BOOK
Retina E-Book
Stephen J. Ryan | Andrew P. Schachat | Charles P. Wilkinson | David R. Hinton | Peter Wiedemann | SriniVas R. Sadda
(2012)
Additional Information
Book Details
Abstract
Unequalled in scope, depth, and clinical precision, Retina, 5th Edition keeps you at the forefront of today’s new technologies, surgical approaches, and diagnostic and therapeutic options for retinal diseases and disorders. Comprehensively updated to reflect everything you need to know regarding retinal diagnosis, treatment, development, structure, function, and pathophysiology, this monumental ophthalmology reference work equips you with expert answers to virtually any question you may face in practice.
- Consult this title on your favorite e-reader with intuitive search tools and adjustable font sizes. Elsevier eBooks provide instant portable access to your entire library, no matter what device you're using or where you're located.
- Examine and evaluate the newest diagnostic technologies and approaches that are changing the management of retinal disease, including future technologies which will soon become the standard.
- Put the very latest scientific and genetic discoveries, diagnostic imaging methods, drug therapies, treatment recommendations, and surgical techniques to work in your practice.
- Benefit from the extensive knowledge and experience of esteemed editor Dr. Stephen Ryan, five expert co-editors, and a truly global perspective from 358 other world authorities across Europe, Asia, Australasia, and the Americas.
- Make the best use of new technologies with expanded and updated coverage of optical coherence tomography (OCT), fundus imaging, and autofluorescence imaging.
- Apply the latest knowledge on anti-VEGF therapy for age related macular degeneration, diabetic retinopathy and vein disease.
- Learn about artificial vision, drug delivery to the posterior segment, advances in macular surgery, vitrectomy, and complex retinal detachment, with updates on tumors, retinal genetics, cell biology, important basic science topics, and much more.
- Get the most out of new pharmacologic approaches in the management of age-related macular degeneration and diabetic retinopathy.
- In your practice, diagnostic evaluations, and now even treatments, will be influenced by recent scientific discoveries such as in the areas of nanotechnology, neuro protection, stem cells and gene therapy, among other scientific contributions.
- View videos of surgical procedures and access the complete contents of Retina, 5th Edition online at www.expertconsult.com, fully searchable, with regular updates and a downloadable image gallery.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front cover | cover | ||
| Half title page | i | ||
| Retina | ii | ||
| Copyright page | iv | ||
| Table of Contents | v | ||
| Video Table of Contents | ix | ||
| Contributors | xi | ||
| List of Video Contributors | xxiii | ||
| Dedication | xxv | ||
| Foreword | xxvi | ||
| Preface | xxvii | ||
| 1 Retinal Imaging and Diagnostics | 1 | ||
| Section 1: Optical Imaging Technologies | 2 | ||
| 1 Fluorescein Angiography:Basic Principles and Interpretation | 2 | ||
| Basic principles | 2 | ||
| Fluorescence | 2 | ||
| Pseudofluorescence | 3 | ||
| Equipment | 3 | ||
| Film-based versus digital fluorescein angiography – historical perspectives | 3 | ||
| Camera and auxiliary equipment | 3 | ||
| Stereophotography | 4 | ||
| Matched fluorescein filters | 4 | ||
| Light sources (viewing bulb and flash strobe) | 4 | ||
| Fluorescein solution | 4 | ||
| Technique | 6 | ||
| Aligning camera and photographing | 6 | ||
| Focusing | 6 | ||
| Digital angiography | 7 | ||
| Using stereophotography | 8 | ||
| Photographing the periphery | 8 | ||
| Informing the patient | 8 | ||
| Positioning the patient | 9 | ||
| Injecting the fluorescein | 9 | ||
| Developing a photographic plan | 9 | ||
| Diabetic retinopathy | 11 | ||
| Interpretation | 11 | ||
| Fundus anatomy and histology | 11 | ||
| Normal fluorescein angiogram | 14 | ||
| Abnormal fluorescein angiogram | 16 | ||
| Hypofluorescence | 16 | ||
| Anatomic location of hypofluorescence | 18 | ||
| Blocked retinal fluorescence | 18 | ||
| Blocked choroidal fluorescence | 19 | ||
