11 - Macular Disorders

denerative myopia
angioid streaks
choroidal rupture
torpedo maculopathy

What are some retinal/macular disorders that cause CNV?

high myopia = axial elongation = biomechanical forces = progressive stretching and thinning of RPE, choroid, sclera

What is degenerative myopia?

myopia > -6.00D

axial length > 26.5mm

What amount of myopia and/or axial length is typically high enough for degenerative myopia?

posterior staphyloma = posterior elongation of the eyeball = progressive scleral thinning

What is the primary alteration seen in degenerative myopia?

malinserted ONH

PPA

posterior staphyloma = outpouching of sclera

vitreous degeneration

cobblestone degeneration

retinal degeneration

RD

retinal holes/tears

lattice

subretinal neo/small CNVM

Fuch's spot = hyperpigmentation

lacquer cracks = healed breaks of RPE, Bruch's choriocapillaris

macular schisis

What are some of the possible findings/appearance of degenerative myopia?

posterior staphyloma = outpouching of globe

What findings of degenerative myopia are seen here?

lacquer cracks in macula

Fuch's spot in the macula

malinserted ONH

scleral cresent

What findings of degenerative myopia are seen here?

lacquer cracks in macula

posterior staphyloma

malinserted ONH

scleral cresent

subretinal heme = possible CNV

What findings of degenerative myopia are seen here?

none
reduced VA
scotoma
metamorphopsia
F/F
loss of side vision

What are some symptoms of degenerative myopia?

develops in youth = progressive visual decline in 40% of pt's

What is the evolution of degenerative myopia?

CNV = can be treated

macular atrophy = cannot be treated = LV referral

holes/tears/RD = can be treated

What are some complications of degenerative myopia? Which can be treated vs cannot?

type 2 (classic) = above RPE

smaller in size than other conditions

flat, small, greyish subretinal lesion at or near fovea +/-heme

What are some features of the CNV seen in degenerative myopia?

hyper-reflective elevated lesion in subretinal space (usually without much edema, exudate, subretinal fluid)

What are some OCT features of the CNV seen in degenerative myopia?

lacy wheel pattern = numerous tiny capillaries

What are some OCTA features of the CNV seen in degenerative myopia?

monitor annually for complications

caution against trauma and using protective eyewear

LV referral if reduced BCVA

retina referral if CNVM, RD, etc

What is the management for degenerative myopia?

pathological changes in RPE, Bruch's, choriocapillaris complex = RPE migration or loss = breaks in a thickened or calcified Bruch's

What are angioid streaks?

bilateral

Are angioid streaks typically unilateral or bilateral?

outward spoking lines of circumpapillary degeneration with an irregular jagged red, brown, gray appearance

Describe the appearance of angioid streaks.

none early on
reduced VA

What are some symptoms of angioid streaks?

leakage of blood through breaks

OR

CNV grows through breaks in 40s/50s = visual impairment in 70% of pt's

What is the evolution of angioid streaks?

CNV

disciform scar

What are some complications of angioid streaks?

Caucasians

systemic PEPSI diseases in 50% of cases

disc drusen

peau d'orange = orange skin

What are some risk factors/associations for angioid streaks?

pseudoxanthoma elasticum

ehlers danlos

paget's disease

sickle cell anemia

idiopathic

What are the PEPSI systemic conditions that are associated with angioid streaks?

if dark and red, can often look a lot like BV

What can angioid streaks sometimes look like?

hypo autofluorescent, but not as dark/dense as BV

How do angioid streaks appear on FAF?

monitor annually and with home Amsler for complications

R/O PEPSI systemic conditions if not already diagnosed

caution against trauma and using protective eyewear

What is the management for angioid streaks?

congenital macular lesion

What is torpedo maculopathy?

unilateral

Is torpedo maculopathy typically unilateral or bilateral?

single, flat, hypopigmented torpedo-shaped lesion temporal to macula

+/- hyperpigmented tail

What is the appearance of torpedo maculopathy?

asymptomatic, no affect on VA
scotoma possible

What are the symptoms of torpedo maculopathy?

congenital

stable over time

What is the evolution of torpedo maculopathy?

rarely CNV can develop

What are some complications of torpedo maculopathy?

females

Are females or males more at risk for torpedo maculopathy?

monitor annually for CNV

What is the management for torpedo maculopathy?

trauma = eye is compressed and stretched along the horizontal axis = Bruch's, RPE, choriocapillaris tear due to lack of elasticity in these tissues

What is choroidal rupture? Include the main cause.

lesions concentric to ONH with RPE migration and hyperplasia

+/- hemes in various layers

What is the appearance of a choroidal rupture?

reduced VA if involving macula

What are some symptoms of choroidal rupture?

remains stable

What is the evolution of choroidal rupture?

mac edema

CNVM

What are some possible complications of choroidal rupture?

monitor annually for complications like CNV

What is the tx for choroidal rupture?