Pseudophakic (Irvine-Gass) Macular Edema 

  • Author: David G Telander, MD, PhD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 21, 2010
 

Background

Cystoid macular edema (CME) is a painless condition in which swelling or thickening occurs of the central retina (macula) and is usually associated with blurred or distorted vision.

CME is a relatively common condition and is frequently associated with various ocular conditions, such as cataract surgery, age-related macular degeneration (ARMD), uveitis, eye injury, diabetes, retinal vein occlusion, or drug toxicity. When CME develops following cataract surgery and its cause is thought to be directly related to the surgery, it is referred to as Irvine-Gass syndrome.

Chronic CME or multiple recurrences may result in macular photoreceptor damage with permanent impairment of central vision.

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Pathophysiology

The primary cause of cystoid macular edema (CME) depends on the underlying disease process, but most pathways eventually lead to vascular instability and breakdown of the blood-retinal barrier. The Müller cells in the retina become overwhelmed with fluid leading to their lysis. This results in an accumulation of fluid in the outer plexiform and inner nuclear layers of the retina. Diabetes and retinal vein occlusion can both lead to CME by causing vascular instability directly (vascular endothelial cell damage). Alternatively, CME associated with uveitis or following cataract surgery is most likely caused by the cytokines released by activated inflammatory cells. These molecules lead to breakdown of the blood-retinal barrier and capillary leakage.

Inflammatory cause

In the inflammatory pathway, the enzyme phospholipase causes the release of arachidonic acid. Subsequently, cyclooxygenase converts arachidonic acid to prostaglandin. Prostaglandins can cause breakdown of the blood-retinal barrier, including vasodilation, increased capillary permeability from compromise of tight endothelial junctions in the retinal capillaries, and decreased removal of fluid by the retinal pigment epithelium (RPE). The enzyme phospholipase can be inhibited by steroids and thereby blocks the formation of prostaglandins and their effects. The cyclooxygenase pathway is specifically inhibited by aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs).

Another product of arachidonic acid breakdown involves the enzyme lipoxygenase, which alternately converts arachidonic acid to leukotriene, a chemotactic agent. The exact role of leukotriene in CME remains unclear, and, currently, no lipoxygenase specific blocking agents are approved for use in the treatment of CME.

Other causes

Patients with systemic disorders, such diabetes or renal failure, may develop CME from breakdown of the blood-retinal barrier primarily due to vascular compromise. In diabetes, endothelial cells are damaged by advance glycosylation end-products. In addition, cytokines, such as vascular endothelial growth factor (VEGF), accumulate in the vitreous cavity of diabetic patients and lead to capillary leakage. CME can also be caused by mechanical forces (ie, epiretinal membrane, vitreomacular traction) pulling on the retinal surface, leading to vascular compromise and breakdown of the blood-retinal barrier.

Other ocular conditions, such as exudative ARMD, cause CME by the growth of neovascular membranes, which are inherently leaky.

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Epidemiology

Frequency

United States

Incidence of CME depends on the etiology.

The incidence following cataract surgery (Irvine-Gass syndrome) is approximately 1% after modern phacoemulsification surgery. The frequency was more common in older types of cataract surgery, such as extracapsular cataract extraction, where CME could occur in up to 20% of patients.

From other causes, the frequency varies. For example, most patients with wet ARMD have some component of CME. (No CME is found in dry ARMD.) Diabetic macular edema itself is the most common cause of vision loss in patients with nonproliferative diabetic retinopathy. CME is also a common cause of vision loss in patients with uveitis.

International

Studies have reported a similar incidence of CME and Irvine-Gass syndrome worldwide.

Mortality/Morbidity

The natural history depends on the etiology.

CME following cataract surgery, although usually treated medically, has been shown to often resolve spontaneously, with 90% of eyes improving to a visual acuity of 20/40 or better in cases with a posterior chamber intraocular lens (IOL). However, remissions and exacerbations of macular edema can result in photoreceptor damage with permanent impairment of vision.

CME due to diabetes, retina vein occlusion, or chronic uveitis tends to be chronic with periods of remission and exacerbation.

Race

No significant racial predilection exists.

Sex

No sexual predilection exists.

Age

CME can occur at any age depending on the etiology. Advanced age has been reported as a risk factor for the development of Irvine-Gass syndrome.

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Contributor Information and Disclosures
Author

David G Telander, MD, PhD  Assistant Professor, Department of Ophthalmology and Vision Science, Division of Vitreo-Retinal Diseases and Surgery, University of California Davis School of Medicine

David G Telander, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher T Cessna, DO  Vitreo-Retinal Specialist, Geisinger Health System

Christopher T Cessna, DO is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Specialty Editor Board

Brian A Phillpotts, MD  Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine

Brian A Phillpotts, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, George Alexandrakis, MD, to the development and writing of this article.

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Fundus photo of the right eye in a patient with cystoid macular edema.
Fluorescein angiography of the right eye (late phase) showing central macular leakage in cystic spaces around the fovea.
Optical coherence tomography (OCT) of the right eye showing central macular cystic spaces in cross-section.
Fundus photo of the right eye in a patient with cystoid macular edema from diabetic retinopathy.
Fluorescein angiography of the right eye (late phase) showing central macular leakage in cystic spaces around the fovea.
Optical coherence tomography (OCT) of the right eye showing central macular cystic spaces in cross-section.
 
 
 
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