| Deep retinal material | 19 | ||
| Subretinal material | 20 | ||
| Vascular filling defect | 21 | ||
| Retinal vascular filling defect | 23 | ||
| Vascular filling defects of the disc | 25 | ||
| Choroidal vascular filling defect | 25 | ||
| Hyperfluorescence | 26 | ||
| Preinjection fluorescence | 27 | ||
| Autofluorescence | 27 | ||
| Transmitted fluorescence (pigment epithelial window defect) | 29 | ||
| Abnormal retinal and disc vessels | 30 | ||
| Abnormal choroidal vessels | 31 | ||
| Leak | 38 | ||
| Vitreous leak | 39 | ||
| Disc leak | 39 | ||
| Papilledema and optic disc edema | 39 | ||
| Retinal leak | 39 | ||
| Choroidal leak | 42 | ||
| Staining | 49 | ||
| Drusen | 49 | ||
| Scar | 49 | ||
| Sclera | 49 | ||
| Acknowledgments | 50.e1 | ||
| References | 50 | ||
| 2 Clinical Applications of Diagnostic Indocyanine Green Angiography | 51 | ||
| Introduction | 51 | ||
| History | 51 | ||
| Chemical and pharmacokinetics | 51 | ||
| Toxicity | 52 | ||
| Instrument comparison | 52 | ||
| Injection technique | 53 | ||
| Indocyanine green angiography interpretation | 54 | ||
| Normal eye | 54 | ||
| Exudative age-related macular degeneration | 54 | ||
| Type 1 choroidal neovascularization | 54 | ||
| Type 2 choroidal neovascularization | 57 | ||
| Type 3 choroidal neovascularization | 57 | ||
| Polypoidal choroidal vasculopathy | 61 | ||
| Central serous chorioretinopathy | 64 | ||
| Choroidal tumors | 66 | ||
| Choroidal hemangioma | 66 | ||
| Choroidal melanoma | 68 | ||
| Peripheral exudative hemorrhagic chorioretinopathy | 68 | ||
| Varix of the vortex vein ampulla | 70 | ||
| Choroidal inflammation and white-dot syndrome | 70 | ||
| Multiple evanescent white-dot syndrome | 70 | ||
| Multifocal choroiditis | 70 | ||
| Birdshot chorioretinopathy | 70 | ||
| Acute multifocal placoid pigment epitheliopathy | 77 | ||
| Serpiginous choroidopathy | 77 | ||
| Punctate inner chorioretinopathy | 77 | ||
| Acute zonal occult outer retinopathy | 77 | ||
| Conclusions | 79 | ||
| References | 79 | ||
| 3 Optical Coherence Tomography | 82 | ||
| Physical principles of optical coherence tomography | 82 | ||
| Quantitative analysis of OCT datasets | 83 | ||
| Normal macular anatomy | 85 | ||
| SD-OCT IN retinal disorders | 87 | ||
| Vitreoretinal interface disorders | 87 | ||
| Vitreomacular traction | 87 | ||
| Epiretinal membrane | 88 | ||
| Macular hole | 89 | ||
| Age-related macular degeneration | 90 | ||
| Non-neovascular AMD (see Chapter 65, Dry AMD – diagnosis and treatment) | 90 | ||
| Early non-neovascular AMD: drusen and pigmentary changes | 90 | ||
| Late non-neovascular AMD: geographic atrophy | 92 | ||
| Neovascular AMD (see Chapter 66, Wet AMD – diagnosis and treatment) | 94 | ||
| Intraretinal and subretinal fluid | 94 | ||
| Retinal pigment epithelium detachment | 94 | ||
| Tear of the retinal pigment epithelium | 95 | ||
| Disciform scarring | 96 | ||
| Retinal angiomatous proliferation | 97 | ||
| Polypoidal choroidal vasculopathy | 98 | ||
| Choroidal neovascularization: response to treatment | 98 | ||
| Central serous chorioretinopathy | 98 | ||
| Enhanced depth imaging OCT IN CSC | 100 | ||
| Cystoid macular edema | 101 | ||
| Diabetic retinopathy | 101 | ||
| Nonproliferative diabetic retinopathy and diabetic macular edema | 101 | ||
| Proliferative diabetic retinopathy | 103 | ||
| Retinal vein occlusion | 103 | ||
| Central retinal artery occlusion | 104 | ||
| Branch retinal artery occlusion | 105 | ||
| Future directions | 106 | ||
| Disclosures | 106 | ||
| References | 106 | ||
| 4 Autofluorescence Imaging | 111 | ||
| Basic principles | 111 | ||
| Fundus autofluorescence | 111 | ||
| Retinal pigment epithelium and lipofuscin | 111 | ||
| Near-infrared autofluorescence | 111 | ||
| Macular pigment imaging | 111 | ||
| Techniques of fundus autofluorescence imaging | 112 | ||
| Fundus spectrophotometer | 112 | ||
| Scanning laser ophthalmoscopy | 112 | ||
| Fundus camera | 112 | ||
| Wide-field imaging | 113 | ||
| Interpretation of fundus autofluorescence images | 114 | ||
| Clinical applications | 115 | ||
| Age-related macular degeneration | 115 | ||
| Early AMD | 115 | ||
| Geographic atrophy | 117 | ||
| Pigment epithelium detachment | 118 | ||
