Cystoid macular oedema

Cystoid Macular
Oedema
Moderator: Dr. Rekha B.K. MS, DOMS, PhD
Professor & Head of Department
Presenter: Dr. Arushi Prakash
3rd year Resident
Department of Ophthalmology
J.N. Medical college, Belgaum
22-08-2015 Dept. of Ophthalmology 1

Abbreviations Used
 CME- Cystoid Macular Edema
 DR- Diabetic Retinopathy
 DME- Diabetic Macular Edema
 VEGF- Vascular Endothelial Growth Factor
 FA- Flourescein Angiography
 OCT- Optical Coherence Tomography
 COI- Cyclo-Oxygenase Inhibitors
 IRVAN- Idiopathic retinal vasculitis, aneurysms, neuroretinitis

Contents
 Introduction
 Pathogenesis & Etiology
 Ocular conditions associated with Cystoid Macular Oedema
 Ocular Manifestations
 Diagnosis & Ancillary Testing
 Irvine Gass Syndrome
 Diabetic Macular Oedema
 Vascular Occlusions
 Coat’s Disease
 Idiopathic juxtafoveal telangiectasis
 Radiation Retinopathy
 Arterial Macroaneurysm
 Choroidal Tumors
 Ocular Inflammation
 Retinitis Pigmentosa
 Epiretinal Membrane &Vitreomacular
Traction Syndrome
 Dominant Cystoid Macular Edema

 Macula
• anatomic specialization of the neural retina unique to humans
and to higher primates
• defined
• histologically by the presence of multiple layers of retinal
ganglion cells
(extramacular retina- only a single layer of ganglion cells)
• susceptible to such a large number of disorders,
 For eg.- the macular degenerations,
or to edema
in a large variety of diseases.

Introduction
 Cystoid macular oedema (CME) results from the accumulation
of fluid in the outer plexiform and inner nuclear layers of the
retina with the formation of cyst-like changes.
 These are called cystoid because they not lined with a layer of
epithelial cells, which would
make them true cysts;
hence, these spaces are
cystoid and not cystic
 Fluid may initially accumulate
intracellularly in Müller cells,
with subsequent rupture.

(A) Histology shows cystic spaces in
the outer plexiform and inner nuclear
layer
(B) progression to
lamellar hole formation
In long-standing cases, smaller microcystic spaces coalesce into larger
cavities and may progress to lamellar hole formation at the fovea with
irreversible impairment of central vision

Pathogenesis & Etiology
 Intraretinal fluid may accumulate into CME by:
Breakdown of normal anatomical barriers:
a) inner blood retinal barrier
b) outer blood retinal barrier
inflammatory mediators such as
prostaglandins, leukotrienes, protein kinase C, nitric oxide, vascular endothelial
growth factor, various other cytokines
may cause incompetence of this barrier

Pathogenesis & Etiology
 Another mechanism results from failure of
normal physiological process-
 In normal functional retina, RPE cells constantly act to
eliminate fluid from retina.
 If this function is compromised, CME may occur

Ocular Diagnosis Associated with
Cystoid Macular Edema
Vascular
Retinal Vascular
• Diabetic Retinopathy
• Retinal Vein occlusion-branch
or central
• Radiation Retinopathy
• Juxtafoveal retinal
telangiectasis
• Coat’s Disease
• Retinopathy of Prematurity
• Ocular Ischemic Syndrome
Choroidal Vascular
• Choroidal Neovascularization
• Hypertensive Retinopathy

Postoperative
• Irvin-Gass Syndrome
• Penetrating Keratoplasty
• Scleral buckling
• Laser Treatment (Argon or
YAG (Yttrium-Aluimium-Garnet) )
• Cryotherapy
• Panretinal photocoagulation

INFLAMMATORY
CONDITIONSUveitis
• HLA-B27
• Eales' disease
• Cytomegalovirus retinitis
• Pars planitis
• Behçet's syndrome
• Birdshot choroidopathy
• Sarcoidosis
• Idiopathic vitritis
• Scleritis
• Infectious Uveitis
(Toxoplasmosis, Cytomegalovirus)
Neuroretinitis
• Multiple infectious causes
(i.e., Bartonella, Lyme)
• Idiopathic retinal vasculitis,
aneurysms, neuroretinitis
(IRVAN)

