Macular Disorders
CME
CSCR
ARMD
SOLAR RETINOPATHY
Cystoid Macular Edema
(CME)
Definition
• Fluid accumulation in the outer plex. Layer of
the macula
Causes
• Commonest cause is cataract surgery
Other causes
Irvine Gas Syndrome
• 60% uncomplicated cases ICCE have CME
• 20% uncomplicated cases ECCE have CME
• Vision drop in modern cataract surgeries is 0.2 – 1.4 %
because of CME
• Most cases occur 4 – 12 weeks post-op. some years later
• 75% resolve on their own
Mechanism of post-op. CME
• Damage to retinal vascular endothelium
(specially in perifoveal capillary net)
• Most important cause
• Intraocular inflammation
• Other causes
• VMT
• Vascular compromise
• Prostaglandin secretion
Diabetics with any degree of
retinopathy have a higher
incidence of post-cataract CME
YAG Capsulotomy
• Before 3 months incidence of CME goes up
• After 3 months no effect on CME
Signs & Symptoms
• Vision drop (6/9 – 6/60 or less)
• Distortion in vision
• Mild circum corneal congestion
• Mild pain, irritation and watering
On examination
• Just a yellow spot on the fovea
• Cystoid spaces with 90 / 78 D lens
Important Ancillary Findings
• Vitreous in the A.C
• Peaked pupil
• Vitreous or capsular strand in the wound or
side port
• A.C.Ls in general
• Cells in A.C. and vitreous
• Hypermic disc
Confirmation
• OCT
Confirmation
• FFA
Histology
• Cystic spaces in the outer plex. Layer
• Electron microscopy shows intra cellular fluid
accumulation (reversible)
• When excessive intra cellular fluid breaks
through cell membrane becomes extra
cellular (irreversible)
Prevention
• Topical NSAIDs (cyclo oxygenase inhibitors)
e.g. Ketorolac (Acular)]
• 2 days pre-op.
• 6 weeks post-op.
• Along with steroid drops
Treatment
• Steroid drops
• Pred Acetate 8 /D x 3 weeks
• If no tension rise (steroid response) then
• Subtenon Kenacort
• If still no response
• Oral steroids
Treatment
• NSAIDs
• Flur 3-4 /d x 2 months
• Acular 3-4 /d x 2 months
• Tab. Diamox 250mg bid x 1 month
• Hyperbaric O2
Surgical Treatment
• YAG to capsular or vitreous strands
• Vitrectomy
CSCR
• Central serous chorioretinopathy (CSCR) is an idiopathic
disorder characterized by a localized serous detachment of the
sensory retina at the macula secondary to leakage from the
choriocapillaris through focal, or less commonly diffuse,
hyperpermeable RPE defects.
• CSCR typically affects one eye of a young or middle-aged
Caucasian man; women with CSCR tend to be older.
• Imperfectly defined additional risk factors include
psychological stress, type A personality, steroid administration,
Cushing syndrome, systemic lupus erythematosus and
pregnancy.
Clinical features
•
• 1 Presentation is with unilateral metamorphopsia that may be associated with
micropsia, mild dyschromatopsia and decreased contrast sensitivity.
2 VA is usually reduced to 6/9–6/18, but often correctable to 6/6 with a weak
convex lens, because elevation of the sensory retina gives rise to an acquired
hypermetropia.
3 Signs
• A round or oval detachment of the sensory retina is present at the macula
• The subretinal fluid may be clear (particularly in early lesions), turbid or
fibrinous, and precipitates may be present on the posterior retinal surface.
• One or more abnormal depigmented RPE foci (sometimes small PEDs) of
variable size may be visible within the neurosensory detachment.
• Small patches of RPE atrophy and hyperplasia elsewhere in the posterior
pole may indicate the site of previous lesions.
• The optic disc should be examined to exclude a congenital pit .
Investigations
• 1 Amsler grid confirms metamorphopsia corresponding to the
neurosensory detachment.
2 OCT shows an optically empty neurosensory elevation (Fig.
14.57B). A RPE detachment or a deficit in the RPE may also be
seen.