| Choroidal neovascularization | 118 | ||
| Macular and diffuse retinal dystrophies | 121 | ||
| Macular telangiectasia | 123 | ||
| Pseudoxanthoma elasticum | 128 | ||
| Central serous chorioretinopathy | 129 | ||
| Chloroquine and hydroxychloroquine retinopathy | 129 | ||
| Functional correlates of FAF abnormalities | 129 | ||
| References | 131 | ||
| 5 Advanced Imaging Technologies | 133 | ||
| Introduction – retinal imaging to date | 133 | ||
| Adaptive optics – imaging of single cells in the retina | 135 | ||
| Basic principles | 135 | ||
| Technology | 135 | ||
| Visualization of retinal structures | 137 | ||
| Early clinical applications | 138 | ||
| Conclusions | 139 | ||
| Doppler imaging – assessment of blood flow | 139 | ||
| Basic principles | 139 | ||
| Non-Doppler assessment of retinal blood flow | 139 | ||
| Doppler ultrasound | 139 | ||
| Laser Doppler velocimetry | 140 | ||
| Laser Doppler flowmetry | 141 | ||
| Doppler optical coherence tomography | 141 | ||
| Conclusions | 141 | ||
| Spectral imaging – assessment of retinal oxygenation | 141 | ||
| Basic principles | 141 | ||
| Technology | 143 | ||
| Clinical applications | 144 | ||
| Conclusions | 144 | ||
| Photoacoustic imaging – assessment of retinal absorption | 144 | ||
| Basic principles | 144 | ||
| Technology | 144 | ||
| Conclusions | 145 | ||
| Magnetic resonance imaging | 145 | ||
| Basic principles | 145 | ||
| Retinal imaging | 145 | ||
| Conclusions | 146 | ||
| Nanotechnology | 146 | ||
| Basic principles | 146 | ||
| Iron oxide nanoparticles | 146 | ||
| Gold nanoparticles | 147 | ||
| Quantum dots | 147 | ||
| Conclusions | 148 | ||
| Conclusions and future directions | 148 | ||
| Disclosure | 149 | ||
| References | 149 | ||
| 6 Image Processing | 151 | ||
| Introduction | 151 | ||
| History of retinal imaging | 151 | ||
| History of retinal image processing | 152 | ||
| Current status of retinal imaging | 152 | ||
| Fundus imaging | 152 | ||
| Optical coherence tomography imaging | 152 | ||
| Time domain OCT | 154 | ||
| Frequency domain OCT | 154 | ||
| Spectral domain OCT | 154 | ||
| Swept source OCT | 154 | ||
| Areas of active research in retinal imaging | 154 | ||
| Portable, cost-effective fundus imaging | 154 | ||
| Functional imaging | 154 | ||
| Adaptive optics | 155 | ||
| Longer-wavelength OCT imaging | 155 | ||
| Clinical applications of retinal imaging | 155 | ||
| Early detection of diabetic retinopathy | 155 | ||
| Early detection of systemic disease from fundus photography | 155 | ||
| Image-guided therapy for retinal diseases with 3D OCT | 155 | ||
| Image analysis concepts for clinicians | 156 | ||
| The retinal image | 156 | ||
| Definition of a retinal image | 156 | ||
| Retinal image quantities | 156 | ||
| Retinal image compression | 156 | ||
| Lossy image compression | 156 | ||
| Legal issues with lossy image compression | 156 | ||
| Storing and accessing retinal images: ophthalmology picture-archiving systems | 156 | ||
| Different strategies for storing ophthalmic images | 157 | ||
| Digital exchange of retinal images and DICOM | 157 | ||
| Retinal image analysis | 157 | ||
| Common image-processing steps | 157 | ||
| Preprocessing | 157 | ||
| Detection | 157 | ||
| Segmentation | 158 | ||
| Registration | 158 | ||
| Interpretation | 158 | ||
| Unsupervised and supervised image analysis | 158 | ||
| Pixel feature classification | 158 | ||
| Measuring performance of image analysis algorithms | 158 | ||
| Sensitivity and specificity | 158 | ||
| Receiver operator characteristics | 159 | ||
| Repeatability and variability | 159 | ||
| The reference standard or gold standard | 159 | ||
| Clinical safety relevant performance measurement | 159 | ||
| Fundus image analysis | 159 | ||
| Detection of retinal vessels | 160 | ||
| Detection of fovea and optic disc | 160 | ||
| Detection of retinal lesions | 161 | ||
| Vessel analysis | 163 | ||
| Retinal atlas | 163 | ||
| Performance of DR detection algorithms | 166 | ||
| Areas of active research in fundus image analysis | 168 | ||
| OPTICAL COHERENCE TOMOGRAPHY image analysis | 168 | ||
| Retinal layer analysis from 3D OCT | 169 | ||
| Retinal layer detection | 169 | ||
| OCT image flattening | 169 | ||