Retinal Dystrophies
• Retinitis pigmentosa
• Autosomal dominant cystoid
macular edema
• Goldman Favre (enhanced S-
cone syndrome)
• Juvenile X-linked retinoschisis
Tractional
• Epiretinal Membrane
• Vitreomacular traction
syndrome
• Vitreomacular traction
assosiated with Myopia
• Macular Hole

Anatomical Abnormailities
Optic Nerve
• Optic pit maculopathy
• Optic disc coloboma
• Morning glory disc anomaly
Retina
• Retinal Detachment
Neoplastic
Tumors
• Retinal- hemangioma
• Choroidal- melanoma,
hemangioma, osteoma

Medications Causing
Macular Edema• Benzalconium Chloride
• Carmustine (BCNU)
• Docetaxel
• Epinephrine
• Fingolimod
• Glitazones
• Niacin
• Paclitaxel
• Prostaglandin Analogs
• Tamoxifen
• Timolol

OCULAR MANIFESTATIONS
 The major symptom is decreased central visual acuity.
 Accompanying symptoms may include metamorphopsia,
micropsia, scotomata, ocular irritation, photophobia, and
conjunctival injection.
 Presenting visual acuities usually range from 20/25 (6/8) to
20/80 (6/26) but may be as poor as 20/400 (6/133).
 Clinically, CME is seen best using the slit lamp and either a
contact lens (e.g., Goldmann lens) or a handheld, noncontact
lens (e.g.,78 D, 60 D).

 The edema results in light scattering due to the multiple
interfaces created by the separated retinal cells.
 This light scattering decreases the neural retina’s translucency so
that the normal retinal pigment epithelial and choroidal
background patterns are blurred.
 Individual pockets of fluid in the outer plexiform layer are seen,
with the largest pockets centrally and progressively smaller cysts
peripherally.

 Retroillumination can help to delineate the polycystic spaces.
 As these changes can be subtle and media opacity can affect the
view, clinical confirmation of CME may be difficult in certain
eyes.
 A yellow spot, believed to be due to diffusion of the luteal
pigment, may be evident in the central macula.
 Small intraretinal and intracystic hemorrhages, microaneurysms,
and telangiectasias may be seen as well.

Cystoid macular edema in the Irvine-Gass syndrome. Note the radial
cystoid changes centered in the fovea causing a “yellow spot.”

 The underlying cause of CME does not alter its appearance, but
the associated ocular findings vary widely depending on etiology.
 After cataract surgery, aqueous and/or vitreous cell and flare,
optic nerve head swelling, and a ruptured anterior hyaloid face
may be evident.
 Other patients show vitreous traction to anterior chamber
structures.
 Epiretinal membranes are concurrently present in about 10% of
eyes.

 Inflammatory, vascular, tractional, inherited, and tumor-related
causes result in the corresponding associated ocular findings.
 As a result of CME, a rupture of the inner retinal cyst can occur
to give a lamellar macular hole.
 Prolonged CME may induce atrophy of the macular
photoreceptors – visual acuity is poor, but clinical examination
and fluorescein angiography may be grossly normal with the
exception of a blunting of the foveal reflex.

Diagnosis and ancillary Testing
 In eyes with clear media, clinical examination usually yields the
diagnosis.
 Angiography reveals a typical petalloid pattern in the central
macula secondary to dye leakage from the perifoveal capillaries.
The dye accumulates in cyst-like spaces within the outer
plexiform layer (Henle’s layer).
 Late fluorescein angiographic pictures should be taken to allow
time for the dye to accumulate within the anatomical fovea. Also
seen on fluorescein angiography is leakage from the disc and
retinal vessels.

Fluorescein
angiography
demonstrates cystoid
macular edema very
well. (A) In the early
stage of the angiography,
minimal leakage is seen.
(B) The petalloid leakage
pattern, present in the late
stages of the angiography,
is diagnostic of CME. (C)
Note the profuse leakage
from the optic nerve head
in the late stages.

