Early macular signs are usually subtle, be armed with other clinical tools.

1. Acquired macular diseases
Samhaa Mohammed Abd El Moneim
Zagazig Ophthalmology Hospital, 2018
Samhaa Mohammed

2. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED √
Interfacedisorders(ERM,MH,VMT)
CSR
CME
Idiopathic maculartelangectasia
Degenerativemyopia
Angioidstreaks
Choroidalfolds,hypotony maculopathy,solarretinopathy,focalchoroidal
excavation Samhaa Mohammed

3. Macular
assessment
tests
VA
(distant
& near)
Amsler
grid
Contrast
sensitivity
(↓ in ON)
Pupil &
color (↓
in ON)
Photostress
test
Samhaa Mohammed

4. AMD
↓ VA +
AMD
- Drusen
- RPE changes
- GA
Dry
- CNV
- Soft drusen
Wet
Samhaa Mohammed

5. AMD Risk
factors
Age
Race
(white,
caucasian)
Hereditary
(CFH
gene↓)
- Smoking
- Dietary Fat
- HTN,
CVD
- F > M
-
Hypermet
ropia
- Cat.
Surgery
AMD
Samhaa Mohammed

6. Drusen
• Alone Not equal AMD
• Extracellular deposits (lipid, lipofuscin like!) between PRE
& Bruch̛s
RF (drusen convert into AMD):
• Size of drusen (small, intermediate, large)
• Type (hard or soft)
• GA, RPE changes
• Age, FH, other eye
Samhaa Mohammed

7. Drusen
- < 63 mic (<1/2
v width)
- Well defined
- Dry AMD
Small (hard)
- 63-125 mic
Intermediate
- > 125 mic
- One large
drusen ↑risk of
Wet AMD
Large (soft)
Hard Soft Confluent Calcific
DD
Doyne honey comb retinal dystrophy (AD
drusen, Malattia leventine): 40 y
Cuticular drusen (stars in sky): 20y, small,
PED
Type 2 membrano-proliferate GN: older
children Samhaa Mohammed

8. Drusen
Kanski 8th
edition
Samhaa Mohammed

9. AMD
↓ VA +
AMD
- Drusen
- RPE changes
- GA
Dry
- CNV
Wet
Samhaa Mohammed

10. Dry AMD
• Hyperfl: RPE atrophy (window) or late staining
• Hypofl: high lipid content (masking)FFA
• Hyperreflective between RPE & Bruchs membrane.
• Outer retinal layers corrugation: basal laminar (between
RPE & RPE BM), basal linear (between RPE & bruch
inner collagenous layer)
OCT
Prevention (RF, Antioxidant?) Amsler grid
Investigation LVA
AMD work up
Samhaa Mohammed

11. Dry AMD
Small,
intermediate
drusen
Small, intermediate
drusen, pigmentary
changes
Small,
intermediate
drusen, GA
Pigmentary
changes, GA
Samhaa Mohammed

12. AMD
FAF
hyperautofl.
of GA edges
Outer
retinal
tubulation
OCT
Samhaa Mohammed

13. Dry AMD TTT
Indication
Extensive intermediate confluent drusen
One large drusen
GA in one/ both eyes
Late AMD in one eye
Daily TTT regimen/d
Vit E
400 IU
Vit c
500mg
Lutein 10mg
Zeaxanthin 2mg
(instead of B
carotene, vit A)
→ lung cancer in
smokers.
Zinc (25-
80mg)
↓ dose to ↓
SE
(genitourinary
problems)
Copper
(2mg)
With high
zinc dose
Treat
RF
Samhaa Mohammed

14. AMD
↓ VA +
AMD
- Drusen
- RPE changes
- GA
Dry
- CNV
Wet
Samhaa Mohammed

15. CNV
• NVs from choroid → penetrate bruch membrane to retina.
• C/P:
• Acute, subacute or chronic drop of vision.
• Metamorphopsia (amsler chart).
• Associated drusen, cause, haemorhage, scar, subretinal fluid.
• Greyish green, pinkish yellow lesion.
• Poor prognosis.
Type 1
• Sub RPE
Type 2
• Sub retinal
Samhaa Mohammed

16. CNV
causes
Degenerative (AMD, myopia, RAP, PCV,
angioid streaks)
Inflamatory (post uveitis, POHS,
toxoplasmosis)
Trauma (choroidal rpture, extensive
laser)
Tumours (choroidal naevus,
haemangioma)
Samhaa Mohammed