| Retinal layer thickness analysis | 169 | ||
| Retinal texture analysis | 169 | ||
| Detection of retinal vessels from 3D OCT | 170 | ||
| Detection of retinal lesions | 170 | ||
| Fluid detection and segmentation | 170 | ||
| Fluid segmentation in 3D | 171 | ||
| Intraretinal layer segmentation in the presence of SEADs | 171 | ||
| Multimodality retinal imaging | 171 | ||
| Registration of fundus retinal photographs | 172 | ||
| Registration of OCT with fundus retinal photographs | 172 | ||
| Mutual registration of 3D OCT images | 172 | ||
| Future of retinal imaging and image analysis | 173 | ||
| References | 173 | ||
| Section 2: Retinal Diagnostics | 177 | ||
| 7 Electrogenesis of the Electroretinogram | 177 | ||
| Introduction | 177 | ||
| Generation of extracellular potentials: general concepts | 178 | ||
| Spatial buffering by glial cells | 179 | ||
| Approaches for determining the origins of the electroretinogram | 180 | ||
| Intraretinal depth recordings | 180 | ||
| Correlation of ERG with single-cell recordings | 180 | ||
| Pharmacologic dissection | 180 | ||
| Site-specific lesions/pathology or targeted mutations | 180 | ||
| Modeling of cellular responses and ERG components | 180 | ||
| Standard ERG tests in the clinic | 181 | ||
| Distal retinal components: slow PIII, C-wave, fast oscillation trough, and light peak | 181 | ||
| c-Wave | 182 | ||
| Müller cell contribution (slow PIll) | 183 | ||
| Distal versus proximal PIII | 183 | ||
| Retinal pigment epithelial component | 183 | ||
| The fast oscillation trough | 184 | ||
| The light peak | 184 | ||
| Origin of the A-wave | 184 | ||
| The a-wave as a reflection of rod and cone receptor photocurrent | 184 | ||
| Postreceptoral contributions to the a-wave | 185 | ||
| The timecourse of the photoreceptor response | 187 | ||
| Origin of the b-wave | 188 | ||
| Müller cell hypothesis | 189 | ||
| ON bipolar cells as the generator of the b-wave | 190 | ||
| Scotopic b-wave (PII) in mammals | 190 | ||
| Cone-driven b-wave | 191 | ||
| Origin of the d-wave | 191 | ||
| Photopic hill | 192 | ||
| Origin of the photopic fast-flicker ERG | 192 | ||
| Origin of the multifocal ERG | 192 | ||
| ERG waves from proximal retina | 192 | ||
| Origin of the proximal negative response and the M-wave | 192 | ||
| Origin of the photopic negative response | 193 | ||
| Relation to the pattern ERG | 195 | ||
| Origin of the scotopic threshold response | 195 | ||
| K+ Müller cell mechanism for generation of the STR | 196 | ||
| Neuronal origins of the STR | 196 | ||
| Origin of oscillatory potentials | 196 | ||
| Do all the OPs have the same origin? | 196 | ||
| Which cells generate the OPs? | 197 | ||
| What mechanisms are involved in generating OPs? | 198 | ||
| Neuronal interaction; inhibitory feedback circuits | 198 | ||
| OPs in intracellular responses from neurons | 198 | ||
| Closing remarks | 198 | ||
| References | 199 | ||
| 8 Clinical Electrophysiology | 202 | ||
| Standard full-field ERG | 202 | ||
| Stimulus and recording devices | 202 | ||
| Stimulus intensity versus ERG responses and components | 202 | ||
| Scotopic condition | 202 | ||
| Photopic condition | 203 | ||
| Bright flash mixed rod–cone ERG | 203 | ||
| Normal | 203 | ||
| Selectively abnormal oscillatory potentials | 204 | ||
| Subnormal | 204 | ||
| Negative | 205 | ||
| Prognostic value | 205 | ||
| Diagnostic value | 207 | ||
| Extinct | 208 | ||
| Isolation of rod and cone components in standardized ERG | 208 | ||
| Cone photoreceptor dysfunction | 208 | ||
| Rod photoreceptor dysfunction | 209 | ||
| Rod–cone or cone–rod photoreceptor dystrophy | 210 | ||
| Second-order neuron dysfunction | 211 | ||
| Focal ERG | 214 | ||
| Principle, method, and characteristics | 215 | ||
| Clinical applications | 216 | ||
| Other special responses or techniques in ERG | 216 | ||
| Pattern ERG | 216 | ||
| Photopic negative response | 220 | ||
| ERG recordings by LED | 220 | ||
| ERG recording under general anesthesia | 220 | ||
| ERG monitoring during eye surgery | 220 | ||
| S-Cone ERG | 222 | ||
| Electro-Oculogram | 222 | ||
| Visual Evoked Potential | 225 | ||
| References | 225 | ||
| 9 Diagnostic Ophthalmic Ultrasound | 227 | ||
