- •Retinal Vein Occlusions
- •Preface
- •Acknowledgments
- •Contents
- •1.1 Anatomy and Histology
- •1.2 Microanatomy of the Retina
- •1.3 Vascular Anatomy
- •Bernoulli’s Principle and Deductions Concerning Changes in Central Retinal Vein Diameter at the Lamina Cribrosa
- •1.4 Pathologic Anatomy
- •1.4.1 Abnormalities of the Vessel Wall
- •1.4.2 Branch Retinal Vein Occlusion
- •1.4.3 Central Retinal Vein Occlusion
- •1.4.4 Hemicentral Retinal Vein Occlusion
- •1.5 Summary of Key Points
- •References
- •2.1 Abnormalities of the Blood
- •2.1.1 Thrombosis
- •2.1.2 Viscosity of Blood
- •2.2 Abnormalities of Blood Flow
- •2.2.1 Retinal Vascular Hemodynamics
- •2.2.1.1 Laplace’s Law
- •2.2.1.2 Poiseuille’s Law
- •A Misapplication of Poiseuille’s Law
- •2.2.1.3 Hemodynamics of Central Retinal Vein Occlusion
- •How Severe Must Central Venous Obstruction Be to Produce Symptoms?
- •The Central Retinal Artery in Central Retinal Vein Occlusion
- •2.2.1.4 Hemodynamics of BRVO
- •2.3 Macular Edema
- •2.3.1 Macular Anatomy and Its Relationship to Macular Edema in Retinal Vein Occlusion
- •2.3.2 Starling’s Law
- •2.3.3 The Retinal Pigment Epithelial Pump
- •2.3.4 Molecular Signaling in Macular Edema
- •Relevant Molecular Biologic Terminology
- •2.3.4.1 Vascular Endothelial Growth Factor
- •2.3.4.2 Other Retinal Cytokines with Lesser Roles
- •2.3.4.3 Molecular Signaling in BRVO
- •2.3.4.4 Molecular Signaling in CRVO
- •What Does the Response of RVO to Intravitreal Anti-VEGF Drugs Say About Pathophysiology?
- •2.4 Retinal Neovascularization
- •Spontaneous Venous Pulsations and CRVO
- •2.7 Animal Models of Retinal Vein Occlusion
- •2.7.1 Animal Models of BRVO
- •2.7.2 Animal Models of CRVO
- •2.8 Summary of Key Points
- •2.9 Future Directions
- •References
- •3.1 Background for Clinical Genetics
- •3.2 The Role of Polymorphisms in Genetic Studies
- •3.3 Types of Genetic Study Design
- •Why Are So Many Association Studies for Retinal Vein Occlusion Negative?
- •3.4 Studies of the Genetics of Retinal Vein Occlusion
- •3.4.1 Platelet Glycoprotein Receptor Genes
- •3.4.2.1 Pooled Retinal Vein Occlusion
- •3.4.2.2 Central Retinal Vein Occlusion
- •3.4.2.3 Branch Retinal Vein Occlusion
- •3.4.4 202210G > A Mutation of the Prothrombin Gene (Factor II Leiden)
- •3.4.6 Protein C
- •3.4.7 Protein S
- •3.4.8 Fibrinogen
- •3.4.9 Factor XII
- •3.4.12 Other Negative Genetic Association Studies
- •3.5 Summary of Key Points
- •References
- •4.1 Nosology of Retinal Vein Occlusions
- •4.2 Branch Retinal Vein Occlusion
- •4.3 Central Retinal Vein Occlusion
- •Central Retinal Vein Occlusion with Nonischemic and Ischemic Hemispheres
- •4.3.1 Conversion from Nonischemic to Ischemic Forms of Retinal Vein Occlusion
- •4.4 Summary of Key Points
- •References
- •Quantifying Risk
- •The Major Epidemiologic Studies of Retinal Vein Occlusion
- •5.2 Prevalence
- •5.2.1 Pooled Retinal Vein Occlusion
- •5.2.2 Branch Retinal Vein Occlusion
- •5.2.3 Central Retinal Vein Occlusion
- •5.2.4 Hemicentral Retinal Vein Occlusion
- •5.3 Incidence
- •5.3.1 Pooled Retinal Vein Occlusion
- •5.3.2 Branch Retinal Vein Occlusion
- •5.3.3 Central Retinal Vein Occlusion
- •5.4 Risk and Protective Factors for Retinal Vein Occlusion
- •5.4.1.1 Pooled Retinal Vein Occlusion
