BASICS

DESCRIPTION

• Accumulation of fluid within and under the perifoveal region causing decreased acuity.

• Changes are often visible on clinical examination but sometimes may only be evident on fluorescein angiography (FA) or optical coherence tomography (OCT).

• Cystoid macular edema (CME) is a final common pathway of various retinal disorders. The most common cause is postoperative CME.

EPIDEMIOLOGY

Incidence

• CME may be detected by FA in up to 20% of uncomplicated cataract surgery cases.

• Clinically significant CME (vision worse than 20/40) occurs in up to 2.4% of patients after uncomplicated phacoemulsification (1).

RISK FACTORS

• Complicated cataract surgery (vitreous incarceration, iris trauma, long-operative times, prior history of intraocular inflammation)

• Diabetes

• Other retinal vascular disease

• Intraocular inflammation

• Macular pucker

• Preoperative use of topical prostaglandin agonists

Genetics

Unknown

GENERAL PREVENTION

• Postoperative topical steroids and nonsteroidal anti-inflammatory drugs (NSAIDs)

• Consider preoperative topical NSAIDs

PATHOPHYSIOLOGY

Not completely understood. Theories of contributing factors include UV light exposure, mechanical vitreous traction, and inflammation leading to vascular instability/breakdown of the blood aqueous barrier.

ETIOLOGY

• May occur after any type of ocular surgery, including laser.

• Uveitis, retinal vasculitis

• Diabetic retinopathy

• Exudative age-related macular degeneration

• Retinal vein occlusions

• Retinitis pigmentosa

• Retinal telangiectasis (Coats’ disease)

• Venous or arterial macroaneurysms

• Drugs (tamoxifen, niacin, betaxolol, epinephrine, dipivefrin, prostaglandin agonists)

• Macular pucker, vitreomacular traction

• Radiation retinopathy

• Other (intraocular tumor, systemic hypertension, serous and rhegmatogenous retinal detachments)

• Optic nerve head abnormalities (optic pit, coloboma, diabetic papillopathy)

• Pseudo-CME (no leakage seen in: Nicotinic acid retinopathy, docetaxel, X-linked retinoschisis, and Goldmann-Favre disease)

DIAGNOSIS

HISTORY

• Recent intraocular surgery or lasers?

• History of diabetes, uveitis, radiation, hypertension?

• Medication history: Does it include tamoxifen, niacin, betaxolol, epinephrine, dipivefrin, prostaglandin agonists, docetaxel?

• Any family history of CME?

PHYSICAL EXAM

• Foveal thickening

• Cystic spaces in the perifoveal area

• Evaluate for signs of retinal vascular disease (diabetic retinopathy, retinal vascular occlusion, retinal telangiectasis, or macroaneurysm)

• Evaluate for signs of intraocular inflammation

• Evaluate for occult retinal detachment and intraocular tumors

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• None for post-op CME

• If clinical suspicion is present for uveitis, hypertension, retinal venous occlusion, or undiagnosed diabetes, laboratory studies may be indicated.

Imaging

Initial approach

• OCT: Increased foveal thickness, cystic spaces in outer plexiform layer, and subretinal fluid

• Fluorescein angiogram: Most cases of pseudophakic CME will show late intraretinal pooling of dye in a petaloid fashion, as well as hyperfluorescence of the optic nerve.

• Causes of CME without leakage on FA include nicotinic acid retinopathy, docetaxel, X-linked retinoschisis, and Goldmann-Favre disease.

Follow-up & special considerations

• OCT is helpful for monitoring response to treatment and for evaluating CME caused by an epiretinal membrane or vitreomacular traction.

Pathological Findings

May be intracellular (Muller cells) or extracellular accumulation of fluid.

DIFFERENTIAL DIAGNOSIS

• Diabetic macular edema

• Retinal venous occlusions

• Macular pucker

• Exudative AMD

• Chronic retinal detachment

• Other (intraocular tumor, systemic hypertension, serous and rhegmatogenous retinal detachments)

• Optic nerve head abnormalities (optic pit, coloboma, diabetic papillopathy)

• Pseudo-CME (no leakage seen in: Nicotinic acid retinopathy, docetaxel, X-linked retinoschisis, and Goldmann-Favre disease)

– See Algorithm.

TREATMENT

MEDICATION

First Line

• Treatment outlined below focuses on post-op CME.

• 70% of post-cataract CME resolves spontaneously within 6 months.

• Treat if symptomatic with decreased vision or metamorphopsia.

• Trial of topical steroids (prednisolone 1% q.i.d, or difluprednate q.i.d) and topical NSAIDs (diclofenac 0.1% t.i.d, ketorolac 0.4% t.i.d, nepafenac t.i.d (2)[C], or bromfenac 0.09% b.i.d). Use in combination for 4–6 weeks.

• Consider stopping prostaglandin agonists and other exacerbating medications.

Second Line

• Periocular steroids (triamcinolone 40 mg) or intravitreal steroids (triamcinolone 4 mg)

• Intravitreal anti-VEGF agents (bevacizumab 1.25 mg) (3)[B]

• Steroids and anti-VEGF may be used simultaneously or sequentially while continuing topical therapy (4)[C].

• Repeat treatments may be necessary at 1 (for bevacizumab) to 3 months (for depot steroids) intervals.

ADDITIONAL TREATMENT

Issues for Referral

Consider referral to a vitreoretinal specialist if CME is not responsive to topical treatment, or if there are coexisting conditions such as DME, uveitis, retinal vascular occlusions, and others.

Additional Therapies

• Acetazolamide 250–500 mg b.i.d may be beneficial in post-op CME.

• Oral acetazolamide 250–500 mg b.i.d for CME associated with retinitis pigmentosa

• Intravitreal ranibizumab 0.5 mg may be useful for CME associated with retinitis pigmentosa (5)[C].

SURGERY/OTHER PROCEDURES

• Nd:YAG vitreolysis may be helpful in eyes with vitreous to cataract wound.

• Focal and/or grid laser is useful in CME predominantly related to diabetic macular edema and retinal vein occlusions.

• Vitrectomy and membrane peel

Useful in diffuse DME, macular pucker, vitreomacular traction, and in cases of post-op CME unresponsive to other treatment modalities.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

• Every 4–6 weeks until CME resolves, then as needed

• Ocular steroids may cause acceleration of cataract formation and may increase the intraocular pressure.

PROGNOSIS

Usually good for post-op CME.

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