| Introduction | 227 | ||
| Ultrasound – past and present | 227 | ||
| Examination techniques | 227 | ||
| A-mode technique | 228 | ||
| B-mode technique | 228 | ||
| High-frequency ultrasound technique | 228 | ||
| Doppler ultrasound | 229 | ||
| Ultrasound biometry | 229 | ||
| Three-dimensional reconstructions | 229 | ||
| Ultrasound in intraocular pathology | 230 | ||
| Changes in the shape of the globe | 230 | ||
| Staphyloma | 230 | ||
| Scleral buckle | 230 | ||
| Microphthalmos | 232 | ||
| Phthisis | 232 | ||
| Vitreous | 232 | ||
| Vitreous degeneration | 232 | ||
| Asteroid hyalosis | 232 | ||
| Synchysis scintillans | 233 | ||
| Persistent and hyperplastic primary vitreous | 233 | ||
| Vitreous hemorrhages | 235 | ||
| Vitreous hemorrhage from neovascularization | 236 | ||
| Terson syndrome | 239 | ||
| Intraocular infections | 239 | ||
| Vitreous inflammation | 239 | ||
| Intraocular foreign bodies | 243 | ||
| Retina | 243 | ||
| Acute retinal detachment | 243 | ||
| Chronic retinal detachment | 250 | ||
| Retinoschisis | 251 | ||
| Coats disease | 251 | ||
| Retinoblastoma | 255 | ||
| Retinopathy of prematurity | 255 | ||
| Optic nerve | 255 | ||
| Coloboma of the ocular fundus | 255 | ||
| Assessment of optic nerve cupping | 255 | ||
| Choroid | 258 | ||
| Changes in the ocular layers due to hypotony | 258 | ||
| Choroidal neovascularization | 258 | ||
| Choroidal melanoma | 258 | ||
| The characteristics of a choroidal melanoma on B-mode echography | 263 | ||
| The characteristics of a choroidal melanoma on A-mode ultrasonography | 263 | ||
| Determining the volume of a choroidal melanoma by ultrasonography | 267 | ||
| The role of ultrasonography for planning the treatment of choroidal melanomas | 271 | ||
| Metastatic choroidal tumors | 274 | ||
| Choroidal hemangioma | 275 | ||
| Choroidal osteoma – metastatic calcifications | 275 | ||
| Choroidal tuberculoma | 275 | ||
| The uveal effusion syndrome | 279 | ||
| Sclera | 279 | ||
| Posterior scleritis | 279 | ||
| Ultrasound imaging used to differentiate ocular disease | 279 | ||
| Future developments | 283 | ||
| Acknowledgments | 283.e1 | ||
| References | 283 | ||
| 10 Color Vision and Night Vision | 285 | ||
| Overview | 285 | ||
| Rod and cone functions | 285 | ||
| Light adaptation | 286 | ||
| Spectral sensitivity | 286 | ||
| Spatial and temporal resolution | 287 | ||
| Visual pathways for rod and cone functions | 287 | ||
| Retinal pathways | 287 | ||
| Retinogeniculate pathways | 287 | ||
| Dark adaptation functions: assessment of the shift from day vision to night vision | 288 | ||
| Clinical evaluation using dark adaptation functions | 288 | ||
| Color vision | 289 | ||
| Color matching | 289 | ||
| Color matching as the foundation for the theory of trichromacy | 289 | ||
| Color-matching experimental techniques and data | 289 | ||
| The CIE colorimetric system | 289 | ||
| Cone chromaticity space | 290 | ||
| Chromatic discrimination | 290 | ||
| Wavelength discrimination | 290 | ||
| Purity discrimination | 290 | ||
| Chromaticity discrimination | 291 | ||
| Color appearance | 291 | ||
| Variations in human color vision | 291 | ||
| Color vision classifications | 292 | ||
| The genes encoding the human photopigments | 292 | ||
| Clinical evaluation of color vision | 293 | ||
| Screening tests | 293 | ||
| Pseudoisochromatic plate tests | 293 | ||
| Other rapid tests of color vision | 293 | ||
| Chromatic discrimination ability tests | 293 | ||
| Importance of the test illuminant for plate and discrimination color vision tests | 293 | ||
| Color-matching tests | 293 | ||
| Anomaloscope color matching test using the Rayleigh equation | 294 | ||
| Anomaloscope color-matching test using the Moreland equation | 296 | ||
| Considerations in the use of anomaloscopes | 297 | ||
| Computerized color vision tests | 297 | ||
| Color assessment and diagnosis (CAD) test | 297 | ||
| Cambridge color test (CCT) | 297 | ||
| The portal color sort test (PCST) | 297 | ||
| Smartphone/tablet applications for color vision screening | 297 | ||
| Which test to use in a clinical setting? | 297 | ||
| New developments in color vision research | 298 | ||
| Gene therapy for color vision defects | 298 | ||