 In the early phase of
FA, capillary dilation in
the perifoveal region is
appreciated
 In the late phase of FA, leakage into
the cystoid spaces is distributed
radially in Henle’s layer forming the
classic petaloid leakage pattern or
expansile dot appearance

 DEPRIVENS is a common mnemonic for risk
factors that cause leakage on FA
 Diabetes
 Epinephrine
 Pars Planitis/Uveitis
 Retinitis Pigmentosa (RP)
 Irvine-Gass
 Vein Occlusion
 E2-prostaglandin
 Nicotinic acid and Niacin
 Surgery

 Risk factors that do not demonstrate leakage on
FA include:
 Juvenile retinoschisis
 Goldmann-Favre disease
 certain types of RP
 Nicotinic Acid maculopathy
 Phototoxicity
 Antimicrotubule agents

Other methods proposed to quantify macular edema -
 confocal scanning laser ophthalmoscopy (SLO)
 retinal thickness analyzers
 optical coherence tomography (OCT).
22-08-2015
Clinically significant DME
with large foveal cysts and
subfoveal neuroretinal
detachment (right eye):
(A) Color fundus photo; (B)
FAF showing pattern of
multiple-spot iFAF;
(C) microperimetry sensitivity
map overlaid on the FAF
image;
(D) fluorescein angiography
confirming cystoid macular
edema;
(E) OCT (retinal thickness
map and line scan). Mean
retinal sensitivity in the central
4° (∼1 mm) was 5.5 dB;
BCVA was +0.16 logMar (0.7
Snellen equivalent).

Optical coherence tomogram of a patient who has postcataract
cystoid macular edema.
Note the central cysts, loss of the foveal depression, and macular
thickening (normal is approx 212 μm)

Diagnosis
and ancillary
Testing
 In cases of aphakic and pseudophakic CME, it is critical to
perform gonioscopy, which is helpful in diagnosing structural
problems of the wound, such as in the iris or vitreous, that may
be playing a role.

Pseudophakic Cystoid
Macular Edema
(Irvine-Gass Syndrome)

 In 1953, Irvine described a cystoid macular edema
(CME) that specifically arised after cataract surgery.
 Gass and Norton subsequently studied the
characteristics of the new disease entity with
fluorescein angiography

Definitions
 Angiographic pseudophakic CME: seen on fluorescein
angiography (FA)
 Clinical pseudophakic CME: associated with decreased visual
acuity
 Acute pseudophakic CME: within 6 months
 Chronic pseudophakic CME: over 6 months

Incidence
 Angiographic CME after intracapsular cataract
extraction: As high as 60%*
 Angiographic CME after extracapsular cataract
extraction: 15% to 30%
 Clinical CME after small incision phacoemulsification:
0.1% to 2.35%
 OCT evidence of CME after small incision
phacoemulsification: 4% to 11%, but also reported to be
as high as 41%
 Most patients with CME found via angiography or OCT will not
have visual changes.
 Furthermore, most patients with clinical CME will experience
spontaneous improvement by 3 to 12 months

Pathophysiology
 inflammatory mediators that are upregulated in
the aqueous and vitreous humors after surgical manipulation
 Inflammation breaks down the blood-aqueous and blood-
retinal-barriers, leads to increased vascular permeability
 Eosinophilic transudate accumulates in the outer plexiform and
inner nuclear layers of the retina to create cystic spaces that
coalesce to form larger pockets of fluid
 In chronic CME, lamellar macular holes and subretinal fluid may
also form

Risk Factors
 Surgical
 • Vitreous loss
• Vitreous traction at incision sites
• Vitrectomy for retained lens fragments
• Iris trauma
• Posterior capsule rupture
• Intraocular lens dislocation
• Early postoperative capsulotomy
• Iris-fixated intraocular lenses
• Anterior chamber intraocular lenses

Risk Factors
 Diabetic Macular Edema
 Cataract surgery was historically thought to accelerate
progression of diabetic retinopathy (DR),
 However, postoperative macular edema usually develops in
those with a prior history of diabetic macular edema (DME).
 DME and severe DR should be thoroughly treated before
undergoing cataract extraction.

Risk Factors
 Diabetic Macular Edema
 The source of the fluid has been presumed to be due to
excessive retinal capillary leakage, but there is some evidence
that the retinal pigment epithelium may also have dysfunctional
fluid homeostatic
function in diabetes and
contribute to this
appearance.

Risk Factors
 Uveitis
 advent of modern surgical techniques is allowing patients with
uveitis, who often develop cataract prematurely, opportunities
for intraocular lens placement.
 Incidence of pseudophakic CME is greater in these patients

Risk Factors
 Glaucoma Medications
 Preoperative and postoperative topical glaucoma medications,
specifically latanoprost and timolol, may increase the incidence
of pseudophakic CME.
 It appears that a commonly used preservative, benzalkonium
chloride, is cytotoxic and stimulates inflammatory responses.
 The effects may be prevented by concomitant use of NSAID
drops.

