OTHER ACQUIRED MACULOPATHIES

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OTHER ACQUIRED MACULOPATHIES
1. Central serous retinopathy
2. Idiopathic macular hole
3. Idiopathic premacular fibrosis

• Cellophane maculopathy

• Macular pucker

4. Cystoid macular oedema
5. Myopic maculopathy
6. Choroidal folds
7. Angioid streaks
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Central serous retinopathy ( CSR )

• Self-limiting disease of young or middle-aged
  men

• Usually unilateral

• Localized, shallow detachment of sensory retina
  at posterior pole

• Often outlined by glistening reflex

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FA of central serous retinopathy (1)
Smoke-stack appearance
Later dye passage into subretinal space and
vertical ascend
Subsequent lateral spread until entire area
filled
Early hyperfluorescent spot
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FA of central serous retinopathy (2)
Ink-blot appearance - less common
Early hyperfluorescent spot
Subsequent concentric spread until entire area
filled
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Treatment of central serous retinopathy
Most cases are self-limiting and do not require
treatment
Laser photocoagulation to RPE leak
Post-treatment
Pre-treatment

• 4 months should elapse before considering
  treatment

• Treatment induces resolution and lowers
  recurrence rate

• Does not influence final visual outcome

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Staging of idiopathic macular hole
Stage 1a ( impending )
Stage 1b (occult)
Normal fovea
Stage 1a - ( impending hole )
Stage 1b - ( occult hole )
Dehiscence of photoreceptors
Vitreous contraction with foveal detachment
Stage 2
Stage 3
Stage 4
Seperation of cortex from retinal surface
to form pseudo-operculum
Seperation of pseudo- operculum from edge
of hole
Complete vitreous separation
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Clinical features of full-thickness macular hole

• Typically affects elderly females

• Eventually bilateral in 10

• VA about 6/60

• Round punched-out area at fovea

• Multiple yellow deposits within crater

• Surrounding halo of sub-retinal fluid

• Positive Watzke-Allen sign

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FA of full-thickness macular hole
Hyperfluorescence due to RPE window defect
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Treatment of Macular Hole
1. Indications

• Full-thickness macular hole

• Visual acuity lt 6/18

• Duration lt 1 year

2. Technique

• Vitrectomy and fluid-gas exchange

3. Results

• Closure in about 60

• 40 regain 2 or more lines of VA

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Idiopathic premacular fibrosis
Macular pucker
Cellophane maculopathy

• Opaque epiretinal membrane

• Severe retinal wrinkling and
• vascular distortion

• Translucent epiretinal
• membrane

• May be associated with
• macular pseudo-hole

• Pucker emanating from
• epicentre

• Fine retinal striae and mild
• vascular distortion

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Cystoid macular oedema ( CMO )
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Important causes of CMO
Retinal vein occlusion
Background diabetic retinopathy
Intermediate uveitis
Post-cataract surgery
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Clinical diagnosis of CMO

• Retinal thickening

• Loss of foveal depression

• Multiple cystoid areas

• Yellow spot at foveola

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FA of cystoid macular oedema
Late pooling with flower-petal pattern
Coalescence of leaking points
Early parafoveal leakage
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Treatment Options of CMO
1. No treatment

• Very mild - good VA

• Too early - wait for spontaneous improvement

• Too late - poor VA (lamellar hole)

• Treatment not beneficial - CRVO

2. Laser photocoagulation

• Diabetic retinopathy

• Branch retinal vein occlusion

3. Periocular steroids

• Intermediate uveitis

• Post cataract

4. Systemic carbonic anhydrase inhibitors

• Intermediate uveitis

• Post cataract

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Myopic maculopathy
Atrophic
Lacquer cracks

• Large breaks in Bruch membrane

• Progressive chorioretinal atrophy

• May be associated with macular hole

• Develop in about 5 of highly myopic eyes

Macular haemorrhage
Fuchs spot

• Secondary pigment proliferation

• From CNV with lacquer cracks

• From lacquer cracks alone

• Follows absorption of blood

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Other fundus changes in myopia
Tilted disc
Posterior staphylomas
Peripheral chorioretinal degeneration
Lattice degeneration, holes and retinal
detachment
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Ocular associations of myopia
Cataract
Early onset of nuclear sclerosis
Posterior subcapsular
Glaucoma
Primary open-angle
Pigmentary
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Choroidal folds
Signs
FA

• Alternating hyperfluorescent and
• hypofluorescent streaks

• Parallel, horizontal striae at posterior pole

• Occasionally vertical, oblique or irregular

• Trough is hypofluorescent - blocked
• background fluorescence

• Trough is darker than crest

• Crest is hyperfluorescent - window defect

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Causes of choroidal folds
Bilateral in hypermetropic patients
Thyroid ophthalmopathy
Orbital mass
Severe ocular hypotony
Posterior scleritis
Choroidal tumour
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Angioid streaks

• Bilateral, crack-like dehiscence in Bruch
  membrane

• Secondary changes in RPE and choriocapillaris

• Eventually surround disc

• Linear lesions with irregular
• serrated edges

• Peau dorange mottling of RPE
• particularly temporally

• Radiate outwards from disc

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Ocular associations of angioid streaks
Peripheral focal atrophic salmon spots -
common
Optic disc drusen - uncommon
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FA of angioid streaks
Hyperfluorescence due to RPE window defects over
streaks
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Complications of angioid streaks
Traumatic choroidal rupture
Choroidal neovascularization
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Systemic associations of angioid streaks
Present in approx. 50 of patients
Pseudoxanthoma elasticum
Paget disease
Haemoglobinopathies
(rare)
(most common)
(uncommon)