| Adaptive optics (AO) retinal imaging system | 298 | ||
| Rod and cone interactions in color vision | 298 | ||
| Acknowledgments | 298.e1 | ||
| References | 298 | ||
| 11 Visual Acuity and Contrast Sensitivity | 300 | ||
| Visual acuity tests | 300 | ||
| Introduction | 300 | ||
| Chart design | 300 | ||
| Optotypes | 300 | ||
| Chart layout | 300 | ||
| Testing procedure | 300 | ||
| Acuity test distance | 300 | ||
| Luminance and contrast | 301 | ||
| Test administration | 301 | ||
| Scoring | 302 | ||
| Near and reading acuity tests | 302 | ||
| Specifying letter size | 302 | ||
| Words versus continuous text | 302 | ||
| Electronic acuity tests | 302 | ||
| Contrast sensitivity tests | 303 | ||
| Introduction | 303 | ||
| Utility of contrast sensitivity tests | 303 | ||
| Methods | 303 | ||
| Common contrast sensitivity tests | 303 | ||
| Gratings versus optotypes | 303 | ||
| Test design and procedure | 305 | ||
| Interpretation of clinical versus statistical significance: an example from the literature | 305 | ||
| References | 305 | ||
| 12 Visual Fields in Retinal Disease | 307 | ||
| Principles of perimetry | 307 | ||
| The island of Traquair | 307 | ||
| Methods of visual field testing | 307 | ||
| Qualitative techniques | 307 | ||
| Quantitative techniques | 309 | ||
| Other methodologies of visual field testing in retinal disease | 311 | ||
| Reliability and reproducibility of visual field tests | 311 | ||
| Perimetry in specific retinal diseases | 312 | ||
| Retinal dystrophies | 312 | ||
| Diabetic retinopathy | 313 | ||
| Other vascular diseases and nondiabetic macular edema | 316 | ||
| Age-related macular degeneration and other maculopathies | 317 | ||
| Macular holes and epiretinal membrane | 318 | ||
| Toxic retinopathies | 320 | ||
| Infectious and inflammatory retinopathies | 320 | ||
| Retinal detachment | 323 | ||
| Tumors | 323 | ||
| Future of perimetry in retinal disease | 325 | ||
| Layer-by-layer perimetry | 325 | ||
| Color perimetry | 325 | ||
| High-resolution OCT and adaptive optics with microperimetry | 325 | ||
| Conclusions | 325 | ||
| References | 325 | ||
| 2 Basic Science and Translation to Therapy | 329 | ||
| Section 1: Anatomy and Physiology | 330 | ||
| 13 The Development of the Retina | 330 | ||
| Embryology of the eye | 330 | ||
| The eye field | 331 | ||
| Patterning the retinal, RPE, and anterior domains of the optic cup | 333 | ||
| Histogenesis of the retinal cell types | 333 | ||
| Inner retinal development | 335 | ||
| Photoreceptor development | 338 | ||
| Ganglion cell death | 339 | ||
| Retinal maturation | 339 | ||
| Conclusion | 340 | ||
| References | 340 | ||
| 14 Structure and Function of Rod and Cone Photoreceptors | 342 | ||
| Introduction | 342 | ||
| Photoreceptor fundamentals | 342 | ||
| Photoreceptor outer-segment structure | 343 | ||
| Proteins that stabilize the structure of outer-segment discs | 345 | ||
| Disc morphogenesis | 346 | ||
| Outer-segment plasma membrane | 346 | ||
| Outer-segment lipids | 346 | ||
| Phototransduction | 347 | ||
| Signal activation and amplification | 347 | ||
| Signal deactivation | 347 | ||
| Quenching R*: phosphorylation and arrestin binding | 347 | ||
| Deactivating PDE: control of transducin’s GTPase activity | 349 | ||
| Resynthesis of cGMP: Ca2+ dependence of guanylyl cyclase | 349 | ||
| Light adaptation | 349 | ||
| The role of Ca2+ feedback | 349 | ||
| Adaptation mediated by Ca2+ feedback to retinal guanylyl cyclase | 349 | ||
| Recoverin and control of rhodopsin kinase | 350 | ||
| Feedback regulation of the cGMP-gated channel | 350 | ||
| Other (Ca2+-independent) adaptation mechanisms: protein translocation | 350 | ||
| Differences between rod and cone phototransduction | 351 | ||
| Inner segment and connecting cilia | 351 | ||
| Inner segment | 351 | ||
| Targeting of phototransduction proteins from the inner segment to the outer segment | 352 | ||
| The connecting cilium | 352 | ||
| Nucleus | 353 | ||
| Photoreceptor synaptic terminal | 353 | ||
| Photoreceptor dysfunction and disease | 354 | ||
| Rhodopsin mutations | 354 | ||
| Constitutive phototransduction and retinal disease | 354 | ||
| Transducin defects and retinal disease | 355 | ||
