what is the chance of an idiopathic macular hole becoming bilateral if there is a PVD present in the other eye?

vitrectomy w/ fluid-gas exchange

what is the tx for an idiopathic macular hole?

polypoidal choroidal vasculopathy

polypoidal choroidal vasculopathy

polypoidal choroidal vasculopathy

ICSC

ICSC

ICSC

ICSC

ICSC

ICSC

ICSC

VMT

VMT

VMT

macular hole

macular hole

macular hole

stage 1 macular hole

stage 1 macular hole

stage 2 macular hole

stage 2 macular hole

macular hole

macular hole

macular hole

full thickness macular hole

full thickness macular hole

macular hole

macular hole

macular hole

macular hole