Other Risk Factors
Previous histories of
 retinal vein occlusion (RVO),
 epiretinal membrane (ERM),

Diagnosis
History and Clinical Presentation
 occurrence peaks at approximately 4-6 weeks postoperatively.
 most common presentation is blurry vision.
 Less common presentations include central scotomas,
metamorphopsia, and mild photophobia.
 On biomicroscopy, retinal thickening and loss of the foveal
depression is usually appreciated.
 findings are best observed with fundus contact lens, red-free
light may aide in demonstrating cystic changes.
 In severe or chronic cases, optic disc swelling and/or a lamellar
hole may also be seen.
 Splinter hemorrhages may also be present.
 Biomicroscopy may not show any abnormalities in 5-10% eyes

Imaging Studies
 On fluorescein angiography, pseudophakic CME is characterized
by retinal telangiectasis, capillary dilatation, and leakage from
perifoveal capillaries in the early phase frames, and perifoveal
hyperfluorescent spots classically described as a “petaloid”
pattern in late phase frames, representing fluorescein
accumulation in cystic spaces.
 Cystoid changes may also be apparent in the fovea and
extramacular areas.
 Optic nerve staining is also commonly seen,
 FA is the gold standard in diagnosing pseudophakic CME, but
treatment responses would be more conveniently monitored by
biomicroscopy, visual acuity and OCT.

 OCT is a relatively new, widely adopted technology that
allows high-resolution cross-sectional imaging of
the macula .
 Pseudophakic CME on OCT is characterized by loss
of the foveal depression, retinal thickening, and cystic
hyporeflective areas within the macula.
 However, OCT has not replaced FA as the gold
standard in diagnosing pseudophakic CME, because
FA can also rule out other causes of CME such
as diabetic macular edema and retinal vein occlusion .

Differential Diagnosis
 Diabetic macular edema
 Retinal vein occlusion
 Radiation retinopathy
 Hypertensive retinopathy
 Uveitis
 Topical prostaglandins
 Retinal dystrophies
 Choroidal tumors
 Leukemia
 Chronic renal failure
 Of special note, it is important not to misdiagnose pseudophakic CME
with choroidal neovascularization seen in age-related macular degeneration
(AMD). Many patients undergoing cataract surgery are also within the age
range for developing AMD.

Prophylaxis and Treatment
 no standardized treatment or prophylactic protocol for
pseudophakic CME, due a lack of strong randomized
clinical trials and comparative effectiveness studies.
 lack of large studies due to fact that most cases of
acute pseudophakic CME spontaneously resolve.
 treatment of chronic pseudophakic CME remains a
challenge.

 The management of pseudophakic CME is based on its
pathogenesis.
 In the inflammation cascade, cell membrane lipids are
converted to arachidonic acid by phospholipase A2,
and prostaglandins are then formed by cyclooxygenases
(COX).
 Corticosteroids reduce inflammation by inhibiting
phospholipase A2, and
 nonsteroidal anti-inflammatory drugs (NSAIDs) inhibit
COX.
 The 2 critical COX isoforms are COX-1 andCOX-2, and
the later is the major isoform expressed in the retina

Cyclo-Oxygenase Inhibitors
 Cyclo-oxygenase is inhibited by COI drugs, which prevents the
conversion of arachidonic acid into endoperoxides and, hence,
inhibits PG synthesis. Clinically, COIs decrease capillary leakage.
 Topical COIs (Ketorolac tromethamine 0.5%, indomethacin
1%, Nepafenac 0.1%, Bromofenac 0.09%)
 Complications associated with the use of topical anti-
inflammatory agents include ocular irritation and discomfort
following application, conjunctival injection, mild punctate
keratopathy, and mydriasis. Allergic and hypersensitivity
reactions have been reported.

Cyclo-Oxygenase
Inhibitors
Systemic COI
 associated with multiple side effects.
 Gastrointestinal- nausea, vomiting, diarrhea, anorexia,
abdominal discomfort, ulceration, gastrointestinal bleeding.
 Can interfere with platelet function and clotting.
 Bone marrow suppression, hepatotoxicity, impaired renal
function, and central nervous system symptomatology, including
headache, dizziness, somnolence, depression, fatigue, insomnia,
and confusion, have been reported.
 Hypersensitivity and induction of asthmatic attacks can occur.