| cGMP and photoreceptor cell physiology | 355 | ||
| References | 356 | ||
| 15 Function and Anatomy of the Mammalian Retina | 360 | ||
| Visual illusions and multiple channels | 360 | ||
| The retina is a piece of brain | 361 | ||
| Neuronal communication: chemical and electrical | 361 | ||
| The retina is a layered structure | 361 | ||
| Gross retinal morphology | 362 | ||
| The fovea | 362 | ||
| The blind spot and how to find it | 363 | ||
| Painting the retina – techniques to label and visualize retinal neurons | 363 | ||
| Six major neuronal cell classes | 364 | ||
| Classification of retinal cells | 364 | ||
| Photoreceptors | 365 | ||
| Cones | 365 | ||
| Rods | 366 | ||
| Cone pedicles and rod spherules | 366 | ||
| Photoreceptor coupling | 366 | ||
| Photoreceptors release glutamate in the dark | 368 | ||
| Second-order neurons: horizontal and bipolar cells | 369 | ||
| Horizontal cell function | 371 | ||
| Bipolar cell function | 372 | ||
| OFF cone bipolar cells | 373 | ||
| ON cone bipolar cells | 374 | ||
| Midget bipolar cells | 374 | ||
| Blue cone bipolar cells | 376 | ||
| Rod bipolar cells | 376 | ||
| Multiple rod pathways | 377 | ||
| Secondary and tertiary rod pathways | 380 | ||
| Amacrine cells | 380 | ||
| AII amacrine cells | 381 | ||
| S1 and S2 amacrine cells | 382 | ||
| Dopaminergic amacrine cells | 384 | ||
| Starburst amacrine cells | 385 | ||
| Ganglion cells | 386 | ||
| Does each ganglion cell type represent a visual channel? | 388 | ||
| A ganglion cell for the control of pupil diameter and circadian rhythm | 391 | ||
| Color vision and ganglion cells | 391 | ||
| Gene therapy to cure color blindness | 392 | ||
| New tools to identify ganglion cell types | 392 | ||
| Clinical relevance of functional anatomy | 393 | ||
| Conclusions | 394 | ||
| Acknowledgments | 395.e1 | ||
| References | 395 | ||
| 16 Cell Biology of the Retinal Pigment Epithelium | 401 | ||
| Embryology | 401 | ||
| Anatomy and histology | 401 | ||
| Heterogeneity and polarity of the RPE | 401 | ||
| Cellular junctions | 403 | ||
| Cytoskeleton | 405 | ||
| Role of RPE in Bruch’s membrane synthesis and remodeling | 405 | ||
| Cell culture models of RPE | 406 | ||
| specialized functions of the RPE | 406 | ||
| Absorption of light | 406 | ||
| Phagocytosis of rod outer segments | 406 | ||
| Role in visual cycle | 407 | ||
| Protection from oxidative stress | 408 | ||
| Role in maintaining avascular outer retina | 408 | ||
| Immune privilege | 409 | ||
| Transport of nutrients, ions, and water | 409 | ||
| Secretion of cytokines and growth factors | 409 | ||
| Acknowledgments | 410.e1 | ||
| References | 410 | ||
| 17 Cell Biology of the Müller Cell | 415 | ||
| Introduction | 415 | ||
| Morphology of müller cells | 415 | ||
| Müller cells constitute the cores of functional retinal columns | 417 | ||
| Light guidance | 417 | ||
| Recycling of cone photopigments | 418 | ||
| Regulation of the synaptic activity by neurotransmitter uptake | 418 | ||
| Malfunction of glial glutamate uptake contributes to glutamate toxicity | 418 | ||
| Production of neurotransmitter precursors | 421 | ||
| Trophic support of photoreceptors and neurons | 422 | ||
| Antioxidative support of photoreceptors and neurons | 422 | ||
| Removal of carbon dioxide | 422 | ||
| Regulation of the extracellular pH | 422 | ||
| Spatial potassium buffering | 422 | ||
| Water clearance | 425 | ||
| Contribution to edema development and resolution | 425 | ||
| Regulation of the blood–retinal barrier | 427 | ||
| Mediation of neurovascular coupling | 427 | ||
| Regulation of the extracellular space volume | 427 | ||
| Responses to mechanical stress | 427 | ||
| Regulation of neuronal activity by release of gliotransmitters | 428 | ||
| Ionotropic receptors of Müller cells | 429 | ||
| Müller cell gliosis | 429 | ||
| Müller stem cells | 430 | ||
| References | 430 | ||
| 18 Retinal and Choroidal Vasculature: Retinal Oxygenation | 433 | ||
| Introduction | 433 | ||
| Comparison of retinal and choroidal vasculatures | 433 | ||
| History of retinal ischemia | 433 | ||
| Normoxia | 434 | ||
| Hyperoxia | 434 | ||
| Hypoxia | 434 | ||
| Hypoxia-inducible factor | 435 | ||
| HIF deficiency and its resultant pathology | 436 | ||