3 FA may show the following patterns: • 'Smokestack’
manifests as an early hyperfluorescent spot that progresses to
form a vertical column by the late venous phase (Fig. 14.58B),
followed by diffusion throughout the detached area.
• ‘Ink blot’ (most common) shows an early hyperfluorescent
spot that gradually enlarges .
• An underlying PED may be demonstrated.
• Multiple leakage points or diffuse leakage can be evident,
particularly in chronic or recurrent disease.
4 ICGA. The early phase may show dilated or compromised
choroidal vessels at the posterior pole. The mid-stage shows areas
of hyperfluorescence due to choroidal hyperpermeability
Course
• 1 Spontaneous resolution occurs in most patients within 3–6
months, with return to near-normal or normal vision in more than
80% but recurrences occur in up to 50% of cases.
2 Chronic course lasting more than 12 months constitutes a
minority and typically affects older patients. • Prolonged
detachment is associated with gradual photoreceptor and RPE
degeneration with resultant visual impairment.
• Multiple recurrent attacks may also give a similar clinical
picture.
• FA shows granular hyperfluorescence with one or more leaks
(Fig. 14.59).
• CMO, CNV or RPE tears may develop in a minority of cases.
3 Bullous CSCR is characterized by large, single or multiple,
serous retinal and RPE detachments that should not be
misdiagnosed as rhegmatogenous retinal detachment or exudative
detachment from another cause.
Management
1 Observation is appropriate in most cases.
2 Discontinue any corticosteroid treatment if possible,
particularly in chronic, recurrent or severe cases.
3 Lifestyle change to reduce stress in selected cases.
4 Laser photocoagulation to the RPE leak speeds resolution
but does not influence the final visual outcome or recurrence
rate. It is advisable to wait at least 4 months before considering
treatment of a first attack, and 1–2 months for recurrences.
Thermal laser treatment is probably inadvisable if the leak is
within the FAZ. Two or three low-intensity burns (200 µm, 0.2
second) are applied to the leakage site (Fig. 14.60) to produce
mild greying of the RPE.
5 PDT may be considered in subfoveal leaks or chronic
disease. Only 30% of the dose of verteporfin used for CNV, in
conjunction with 50% light intensity is used by some authorities
as first-line treatment.
6 Intravitreal anti-VEGF agents show some promise
AMD
• AMD is the leading cause of vision drop over
age 50, in developed countries
• In the USA 8 million suffer from the
‘intermediate’ form of AMD
What is the intermediate form of AMD ?
• Numerous mid sized drusen or one large
drusen. These patients are at a high risk for
advanced AMD
What is ‘Advanced’AMD ?
• Geographic AMD involving the fovea
• Neovascular ( wet ) AMD
Risk factors
• Advancing age
• Genetic predisposition
• Smoking
• Race / Hypertension
• 85% cases of AMD have the dry form and
only15% exhibit the wet form of AMD
• Geographic atrophy is defined as one or more
patches of RPE atrophy ( choroidal vessels exposed),
each patch at least 175 microns in diameter.
• Dry atrophy can progress to wet AMD with macular
scarring and atrophy with irreversible vision loss if
untreated.
Role of Micronutrients
• The AREDS study showed that antioxidants
and micronutrients slowed the progression of
dry AMD to only a ‘modest’, though
statistically significant level.
• They are therefore recommended in all
intermediate & advanced cases of AMD.
Diagnosis
• Symptoms : painless, progressive blurring and
distortion of vision, with central scotomas
(missing letters or words) Onset can be acute
or insidious.
• Signs : Drusen, RPE changes, atrophic
patches, PEDs with drusen, SRF, SR h’age.
Lipid exudates
FFA
• To confirm diagnosis
• Evaluate location
• Classify the lesion
location
• Sub foveal
(Directly under the foveola)
• Juxta foveal
( 1 to 199 mic.from the foveola)
• Extra foveal
(200 to 250 mic from foveola, amenable
to thermal laser)
.
Composition
Predominantly classic CNVM : Hyperfluorescent lesion in early frames,
fuzzy leakage in late frames, more than 50% of lesion is classic.