Cyclo-Oxygenase Inhibitors
Systemic COI
 Indomethacin can be deposited in the cornea in a pattern.
 Association with optic nerve dysfunction has been reported.
 dermatologic reactions include rash, dermatitis, and Stevens-
Johnson syndrome can occur.
 The availability of topical formulations that provide good ocular
penetration of the drug make it unnecessary to recommend
systemic therapy for ophthalmic indications, particularly in view
of the many serious side effects of systemic administration

Corticosteroids
Topical
 Topical corticosteroids are commonly used in the treatment of
pseudophakic CME.
 lipophilic acetate suspensions of prednisolone penetrate the intact
corneal epithelium and reach the anterior chamber in higher
concentration than water-soluble forms, such as dexamethasone
 Few studies, however, have examined their efficacy.
Furthermore, its effects are often confounded by the
concomitantly used and more efficacious topical NSAIDs.
 Significant potential complications of topical corticosteroid
therapy include glaucoma, posterior subcapsular cataracts,
exacerbations of infections, and corneal problems

Corticosteroids
Topical
 Of note, anecdotal evidence indicates that the visual acuity of
“corticosteroid responders” with postcataract CME improves
more than that of patients whose IOP remains normal. This
may be explained by a hydrostatic effect.

Corticosteroids
Periocular
 Posterior sub-Tenon's injections may have advantages over
topical application.
 exert a maximal, long-lasting response at site of injection,
 Water soluble drugs have excellent penetration through the
sclera as opposed to topical application.
 Carry carry the risk of inadvertent penetration of the globe.
 Contraindications for posterior sub-Tenon's injections include
steroid-induced glaucoma, hypersensitivity to components of
the injected steroid preparation, active
necrotizing scleritis, and active
ocular toxoplasmosis

Corticosteroids
Intravitreal
 A single intravitreal injection of triamcinolone induced clinical
and angiographic resolution of CME.
 Side effects of treatment were similar to those of periocular
injection including IOP rise and increased rate of cataract
formation.
 major limitations of intravitreal corticosteroids is the transient
effect that requires repeated injections.

Spectral domain optical coherence tomography (SD-OCT) of a patient with
chronic refractory pseudophakic CME. There is retinal thickening, cystic
intraretinal lesions, and subretinal fluid.
SD-OCT of the same patient 1 month after a sub-Tenon's
injection of triamcinolone. The retinal thickening, cystic
lesions, and subretinal fluid have improved

Corticosteroids
Systemic corticosteroids
 administered orally or intravenously.
 Numerous potential side effects
 Ocular complications - elevated IOP, posterior subcapsular
cataract, increased incidence of viral ocular infections, ptosis,
mydriasis, scleral melt, and lid skin atrophy.
 Potential short-term systemic effects- peptic ulcer disease, aseptic
necrosis of the femoral head, and mental changes (euphoria,
insomnia, & psychosis).
 Long-term systemic side effects- osteoporosis, a cushingoid state,
electrolyte imbalance, reactivation of latent infections such as
tuberculosis, myopathy, suppression of the pituitary-adrenal axis,
and increased severity of preexisting diabetes and hypertension.

Corticosteroids
Drug Delivery Systems
 Sustained drug delivery systems (DDS) have been developed to
address this limitation of intravitreal corticosteroid injections.
 Ozurdex (Allergan, Irvine, CA) is an injectable, biodegradable
intravitreal DDS that provides sustained release of preservative-
free dexamethasone, a potent corticosteroid.

Anti-VEGF Treatments
 VEGF is well known to be a key mediator of angiogenesis, but
it also plays an important role in the inflammation and capillary
permeability that causes CME.
 Bevacizumab (Avastin, Genentech, South San Francisco, CA) is a humanized
monoclonal antibody that inhibits VEGF-A
“Triple therapy” with intravitreal triamcinolone, intravitreal
bevacizumab and topical NSAIDs has been shown to be
effective as well, although the effects of the intravitreal
medications were transient.

Carbonic Anhydrase Inhibitors
 Oral CAIs may be considered in refractory pseudophakic CME.
 CAIs improve the pumping action of the retinal pigment
epithelium, to decrease intraretinal fluid.
 initial dose of 250mg daily, increased to 500mg daily if no effect
is apparent.
 A trial of several weeks is warranted, with successful outcome
judged by improved visual acuity on careful repeated
measurements or by decreased CME on fluorescein
angiography.
 If decreased CME is observed by fluorescein angiography after
several weeks of use, the continuation of acetazolamide may be
considered even if visual acuity has not improved, provided the
patient is able to tolerate the drug

Immunomodulatory Therapy
 Recent small pilot studies have started to examine interferon
alpha (IFN-a, Imgenex, San Diego, CA) and intravitreal infliximab (Remicade,
Centocor Ortho Biotech, Horsham, PA) with mixed results.