- •5.4.1.2 Branch Retinal Vein Occlusion
- •5.4.1.3 Central Retinal Vein Occlusion
- •5.4.1.4 Hemicentral Retinal Vein Occlusion
- •5.4.2 Gender
- •5.4.2.1 Pooled Retinal Vein Occlusion
- •5.4.2.2 Branch Retinal Vein Occlusion
- •5.4.2.3 CRVO
- •5.4.2.4 Hemicentral Retinal Vein Occlusions
- •5.4.3 Race
- •5.4.4 Laterality
- •5.4.5 Body Mass Index
- •5.4.6 Education
- •5.4.7 Physical Activity
- •5.4.8 Miscellaneous Factors Explored and Not Found Important
- •5.5.1 Pooled Retinal Vein Occlusion
- •5.5.2 Branch Retinal Vein Occlusion
- •5.5.3 Central Retinal Vein Occlusion
- •5.5.4 Hemicentral Retinal Vein Occlusion
- •5.6 Life Expectancy
- •5.7 Visual Impact of Retinal Vein Occlusions
- •5.8 Summary of Key Points
- •References
- •6.1 Introduction
- •6.2 Systemic Associations
- •6.2.1 Hypertension
- •6.2.1.1 Pooled Retinal Vein Occlusions
- •6.2.1.2 Branch Retinal Vein Occlusion
- •6.2.1.3 Central Retinal Vein Occlusion
- •6.2.2 Diabetes Mellitus
- •6.2.2.1 Pooled Retinal Vein Occlusion
- •6.2.2.2 Branch Retinal Vein Occlusion
- •6.2.2.3 Central Retinal Vein Occlusion
- •6.2.3 Hyperlipidemia
- •6.2.3.1 Pooled Retinal Vein Occlusions
- •6.2.3.2 Branch Retinal Vein Occlusion
- •6.2.3.3 Central Retinal Vein Occlusion
- •6.2.4 Cardiovascular Disease
- •6.2.4.1 Pooled Retinal Vein Occlusion
- •6.2.4.2 Branch Retinal Vein Occlusion
- •6.2.4.3 Central and Hemicentral Retinal Vein Occlusion
- •6.2.4.4 Stroke
- •6.2.4.5 Carotid Artery Disease and Peripheral Vascular Disease
- •6.2.5 Rheologic and Hematologic Abnormalities
- •6.2.6 Coagulation Abnormalities
- •6.2.6.1 Antiphospholipid Antibodies
- •6.2.6.2 Factor VII
- •6.2.6.3 Factor VIII
- •6.2.6.4 Lipoprotein a
- •6.2.6.5 Von Willebrand Factor
- •6.2.6.6 Other Coagulation Factors
- •6.2.7 Hyperhomocysteinemia
- •6.2.7.1 Pooled Retinal Vein Occlusion
- •6.2.7.2 Branch Retinal Vein Occlusion
- •6.2.7.3 Central and Hemicentral Retinal Vein Occlusion
- •6.2.8 Serum Folate
- •6.2.9 Serum B12
- •6.2.10 Smoking
- •6.2.11 Alcohol Consumption
- •6.2.14 No Underlying Vascular Risk Factor
- •6.3 Ocular Associations
- •6.3.1 Pooled Retinal Vein Occlusion
- •6.3.2 Branch Retinal Vein Occlusion
- •6.3.3 Central Retinal Vein Occlusion and Hemicentral Retinal Vein Occlusion
- •6.4 Practical Recommendations About the Systemic Workup of Patients with Retinal Vein Occlusion
- •History of the Standard Workup for Systemic Associations in Central Retinal Vein Occlusion
- •6.5 Retinal Vein Occlusion and Cardiovascular Disease Risk
- •6.6 Differences in Systemic Associations Between Ischemic and Nonischemic CRVOs
- •6.7 Summary of Key Points
- •References
- •7.1 Branch Retinal Vein Occlusion
- •7.1.1 Acute Phase
- •7.1.1.1 Symptoms
- •7.1.2 Clinical Signs
- •7.1.2.1 Visual Acuity
- •7.1.3 Chronic Phase
- •7.1.3.1 Clinical Signs
- •7.1.3.2 Visual Acuity
- •Why Does the Visual Outcome in Nonischemic, Macula-Involving Branch Retinal Vein Occlusions Usually Vary with the Size of the Involved Retina?
- •7.2 Central Retinal Vein Occlusion
- •7.2.1 Acute Phase
- •7.2.1.1 Symptoms
- •7.2.1.2 Clinical Signs
- •When Retinal Venous Congestion and Optic Disc Edema Are Not Central Retinal Vein Occlusion
- •What Is the Relationship of Central Retinal Artery Pressure and Cilioretinal Artery Pressure?