Occult with no classic CNVM : a) late leakage from undetermined source
( speckled, irregular leakage in late frames , no distinct starting point in
early pictures)
b) Fibrovascular PEDs with stippled
hyperfluorescence and leakage in late frames.
Minimally classic CNVM : Contains both classic and occult CNVM but
classic element is less than 50% of the total lesion.
Fibrovasc. PED
Implication
• Predominantly classic , sub foveal lesions spread
rapidly with marked vision drop.They need urgent,
early treatment.
• The other two can remain stable without vision drop
for more than one year. They can be observed if they
have not shown progression in the last 3 months.(i.e
no blood/ no vision drop/ no increase in lesion size)
Additional investigations
ICG : It is of little use in AMD. Useful to r/o
IPCV when in doubt.
OCT : Typical picture is of fusiform
thickening of RPE,Bruch’s,Chorio
cap. complex with a PED and SRF.
Very useful for monitoring treatment
response.
OCT of CNVM
Management options
• Thermal laser ( Argon green/double freq.Yag).
Reserved for small, well demarcated,
extra foveal lesions.
Recurrence rate upto 5 yrs. 54%.
Max. recurrence is between 4 wks and
one year.
(Mngmt – re laser /anti VEGF)
PDT (Photo Dynamic Therapy)
• Targets CNV without damaging overlying
neurosensory retina.
• I/venous Verteporphin collects in CNVM & is
activated by light ( non thermal laser), release of free
radicals, endothelial damage & thrombosis of
abnormal CNV vessels.
• Useful for predom. classic sub and juxta foveal
CNVMs. Not so
good for occult or min. classic lesions.
• There is no gain in visual acuity.
ANTI VEGFs
• Pegaptanib (Macugen) :
Delivered intravitreally. It is specific against
VEGF 165.( There are 6 known isoforms of VEGF that
promote neo vasc. in response to hypoxia. 165 is most
responsible for ocular neo vasc.)
Macugen slows the disease but does not
improve vision.
• Lucentis (Ranibizumab)
Broad spectrum anti VEGF, active against all isoforms.
It stops progress of CNV in 94 to 96 % of cases and
actually increases vision in 35 to 40 % of cases.
• Avastin (Bevazicumab):
(Off label use in Ophthalmology )
-As effective as Lucentis
-Much cheaper
-Undergoing ATT trial (AMD treatment trial)
to determine if better, as good or worse than
Lucentis.
Side effects
• Endophthalmitis 1.3 %
• Injury to lens 0.6 %
• Ret. Detachment 0.7%
• (Safety concerns about strokes and Myocardial inf. are not statistically
significant )
Combination therapies
• ¼ fluence PDT +Lucentis+Dexameth.
• ½ fluence “ “ “
• ½ fluence PDT +Lucentis only
• Only Lucentis
All showed almost similar visual results, but
the number of injs. was reduced.
Drugs in development
• VEGF Trap Eye, similar to Lucentis
• Role of inflam. in CNV is under investigation
and complement is being targeted.
• Strontium 90 beta radiation along with Anti
VEGF.
• As of today the first line of defence against
Neovascular (wet) AMD is
LUCENTIS ( AVASTIN )
SOLAR RETINOPATHY
1 Pathogenesis. Retinal injury is caused by photochemical effects of
solar radiation by directly or indirectly viewing the sun (eclipse
retinopathy).
2 Presentation is within 1–4 hours of solar exposure with unilateral
or bilateral impairment of central vision and a small central scotoma.
3 VA is variable according to severity.
4 Fundus • A small yellow or red foveolar spot which fades within
a few weeks (Fig. 14.71A).
• The spot is replaced by a sharply defined foveolar defect with
irregular borders (Fig. 14.71B) or a lamellar hole.
5 OCT shows foveal thinning with a focal hyporeflective area, the
depth of which correlates with the extent of visual acuity loss but which
generally includes the photoreceptor inner and outer segments.
6 Treatment is not available.
7 Prognosis is good in most cases with improvement of visual acuity
to normal or near-normal levels within 6 months; in a minority,
significantly reduced vision persists.
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