History

The Eye Disease Case-Control Study reported the following findings^[16]:

Systemic hypertension is a risk factor for branch retinal vein occlusion (BRVO).
Diabetes mellitus and open-angle glaucoma are not risk factors for BRVO.
Moderate alcohol consumption reduces the risk for BRVO.

Patients often complain of a sudden painless decrease of vision in the affected eye.

Some patients may complain of a scotoma.

Physical

In 1877, Leber first described the branch retinal vein occlusion ophthalmoscopically. During the acute phase, intraretinal hemorrhages (usually flame shaped), retinal edema, and cotton-wool spots are seen in the distribution of a retinal vessel. The horizontal raphe is respected. Intraretinal hemorrhages are shown in the image below.

This 42-year-old woman with hypertension noticed a sudden decrease in her vision. Visual acuity was 20/100. Note the intraretinal hemorrhages in just one segment of the retina.

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During the chronic stage, hemorrhages may be absent. Macular edema may be the only sign present. Telangiectatic vessels that extend across the horizontal raphe usually can be demonstrated angiographically.

Exudative retinal detachment is an infrequent complication of a BRVO. It is characterized by layered subretinal blood in the inferior portion of the detachment, very few collateral vessels, and capillary nonperfusion.^[17]

In certain eyes with large areas of nonperfusion, retinal neovascularization may be seen. Vitreous hemorrhage with tractional retinal detachments may ensue. Further traction may create retinal breaks, creating combined rhegmatogenous and tractional retinal detachments. Neovascular glaucoma and neovascularization at the disc are rare events with BRVO.

Causes

Most cases of BRVO are due to idiopathic factors. Usually, patients have an anatomic predisposing factor, such as an arteriovenous crossing where the artery compresses the vein. This compression leads to clot formation and subsequent BRVO.

Inflammatory conditions that affect the retinal veins may cause local damage that predisposes the individual to intravascular clot formation with subsequent BRVO.

Some of the inflammatory conditions reported in the literature are the following:

Sarcoidosis
Lyme disease
Serpiginous choroiditis
Arterial hypertension and hypercholesterolemia, both of which contribute to atherogenesis, have been identified as risk factors for BRVO, as follows:
- Atherosclerosis itself has recently been recognized as a chronic low-grade inflammatory disease with a distinct proinflammatory cytokine pattern. In addition to their role in atherogenesis, some cytokines have been shown to exert procoagulatory effects and may thus contribute to the development of BRVO by a second mechanism.
- Gene polymorphisms affecting the expression of inflammation-related cytokines are candidates as potential risk factors for BRVO. Genotypes of the following functional single nucleotide polymorphisms were determined: interleukin 1 beta (IL-1B) -511C>T, interleukin 1 receptor antagonist (IL-1RN) 1018T>C, interleukin 4 (IL-4) -584C>T, interleukin 6 (IL-6) -174G>C, interleukin 8 (IL-8) -251A>T, interleukin 10 (IL-10) -592C>A, interleukin 18 (IL-18) 183A>G, tumor necrosis factor (TNF) -308G>A, monocyte chemoattractant protein 1. Neither genotype distributions nor allele frequencies of any of the investigated polymorphisms differed significantly between patients with BRVO and controls.^[18]

Thrombophilic conditions, such as the following, may also be involved:

Protein S deficiency
Protein C deficiency
Resistance to activated protein C (factor V Leiden)
Antithrombin III deficiency
Antiphospholipid antibody syndrome
Lupus erythematosus
Gammopathies
Gene polymorphisms related to hemostasis might also contribute to the development of BRVO. Most studies, but not all, failed to detect an association between these genetic variants and BRVO.

Complications

Potential complications of BRVO include the following:

Macular edema
Retinal neovascularization
- Vitreous hemorrhage
- Tractional retinal detachment (these complications are shown in the image below)
  
  Patient with a branch retinal vein occlusion complicated by vitreous hemorrhage and tractional retinal detachment. The patient had undergone vitrectomy and endolaser treatment. Note the sclerotic supertemporal vein.
  View Media Gallery
- Rubeosis iridis
Epiretinal membrane

Differential Diagnoses

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Media Gallery

Patient with an old branch retinal vein occlusion in which the hemorrhages have cleared. Note lipid exudation and evidence of cystoid macular edema.
Patient with a branch retinal vein occlusion complicated by vitreous hemorrhage and tractional retinal detachment. The patient had undergone vitrectomy and endolaser treatment. Note the sclerotic supertemporal vein.
This 42-year-old woman with hypertension noticed a sudden decrease in her vision. Visual acuity was 20/100. Note the intraretinal hemorrhages in just one segment of the retina.
Arterial phase of an angiogram demonstrates the lack of filling in the first branch arteriole of the superior temporal artery.
Late phase of an angiogram demonstrates late leakage in the macular area.
Optical coherence tomography (OCT) of a patient with macular edema secondary to branch retinal vein occlusion (BRVO) prior to the initiation of anti-VEGF therapy. The visual acuity was 20/150.
Optical coherence tomography (OCT) following 3 monthly intravitreal bevacizumab injections (1.25 mg). The visual acuity improved to 20/30.

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Author

Lihteh Wu, MD Ophthalmologist, Costa Rica Vitreo and Retina Macular Associates

Lihteh Wu, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, Club Jules Gonin, Macula Society, Pan-American Association of Ophthalmology, Retina Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Bayer; Quantel Medical, Roche; Iveric Bio<br/>Received income in an amount equal to or greater than $250 from: Bayer Health; Quantel Medical, Roche, Iveric Bio.

Coauthor(s)

Mitchell Pagano Haverford College

Disclosure: Nothing to disclose.

Diana Monge-Lopez, MBBS Research Fellow, Instituto de Cirugía Ocular, Costa Rica

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steve Charles, MD Founder and CEO of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

Chief Editor

Douglas R Lazzaro, MD, FAAO, FACS Chairman, Professor of Ophthalmology, The Richard C Troutman, MD, Distinguished Chair in Ophthalmology and Ophthalmic Microsurgery, Department of Ophthalmology, State University of New York Downstate Medical Center; Chief of Ophthalmology, Director of Cornea, Director of Surgical Training, Kings County Hospital Center

Douglas R Lazzaro, MD, FAAO, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, Association of University Professors of Ophthalmology, Brooklyn Ophthalmological Society, Cornea Society, New York Society for Clinical Ophthalmology, Ophthalmic Laser Surgical Society

Disclosure: Nothing to disclose.

Additional Contributors

V Al Pakalnis, MD, PhD Professor of Ophthalmology, University of South Carolina School of Medicine; Chief of Ophthalmology, Dorn Veterans Affairs Medical Center

V Al Pakalnis, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, South Carolina Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Teodoro Evans, MD Consulting Surgeon, Vitreo-Retinal Section, Clinica de Ojos, Costa Rica

Disclosure: Nothing to disclose.