17. Wet AMD
Grey to green
subfoveal CNV
Intra, sub
retinal hge
Extensive lipid
deposition
Disciforrm
scar
Samhaa Mohammed

18. CNV
• Classic: early hyper f ↑ size & intensity, starts well,
ends ill defined.
• Occult: late leakage with undetermined cause.FFA
• Dx: layers disruption, SRF, scarring, RPE & Bruchs
membrane.
• Mointor TTT response.OCT
Samhaa Mohammed

19. Wet AMD
Samhaa Mohammed

20. CNV
TTT regimen
Anti VEGF (main role)
(treat & extend/ m for 3m then
↑ interval duration)
- Avastin, Bevacuzimab (/m)
- Lucentis, Ranbizumab (/m)
- Elyea, Aflipercept (/2m)
Laser
PDT
SE (genitourinary
problems, CU ↓)
Not available in Egypt
• Treatment is effective in active CNV rather than disciform
fibrotic CNV.
• Activity signs (SRF, haemorhage, enlarging membrane,
progressive decreasing vision).
• Follow up with VA, OCT macular thickness.Samhaa Mohammed

21. PED
1. Serous PED:
RPE problem Immune
related!
Pooling
Samhaa Mohammed

22. PED
2. Fibrovascular PED
3. Drusenoid PED
4. Haemorhagic PED
Worst prognosis
(fibrovascular, hgic)
2
4Samhaa Mohammed

23. RPE Tear
IVI, Laser
Loss of dome shape of PED
Crescent shape hypo +
hyper fl.
Rolled up
RPE Dedunded
RPE
Dedunded
RPE
Rolled up
RPE
Corrugated
elevated RPE
Samhaa Mohammed

24. PCV, RAP
PCV
Late middle age, unilateral sudden
visual drop
Multiple serous PED, reddish
nodules
50% have spontaneous resolution
Anti VEGF has lower effect than
CNV
RAP
Stage 1 IRN
Stage 2 SRN → PED
Stage 3 CNV with RCA
FA: similar to occult or
minimally classic CNV
OCT hyper reflective NV
Samhaa Mohammed

25. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED √
Interfacedisorders(ERM,MH,VMT) √
CSR
CME
Idiopathic maculartelangectasia
Degenerativemyopia
Angioidstreaks
Choroidalfolds, hypotony maculopathy,solarretinopathy, focalchoroidal
excavation Samhaa Mohammed

26. ERM
• PVD → glial fibrocellular tissue proliferation → ERM→ macular
pucker.
 Causes:
• Idiopathic
• 2ry to
Vascular (DR, CRVO)
Inflamation (IU, PU)
Trauma
Retinal surgery, laser, cryo
 TTT indication:
• High visual requirements (occupation, young age)
• Duration of visual loss < 6 months
• VA < 6/12
• Well-defined ERM edge
• No associated CME
PPV +
peeling
Samhaa Mohammed

27. ERM
Advanced
membrane
ERM ERM in OCT
pseudoholeSamhaa Mohammed

28. VMT
ERM
Viteomacular adhesion
Vitreomacular traction
Samhaa Mohammed

29. MH
• ERM, VMT …. MH
• Unilateral, female, old age
• FTMH
• Lamellar MH
• Pseudo hole
• Investigation:
• Amsler grid: scotoma.
• Watze Allen sign: broken slit beam
• OCT: definite dx
• FFA: window defect in late stage (RPE atrophy)
• FAF: hyper autoflurescenceSamhaa Mohammed

30. MH
Stage 1a
Impending MH
Photoreceptors detach from inner
retina, schesis
Stage 1b
Occult MH
Photoreceptors with centrifugal
displacement
Stage 2
Small FTMH
FTMH <400 mic
Stage 3
Large FTMH
FTMH > 400mic without Complete
PVD
Stage 4
Large FTMH
FTMH > 400 MIC
Complete PVD
Samhaa Mohammed

31. MH
Kanski 8th
edition
Samhaa Mohammed

32. MH
TTT options
• Observation (50% of stage 1
spontaneously closed).
• PPV with ILM peeling + gas
tamponade (face down).
• Vitreolysis with ocriplasmin (in
AP or equatorial traction).
Indication
• FTMH (success 90% in stage 2).
• High visual needs.
• Young age.
• < 6m.
• Decrease vision of the
other eye.
Examine the other eye: MH of ther eye will be 1% if PVD, 10% if no PVD.Samhaa Mohammed

33. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED √
Interfacedisorders(ERM,MH,VMT) √
CSR √
CME
Idiopathic maculartelangectasia
Degenerativemyopia
Angioidstreaks
Choroidalfolds,hypotonymaculopathy,solarretinopathy,focalchoroidal
excavation Samhaa Mohammed

34. CSR
• RPE pump dysfunction → loss of outer RBB.
• Unilateral VA 6/12 (impove with weak plus lens)/ or biateral.
• Micropsia.
• Patchy RPE atrophy/ hyperplasia, NSD (in chronic CSR, after
resolution).
 Course :
• Spontneous resolution: in 80% within 6 m
• Recurrence : in 50%
• Chronic or multiple recurrence: RPE, photoreceptors degeneration
 investigation:
• OCT: NSR elevation, RPE changes, associated RPE detachment.
• FFA: ink plot, smoke stack hyperfuorescence.
• Amsler grid
Samhaa Mohammed

35. CSR
Resolving
CSR
FAF
scarring
CSR, PED
Chronic
CSR
Samhaa Mohammed

36. CSR
Smoke stack
Ink blot
CSR,
PED
Samhaa Mohammed

37. CSR
TTT :
• Observation.
• Stop steroid, modify patient life style.
• Micropulse laser: to RPE at site of leakage to speed fluid absorption
only.
• Argon laser: < 10 burns, 0.1 sec., 50-200mic, mild burn at leakage site
• Recently, anti-aldesterone; Emiplerone (tensoplerone): 25mg tab
(twice/d) has a promising effect over > 6m.
• Intervention TTT indication:
• High visual requirement
• Persistent leakage > 6m
• Recurrence with decrease in vision
• Fellow eye with CSR and decrease vision
Samhaa Mohammed

38. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED √
Interfacedisorders(ERM,MH,VMT),MH√
CSR √
CME√
Idiopathic maculartelangectasia
Degenerativemyopia
Angioidstreaks
Choroidalfolds,hypotony maculopathy, solarretinopathy,focalchoroidal
excavation Samhaa Mohammed

39. CME
• Lost inner BRB, inflamatory or mechanical factors.
• Fluid accumuation in OPL, INL → cyst-like spaces → loss of
mullers cells and cyst coalescence → MH.
• Investigation:
• OCT: intraretinal cysts, retinal thickening.
• FFA: flower petal appearance hyperfuorescence.
• TTT:
• According to the cause.
Samhaa Mohammed

40. CME
Samhaa Mohammed

41. CME
• Laser
• Anti VEGF, steroid IVI
Vascular(PDR, RVO)
• Systemic steroid, immunesuppressiveInflammatory (PU, IU)
• CAI (systemic), NSAIDS (oral, systemic)Surgical (Irvine-Gass),
laser
• PPV+ relief of tractionVMT, ERM
• Drug cessationDrug induced (PG)
• CAIRetinal dystrophies (RPE)
Samhaa Mohammed

42. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED √
Interfacedisorders(ERM,MH,VMT),MH√
CSR √
CME√
Idiopathic maculartelangectasia√
Degenerativemyopia
Angioidstreaks
Choroidalfolds,hypotonymaculopathy,solarretinopathy,focalchoroidal
excavation Samhaa Mohammed

43. Idiopathic macular telangectasia IMT,
MacTel
1. Aneurysmal telangectasia (Leber miliary aneurysm):
• Like coats disease, middle age male.
• Unilateral cysts, early temporal central then peripheral. Mild ↓ VA
• FFA: leakage (CME, Tel, microaneurysm)
• OCT: CME, ERD, thickening, photoreceptors disruption, hyperreflective Tel,
pigment clumbs
• FAF: loss of foveal hypoflurescence then hyperreflectivity
2. Perifoveal telangectasia:
• M=F, bilatral, like RAP, more common, worse than type 1 & better than CNV
3. Occlusve telangectasia:
• According to the cause, poor prognosis, occlusion of parafoveal capillaries with
aneurysmal dilatation of terminal ones. The worst
Early detection
(red free light,
FAF)
Aneurysmal
Tel
Perifoveal
Tel
Occlusive Tel 2ry CNV
Samhaa Mohammed

44. Idiopathic macular telangectasia IMT,
MacTel
Early type 1
with red free
FA
microaneurysm
FA
Early phase
FA late leakage
Samhaa Mohammed

45. Idiopathic macular telangectasia IMT,
MacTel
Type 2 greyish loss of
parafoveal temporal
transparency
Crystals in
early type 2
TEL
Pigment
plaques
OCT subfoveal
cyst
Samhaa Mohammed

46. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED√
Interfacedisorders(ERM,MH,VMT)√
CSR√
CME√
Idiopathic maculartelangectasia √
Degenerativemyopia
Angioidstreaks
Choroidal folds, hypotony maculopathy,solarretinopathy, focalchoroidal
excavation Samhaa Mohammed

47. Degenerative myopia
Systemic
association
Down ROP
Stickler Ehler Danlos
Marfan Pierre Robin
High myopia: > -6pd, AL > 26mm.
Pathological myopia: degenerative progressive ++ of AP
diameter with 2ry ocular changes 2ry to mechanical stretch.
Samhaa Mohammed

48. Degenerative myopia
Dx
Tasellated (tigroid) fundus Coin shaped hge
Chorioretinal atrophy CNV, Fuchs spot
ON head anomaly (tilted,
PPA)
Post staphyloma
Lattice degeneration Intrachoroidal excavation
Lacquer cracks RRD, retinoschesis, MH
Samhaa Mohammed

49. Degenerative myopia
Tessellated
Chorioretinal
atrophy
Tilted disc,
focal atrophy
Lacquer
cracks
Samhaa Mohammed

50. Degenerative myopia
Coin
shaped hge
Fuchs
spot
Post staphyloma
in axial CT
Post staphyloma
in OCT
Samhaa Mohammed

51. Degenerative myopia
Dome shaped
macula with
foveoschesis
Intrachoroidal
excavation
Intrachoroidal
excavation
Macular hole
Samhaa Mohammed

52. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED √
Interfacedisorders(ERM,MH,VMT),MH√
CSR √
CME√
Idiopathic maculartelangectasia √
Degenerativemyopia√
Angioidstreaks√
Choroidalfolds,hypotony maculopathy, solarretinopathy, focalchoroidal
excavation Samhaa Mohammed

53. Angioid streaks
• Thickenned brittle brucks membrane leads to cracks in it.
• Grey linear, irregular serrated edges from OD, may
intercommunicate.
• Peau d̛orange fundus.
• OD drusen.
• Complication: CNV, choroidal rupture(trivial trauma).
• Investigation:
• Red free photo, FAF: demonstrate streaks than clinical ex.
• FFA: window defect, or hypo fl (RPE hyperplasia).
• TTT:
• Prevention
• CNV
Samhaa Mohammed

54. Angioid streaks
OD drusen Peau d̛orangeSamhaa Mohammed

55. Anigiod streaks
• Thickenned brittle brucks membrane leads to cracks in it.
Systemic
association
(PEPSI)
Pseudoxanthoma elasticum (elastic fibers,
skin, eye, CVS)
Ehler Danlos (Collagen, AD)
Paget disease (Ca deposition, bone fracture, eye)
Sickle cell disease
Idiopathic
Samhaa Mohammed

56. Acquired
macular
diseases
AMD(dry&wetCNV)√
RAP,PCV,PED√
Interfacedisorders(ERM,MH,VMT)√
CSR√
CME√
Idiopathic maculartelangectasia √
Degenerativemyopia√
Angioidstreaks√
Choroidalfolds,hypotony maculopathy, solarretinopathy,focalchoroidal
excavation√ Samhaa Mohammed

57. Choroidal folds
• Scleral & choroidal compression.
• Causes:
• Idiopathic: high hyperopia (exclude ICT)
• Papillodema
• Orbital diseases: TED, IOID
• Ocular causes: post scleritis, choroidal tumours, hypotony, posterior
uveitis, buckle
• Investigation:
• OCT: differentiate retinal, chorioretinal, choroidal folds
• FAF: demonstrate folds
• FFA: hyperfl CRESTS through stretched RPE, hypofl TROUGH through
compressed corrugated RPE
• CT, MRI: cause
Samhaa Mohammed

58. Hypotony maculopathy
Hypotony
maculopathySamhaa Mohammed

59. Solar maculopathy
• Photochemical retinal injury due to prolonged unprotected gaze
at the sun.
• ↓ VA, returns over the course of months.
• Small yellow to white foveal spot.
• OCT: well-defined defect at IS/OS photoreceptor layer (outer
retinal microhole!).
• Good prognosis.
Samhaa Mohammed

60. Focal choroidal excavation
• Bilateral, middle age in eastern asian, no Hx of ocular disease.
• Variable vision, ↓ with CNV, PCV, CSR formation.
• OCT: IS/OS follow outward indentation of excavation (Conforming
FCE), but seperation of it from RPE (Non-Conforming of FCE).
Conforming
FCE
Non-
Conforming
Samhaa Mohammed