HYPERBARIC
OXYGEN
 Improvement in aphakic CME with hyperbaric oxygen therapy
was reported by Ploff and Thom*.
 The dosage given was 2.2atm oxygen for 1.5h twice a day for 7
days, followed by 2h daily for 14 days. The mechanism was
hypothesized to be macular capillary contraction.
* Williams GA, Haller JA, Kuppermann BD, et al. Dexamethasone posterior-segment drug delivery system in the
treatment of macular edema resulting from uveitis or Irvine-Gass syndrome. Am J Ophthalmol 2009;147(6):1048-1054,
1054 e1041-1042.

Surgical
Treatment
Laser Vitreolysis
 Neodymium:YAG laser anterior vitreolysis can release vitreous
incarceration in the cataract incision wounds that complicate
pseudophakic CME.

Pars Plana Vitrectomy
 may be considered when
pseudophakic CME is
complicated by
vitreoretinal traction,
and/or if the CME is
unresponsive to other
treatments.
 Vitrectomy may also
theoretically reduce the
concentration of
inflammatory mediators
and growth factors.
Surgical Treatment

Situation Recommendations/Options
Intracameral lens (within the pupil;
iris suspended)
Remove, consider exchange for
flexible AC IOL, or suture-fixated
PC IOL
Anterior chamber IOL with
distorted pupil and vitreous strands
in AC
Vitrectomy to restore normal pupil.
Leave IOL if flexible, remove if not
Would a sulcus-fixated PC IOL be
safe? Omitting IOL is another
option, as is a suture-fixated PC IOL
ACIOL without distorted pupil or
vitreous in AC
Remove, especially if rigid IOL;
replace with flexible IOL or would
sulcus-fixated PC IOL be safe?
Elevated, isolated vitreous strand
distorting pupil with AC or PC IOL
Consider YAG vitreolysis

Situation Recommendations/Options
PCIOL with pupillary capture Free capture
PC IOL with moderate pupillary
distortion from vitreous strands
Anterior vitrectomy for restoring
pupil to normal and consider leaving
lens
PCIOL, sulcus fixation, normal pupil Consider removing IOL; possibly
exchange for flexible AC IOL, or
leave IOLs out, or use a suture-
fixated PC IOL
PC IOL; “in-the-bag,” normal pupil Pars plana vitrectomy if evidence of
traction on macula (rare). Rule out
other causes of CME
PCIOL with pupillary capture Free capture
PCIOL with moderate pupillary
distortion from vitreous strands
Anterior vitrectomy for restoring
pupil to normal and consider leaving
lens

Retinal Vein Occlusions
 Both branch and central vein occlusions can result
in severe macular edema.
 Due to hypoxic capillary endothelial damage
secondary to increased intravascular hydrostatic
pressure.
 The Branch Vein Occlusion Study demonstrated
visual benefit following application of a grid
pattern of photocoagulation to the area of chronic
macular edema.
 The Central Retinal Vein Occlusion Study showed
no visual benefit in the use of grid macular
photocoagulation for the treatment of associated
macular edema.
However, a possible benefit may be seen in younger patients who undergo treatment. It
is not unusual for CME to overlie a choroidal neovascular membrane or a serous retinal
detachment. Often it goes unnoticed in light of the accompanying severe subretinal
and/or intraretinal abnormalities.

Coat’s Disease
 Other, more unusual retinal vascular
conditions produce CME. Retinal
telangiectasis (also known as Leber's miliary
aneurysms or Coats' disease) is a unilateral
condition that occurs more commonly in
males. The retinal vasculature is anomalous
and produces leakage with resultant cystoid
and diffuse macular edema.
 The vascular anomalies may be local or
widespread.
 Cryotherapy or laser treatment is applied if
these lesions threaten macular function.

Idiopathic juxtafoveal telangiectasis
 Idiopathic juxtafoveal
telangiectasis can occur
in either a unilateral or
bilateral pattern.
 Exudation and CME
result in visual loss.
 Photocoagulation may
help to improve vision
if there is no
spontaneous
improvement.