- •Retinal Whitening Does Not Equal Infarction
- •A Clinical Picture Predicted by a Hypothesis
- •7.2.1.3 Visual Acuity
- •7.2.2 Chronic Phase
- •Why Are Optic Disc Collaterals Associated with Worse Initial and Final Visual Acuity After CRVO?
- •7.2.2.1 Visual Acuity
- •7.3 Hemicentral Retinal Vein Occlusion
- •7.3.1 Clinical Signs
- •7.3.2 Visual Acuity
- •7.4 Summary of Key Points
- •References
- •Which Measure of Reproducibility Is Best?
- •8.1 Color Fundus Photography
- •8.2 Fluorescein Angiography
- •8.2.1 Branch Retinal Vein Occlusion
- •8.2.2 Central Retinal Vein Occlusion
- •8.3 Optical Coherence Tomography and the Retinal Thickness Analyzer
- •Methods of Analysis of OCT in RVO
- •8.4 Visual Field Testing
- •8.5 Electroretinography
- •Electroretinography Essentials for Retinal Vein Occlusions
- •8.5.1 Branch Retinal Vein Occlusion
- •8.5.2 Central Retinal Vein Occlusion
- •8.5.3 Hemicentral Retinal Vein Occlusion
- •8.6 Indocyanine Green Angiography
- •8.7 Color Doppler Ultrasonographic Imaging
- •8.8 Laser Doppler Flowmetry
- •8.9 Ophthalmodynamometry
- •8.10 Scanning Laser Doppler Flowmetry
- •8.11 Laser Interferometry to Measure Pulsatile Choroidal Blood Flow
- •8.12 Vitreous Fluorophotometry
- •8.13 Summary of Key Points
- •References
- •9.1 Terminology
- •9.2 Branch Retinal Vein Occlusion
- •9.3 Central Retinal Vein Occlusion
- •9.3.1 Clinical Characteristics
- •In the Face of Evidence that Fluorescein Angiography Is Poorly Predictive of Ischemia in Acute Central Retinal Vein Occlusion, Why Is It Widely Used?
- •9.3.2 Conversion from Nonischemic to Ischemic Central Retinal Vein Occlusion
- •9.3.3 Outcomes by Ischemic Status
- •9.4 Interaction of Ischemia with Effects of Treatments
- •9.4.1 Branch Retinal Vein Occlusion
- •9.4.2 Central Retinal Vein Occlusion
- •9.5 Summary of Key Points
- •References
- •10.1 Branch Retinal Vein Occlusion
- •10.2 Central Retinal Vein Occlusion
- •10.3 Hemicentral Retinal Vein Occlusion
- •10.4 Treatment of Posterior Segment Neovascularization in Retinal Vein Occlusion
- •10.5 Summary of Key Points
- •References
- •11.1 The Pathoanatomy and Pathophysiology of Iris and Angle Neovascularization
- •11.2 Clinical Picture of Anterior Segment Neovascularization
- •11.4 Anterior Segment Neovascularization in Branch Retinal Vein Occlusion
- •11.5 Anterior Segment Neovascularization in Central Retinal Vein Occlusion
- •The Problem of Undetected Anterior Segment Neovascularization After Central Retinal Vein Occlusion
- •Why Is Anterior Segment Neovascularization Less Common in Central Retinal Vein Occlusion Than in Central Retinal Artery Occlusion?
