01/05/2023 Dept. of Ophthalmology 1

Cystoid Macular Oedema

Moderator: Dr. Rekha B.K. MS, DOMS, PhDProfessor & Head of Department

Presenter: Dr. Arushi Prakash 3rd year Resident

Department of OphthalmologyJ.N. Medical college, Belgaum

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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

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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

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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.

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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.

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(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

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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 cytokinesmay cause incompetence of this barrier

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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

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Ocular Diagnosis Associated with Cystoid Macular Edema

VascularRetinal 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

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Ocular Diagnosis Associated with Cystoid Macular Edema

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

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Ocular Diagnosis Associated with Cystoid Macular Edema

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)

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Ocular Diagnosis Associated with Cystoid Macular Edema

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

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Ocular Diagnosis Associated with Cystoid Macular Edema

Anatomical AbnormailitiesOptic Nerve • Optic pit maculopathy • Optic disc coloboma • Morning glory disc anomalyRetina • Retinal Detachment

NeoplasticTumors • Retinal- hemangioma • Choroidal- melanoma, hemangioma, osteoma

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Ocular Diagnosis Associated with Cystoid Macular Edema

Medications Causing Macular Edema• Benzalconium Chloride

• Carmustine (BCNU)• Docetaxel• Epinephrine• Fingolimod• Glitazones• Niacin• Paclitaxel• Prostaglandin Analogs• Tamoxifen• Timolol

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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).

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OCULAR MANIFESTATIONS

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.

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OCULAR MANIFESTATIONS

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.

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

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OCULAR MANIFESTATIONS

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.

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OCULAR MANIFESTATIONS

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.

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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.

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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.

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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

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DEPRIVENS is a common mnemonic for risk factors that cause leakage on FA

DiabetesEpinephrinePars Planitis/UveitisRetinitis Pigmentosa (RP)Irvine-GassVein OcclusionE2-prostaglandinNicotinic acid and NiacinSurgery 

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Risk factors that do not demonstrate leakage on FA include:

Juvenile retinoschisisGoldmann-Favre diseasecertain types of RPNicotinic Acid maculopathyPhototoxicityAntimicrotubule agents

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Other methods proposed to quantify macular edema -confocal scanning laser ophthalmoscopy (SLO)retinal thickness analyzersoptical coherence tomography (OCT). 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).

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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)

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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.

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Pseudophakic Cystoid

Macular Edema

(Irvine-Gass Syndrome)

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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

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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

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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

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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

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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

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Risk Factors

Diabetic Macular EdemaCataract 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.

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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.

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Risk Factors

Uveitisadvent 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

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Risk Factors

Glaucoma MedicationsPreoperative 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.

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Other Risk Factors

Previous histories of retinal vein occlusion (RVO), epiretinal membrane (ERM),  

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Diagnosis

History and Clinical Presentationoccurrence 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

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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.

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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 .

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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. 

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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.

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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

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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.

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Cyclo-Oxygenase Inhibitors

Systemic COIassociated 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.

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Cyclo-Oxygenase Inhibitors

Systemic COIIndomethacin 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

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Corticosteroids

TopicalTopical 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

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Corticosteroids

TopicalOf 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.

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Corticosteroids

PeriocularPosterior 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

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Corticosteroids

IntravitrealA 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.

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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

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Corticosteroids

Systemic corticosteroids administered orally or intravenously.Numerous potential side effectsOcular 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.

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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.

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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.

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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

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Immunomodulatory TherapyRecent 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.

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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.

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Surgical Treatment

Laser VitreolysisNeodymium:YAG laser anterior vitreolysis can

release vitreous incarceration in the cataract incision wounds that complicate pseudophakic CME.

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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

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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 notWould 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

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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

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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.

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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.

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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.

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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

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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.

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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.

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Ocular Inflammations

idiopathic uveitis intermediate uveitisbirdshot retinochoroidopathysarcoidosis, toxoplasmosis,posterior scleritis,Vogt- Koyanagi-Harada's syndromeBehçet's syndrome.

The common underlying cause is an inflammatory-mediated breakdown in the blood-retina barrier

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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

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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

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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.

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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.

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PATHOLOGYOn Histopathology-

macular cysts, disorganized and glioticinner 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.

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TREATMENT, COURSE, AND OUTCOMEPatients 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

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# 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.

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