Radiation Retinopathy
 Patients who receive radiation treatment involving the head and neck may
develop signs of radiation retinopathy 6 months to 3 years following this
treatment.
 A bilateral retinopathy occurs in almost one third of patients treated with
external beam irradiation.
 Local plaque therapy requires higher doses than external beam therapy to
produce damage.
 The clinical features of radiation retinopathy mirror those of diabetic
retinopathy and include CME.
 Although no therapy is
proved, grid laser
photocoagulation is
believed to lessen the
macular edema

Arterial Macroaneurysm
 Acquired retinal arterial
macroaneurysms are often
multiple and may thrombose
and close spontaneously.
 This entity is treated if lipid
exudate threatens the macula
or if CME develops.
 Two modalities of laser
treatment to be considered
are direct laser treatment to
close off the lesions and
indirect laser treatment in a
tight grid pattern around the
macroaneurysm.

Choroidal Tumors
 CME with choroidal
tumors, such as nevi,
malignant melanomas,
and cavernous
hemangiomas, can
occur.
 Intraretinal cystoid
changes occur over
the tumor and at sites
distant from the
tumor as a result of
lack of oxygenation
of retinal tissue.

Ocular Inflammations
 idiopathic uveitis
 intermediate uveitis
 birdshot retinochoroidopathy
 sarcoidosis, toxoplasmosis,
 posterior scleritis,
 Vogt- Koyanagi-Harada's syndrome
 Behçet's syndrome.
 The common underlying cause is an
inflammatory-mediated breakdown in the
blood-retina barrier

Retinitis Pigmentosa
 Patients who have
retinitis pigmentosa
can also have CME.
 Although the
precise
pathophysiology is
not clearly defined,
it is felt that the
perifoveal
capillaries
demonstrate
increased
permeability in
such cases

Epiretinal Membrane
&
Vitreomacular Traction Syndrome
 The presence of epiretinal
membranes and the
vitreomacular traction
syndrome are associated with
CME, particularly in areas of
greatest traction and distortion
of retinal blood vessels.
Management of this condition
is to remove the membrane
surgically
22-08-2015 Dept. of Ophthalmology
7
6

DOMINANT CYSTOID MACULAR
EDEMA
 extremely rare autosomal dominant disease
mapped to chromosome 7q.
Interestingly, autosomal dominant retinitis
pigmentosa, known as RP9, also maps to this region.
 Unique macular disorder-
the inner nuclear layer of retina is the site primarily affected.
 Müller's cells are the specific cellular constituents thought to be
involved, based on histopathological evidence.

 Ophthalmoscopy reveals multilobulated cysts
in the macula.
 Peripheral pigmentary changes
may be present.
 Ancillary testing with fluorescein angiography
shows capillary leakage, with petaloid dye accumulation in the
macula.
 Electrophysiology shows normal electroretinographic findings,
but subnormal electro-oculographic light peak–to–dark trough
ratios have been found, and abnormal dark-adaptation studies
have been documented.

PATHOLOGY
 On Histopathology-
macular cysts, disorganized and gliotic
inner nuclear layer, focal Müller's cell necrosis,
epiretinal membrane formation, and
abnormal deposition of basement membrane
in the perivascular space.
Degenerative changes also have been seen in the RPE and
photoreceptors of the macula. Histopathological features seen
in dominantly inherited cystoid macular edema, namely the
involvement of Müller's cells, are quite different from those
features seen in cystoid macular edema secondary to other
causes.

TREATMENT, COURSE, AND OUTCOME
 Patients first begin to notice decreasing visual
acuity at about 30 years of age, with slowly
progressive worsening to a moderate or
severe level years later.
 Advanced cases have maculae of atrophic
appearance, with window defects seen on
fluorescein angiography.
 As in all the macular dystrophies, no known effective treatment

81
# Ophthalmology. Myron Yanoff & Jay S. Duker. 4th Ed. Pg
625-31
# Cataract Surgery And Its Complications. by Norman S. Jaffe,
Mark S. Jaffe, Gary F. Jaffe. 4th Ed. Pg 426-41
#http://eyewiki.aao.org/Pseudophakic_Cystoid_Macular_Edema
_(Irvine-Gass_Syndrome)
#The Retina and its Disorders, Joseph Besharse, Dean Bok. 1st Ed.

Cystoid macular oedema

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