- •11.6 Anterior Segment Neovascularization in Hemicentral Retinal Vein Occlusion
- •11.7 Summary of Key Points
- •References
- •12.1 Branch Retinal Vein Occlusion with Macular Edema
- •12.2 Central Retinal Vein Occlusion with Macular Edema
- •12.3 Summary of Key Points
- •References
- •Visual Acuity Measurement in Treatment Studies
- •OCT Measurement of Macular Thickness in Treatment Studies
- •13.1 Medical Treatment of Retinal Vein Occlusion
- •13.1.1 Anticoagulation
- •13.1.2 Systemic Thrombolytic Therapy
- •13.1.3 Isovolumic Hemodilution
- •Recipe for Isovolumic Hemodilution
- •13.1.4 Plasmapheresis
- •13.2 Treatment of Previously Unsuspected Risk Factors for Retinal Vein Occlusion
- •13.3.1 Treatments for Macular Edema
- •Relative Corticosteroid Potencies
- •13.3.2 Treatments for Intraocular Neovascularization
- •13.4 Results of Clinical Studies of Treatments for Macular Edema Secondary to Retinal Vein Occlusions
- •13.4.1 Branch Retinal Vein Occlusion
- •13.4.1.1 Grid Laser
- •13.4.1.2 Subthreshold Grid Laser Treatment
- •13.4.1.3 Sector Panretinal Laser Photocoagulation
- •13.4.1.5 Posterior Subtenon’s Triamcinolone
- •13.4.1.6 Intravitreal Corticosteroids
- •13.4.1.7 Combination Treatments Involving Intravitreal Triamcinolone Injections
- •13.4.1.8 Arteriovenous Sheathotomy
- •13.4.1.9 Vitrectomy
- •13.4.1.10 Intravitreal Injection of Autologous Plasmin
- •13.4.2 Central Retinal Vein Occlusion
- •13.4.2.2 Combination Regimen: Bevacizumab, Panretinal Laser, and Grid Laser
- •13.4.2.3 Systemic Corticosteroids
- •13.4.2.4 Posterior Subtenon’s Triamcinolone Injection
- •13.4.2.5 Intravitreal Corticosteroids
- •13.4.2.6 Vitrectomy
- •13.5 Treatment of Intraocular Neovascularization
- •13.5.1 Sector Panretinal Laser Photocoagulation for Retinal and Disc Neovascularization After Branch Retinal Vein Occlusion
- •13.5.2 Vitrectomy for Intraocular Neovascularization with Vitreous Hemorrhage
- •13.5.3 Laser Panretinal Photocoagulation for Anterior Segment Neovascularization
- •13.6 Economic Considerations
- •13.7 Future Directions
- •13.8 Summary of Key Points
- •References
- •14.1 Pooled Retinal Vein Occlusions in the Young
- •14.2 Branch Retinal Vein Occlusion in Younger Patients
- •14.3 Central Retinal Vein Occlusion in Younger Patients
- •14.4 Workup in the Younger Patient with Retinal Vein Occlusion
- •14.5 Summary of Key Points
- •References
- •15.1 Failed and Unadopted Treatments for Branch Retinal Vein Occlusion
- •15.1.1 Sector Panretinal Laser Photocoagulation for Serous Retinal Detachment in Branch Retinal Vein Occlusion
- •15.1.2 Laser Chorioretinal Venous Anastomosis for Branch Retinal Vein Occlusion with Macular Edema
- •15.1.3 Intravenous Infusion of Tissue Plasminogen Activator
- •15.1.4 Intravitreal Injection of Tissue Plasminogen Activator
- •15.1.5 Macular Puncture for Branch Retinal Vein Occlusion with Macular Edema
- •15.2 Failed and Unadopted Treatments for Central Retinal Vein Occlusion
- •15.2.1 Grid Laser for Macular Edema in Central Retinal Vein Occlusion
- •15.2.2 Chorioretinal Venous Anastomosis for Nonischemic Central Retinal Vein Occlusion with Macular Edema
- •15.2.3 Radial Optic Neurotomy for Central Retinal Vein Occlusion
- •15.2.4 Retinal Endovascular Surgery with Intravenous Injection of Tissue Plasminogen Activator
- •15.2.5 Intravitreal Injection of Tissue Plasminogen Activator
- •15.2.6 Intravitreal Tissue Plasminogen Activator and Triamcinolone
- •15.2.7 Systemic Acetazolamide for Central Retinal Vein Occlusion with ME
- •15.2.8 Combined Central Retinal Vein Occlusion and Central Retinal Artery Occlusion
- •15.2.9 Optic Nerve Sheath Decompression
- •15.2.10 Section of the Posterior Scleral Ring
- •15.2.11 Infusion of High Molecular Weight Dextran
- •15.3 Failed and Unadopted Treatments for HCRVO
- •15.4 Summary of Key Points
- •References
- •16.1 Case 16.1: An Asymptomatic Central Retinal Vein Occlusion with Asymmetric Hemispheric Involvement
- •16.1.1 Discussion
- •16.2 Case 16.2: Chronic Macular Branch Vein Occlusion with Subtle Ophthalmoscopic Signs, More Obvious Fluorescein Angiographic Signs, and Macular Edema
- •16.2.1 Discussion
- •16.3 Case 16.3: Old Hemicentral Retinal Vein Occlusion with Late Vitreous Hemorrhage and Hyphema
- •16.3.1 Discussion
- •16.4 Case 16.4: Spontaneous Improvement of a Nonischemic Central Retinal Vein Occlusion
- •16.4.1 Discussion
- •16.5 Case 16.5: Conversion of a Nonischemic Hemicentral Retinal Vein Occlusion to an Ischemic One
- •16.5.1 Discussion
- •16.6 Case 16.6: Nonarteritic Ischemic Optic Neuropathy Following Branch Retinal Vein Occlusion
- •16.6.1 Discussion
- •16.7 Case 16.7: Differentiating Central Retinal Vein Occlusion from the Ischemic Ocular Syndrome
- •16.7.1 Discussion
- •16.8 Case 16.8: Late Development of Neovascularization Elsewhere After Ischemic Branch Retinal Vein Occlusion
- •16.8.1 Discussion
- •16.9 Case 16.9: Nonischemic Central Retinal Vein Occlusion with Secondary Branch Retinal Artery Occlusion
- •16.9.1 Discussion
- •16.10 Case 16.10: Nonischemic Central Retinal Vein Occlusion with Macular Edema or Asymmetric Diabetic Retinopathy with Diabetic Macular Edema?
- •16.10.1 Discussion
- •16.11 Summary of Key Points
- •References
- •Index
362 |
16 Case Studies in Retinal Vein Occlusion |
R C S |
Thrombus |
D
A
Pia
PR
OD |
ON |
LC
SAS
CAR CRV
Fig. 16.2 Diagram to attempt to describe a plausible pathoanatomy for the clinical situation depicted in case 16.1. The central retinal vein (CRV) has two trunks entering the optic disc. The thrombus involves both hemicentral retinal veins, but extends closer to the lamina cribrosa (LC) in the case of the superior hemicentral retinal vein. For this reason, there are fewer collateral branches (the white arrows indicating collaterals) to decompress the
obstructed vein leading to dilation of the few collaterals that exist, producing the clinical picture of disc collaterals. There are more collaterals for the more posteriorly obstructed inferior hemicentral retinal vein. They decompress the obstructed inferior hemicentral vein more successfully, thus there are no disc collaterals and there are fewer inferior intraretinal hemorrhages (redrawn and adapted from Hayreh9)
This case exhibits one of the clinical pearls that retina specialists learn. When a patient has blurred vision, a small relative central scotoma, and no obvious fundus abnormalities, it is worthwhile to look closely for signs of an old macular BRVO. The OCT and FA can be helpful by showing more evidence than the clinical examination.
16.3Case 16.3: Old Hemicentral Retinal Vein Occlusion with Late Vitreous Hemorrhage and Hyphema
An 85-year-old man with type 2 diabetes mellitus, hypercholesterolemia, and a cigarette smoking habit was seen in 2003 with blurred vision OD due to an inferior hemicentral retinal vein
occlusion (HCRVO) with ME. Best corrected VA (BCVA) was 20/400 in the right eye and 20/50 in the left eye. The ME was treated with an intravitreal triamcinolone injection (IVTI) of the right eye with improvement in VA to 20/80 and reduction in central subfield mean thickness (CSMT) from 568 to 202 m. Because he was not able to appreciate this objective improvement in BCVA and CSMT as improved visual quality of life, he declined further injections. The cataract of the right eye progressed after the IVTI, and he underwent uneventful cataract surgery with posterior chamber intraocular lens implantation in 2005. Six periodic examinations between 2005 and 2010 revealed no changes of the right eye until he presented on 11/10/2010 with a history of overnight VA loss in the right eye. The VA of the right eye was reduced to light perception, the intraocular pressure was 35, and both hyphema and vitreous
16.3 |
Case 16.3: Old Hemicentral Retinal Vein Occlusion with Late Vitreous Hemorrhage and Hyphema |
363 |
a |
b |
|
c |
d |
e
Fig. 16.3 Images from a patient with a chronic macular branch retinal vein occlusion and ME. (a) Color fundus photograph showing a single microaneurysm superonasal to the center of the macula (the black arrow). (b) Frame from the mid-phase FA of the left eye. More abnormalities are apparent than could be seen clinically or on the color fundus photograph. The microaneurysm is indicated by the yellow arrow. (c) A magnified portion of the frame in (b) shows two microaneurysms (the yellow arrows) and
an intraretinal collateral vessel (the green arrow). (d) A spectral domain OCT line scan showing a cyst (the yellow arrow) involving the center of the macula. (e) Frame from the late-phase FA showing minimal late hyperfluorescence involving the superior hemimacula (surrounded by yellow oval). This is not a retinal pigment epithelial window defect as can be seen by the absence of hyperfluorescence in the superior hemimacula in the earlier phase of the FA (compare to b)