| HIF-activated genes relevant to physiological and pathological ocular angiogenesis | 437 | ||
| VEGF in health and in ocular disease | 437 | ||
| Bone marrow-derived progenitor cells (BMPC) and vascular repair | 439 | ||
| Disease-associated BMPC dysfunction | 439 | ||
| Key factors that modulate VEGF function in the retina | 439 | ||
| Adult retinal hypoxia and etiology | 440 | ||
| Diabetic retinopathy | 440 | ||
| Retinal vein occlusion (RVO) | 440 | ||
| Sickle-cell disease (SCD) | 441 | ||
| Ocular ischemic syndrome (OIS) | 441 | ||
| Retinal detachment | 442 | ||
| Consequences of retinal ischemia | 442 | ||
| Vascular permeability | 442 | ||
| Adult choroidal ischemia | 443 | ||
| Conclusions | 443 | ||
| References | 443 | ||
| 19 Mechanisms of Normal Retinal Adhesion | 447 | ||
| Models for measuring retinal adhesion | 447 | ||
| In vitro methods | 447 | ||
| In vivo methods | 447 | ||
| Adhesive force and environmental factors | 447 | ||
| Magnitude of adhesive force | 447 | ||
| Sensitivity to temperature and ionic environment | 447 | ||
| Mechanical forces outside the subretinal space | 448 | ||
| Fluid pressure: hydrostatic and osmotic | 448 | ||
| Vitreous support and other physical aspects of adhesion | 450 | ||
| Mechanical forces inside the subretinal space | 451 | ||
| Mechanical interdigitation | 451 | ||
| Interphotoreceptor matrix properties | 451 | ||
| Subcellular components and mobility | 452 | ||
| Metabolic factors | 454 | ||
| Critical dependence on oxygen | 454 | ||
| Metabolic inhibitors and other agents | 455 | ||
| Relationship of adhesion to subretinal fluid transport and subretinal protein | 456 | ||
| Pharmacologic modification of adhesion | 456 | ||
| Mannitol | 456 | ||
| Acetazolamide | 457 | ||
| Cold temperature and ouabain | 458 | ||
| Ionic changes | 458 | ||
| Implications for vitreoretinal surgery | 458 | ||
| Recovery after rhegmatogenous retinal detachment | 458 | ||
| Recovery of adhesiveness without retinopexy | 458 | ||
| Effects of retinopexy | 458 | ||
| Effects of vitreous in the subretinal space | 459 | ||
| Pathophysiology of serous detachment | 459 | ||
| Conclusions and general implications | 461 | ||
| References | 462 | ||
| 20 Structure, Function, and Pathology of Bruch’s Membrane | 465 | ||
| Introduction, history, embryology | 465 | ||
| Early history | 465 | ||
| Development of Bruch’s membrane | 465 | ||
| Structure of Bruch’s membrane in the young adult eye | 465 | ||
| RPE basal lamina (RPE-BL) | 465 | ||
| Inner collagenous layer (ICL) | 465 | ||
| Elastic layer (EL) | 465 | ||
| Outer collagenous layer | 467 | ||
| Choriocapillaris basal lamina (ChC-BL) | 467 | ||
| Bruch’s membrane in an aged eye | 467 | ||
| Lipid accumulation: Bruch’s membrane lipoproteins | 467 | ||
| Other aging changes | 469 | ||
| Function of Bruch’s membrane | 469 | ||
| Structural role of Bruch’s membrane | 470 | ||
| Transport role of Bruch’s membrane | 470 | ||
| Hydraulic conductivity of Bruch’s membrane | 470 | ||
| Age-related changes in hydraulic conductivity and disease | 471 | ||
| Permeability of Bruch’s membrane to solute transport | 473 | ||
| Summary and implications | 473 | ||
| Pathology of Bruch’s membrane | 474 | ||
| AMD lesions | 474 | ||
| Drusen | 474 | ||
| Basal linear deposit | 475 | ||
| Basal laminar deposit | 475 | ||
| Subretinal drusenoid debris | 476 | ||
| Summary | 476 | ||
| Response-to-retention hypothesis of AMD | 476 | ||
| Neovascular AMD | 477 | ||
| Angioid streaks (ABCC6, MTP genes) | 477 | ||
| Thick basal laminar deposits (TIMP-3, CTRP5, EFEMP1 genes) | 477 | ||
| Conclusion | 477 | ||
| References | 478 | ||
| 21 Vitreous and Vitreoretinal Interface | 482 | ||
| Biochemistry of vitreous | 482 | ||
| Collagen | 482 | ||
| Hyaluronan | 482 | ||
| Chondroitin sulfate | 482 | ||
| Noncollagenous structural proteins | 483 | ||
| Fibrillins | 483 | ||
| Opticin | 483 | ||
| Anatomy and histology | 483 | ||
| Vitreous body | 483 | ||
| Vitreoretinal interface | 486 | ||
| Posterior vitreous cortex | 486 | ||
| Hyalocytes | 486 | ||
| Internal limiting lamina (ILL) of the retina | 488 | ||
| Retinal sheen dystrophy | 488 | ||
| Degenerative remodeling | 488 | ||
| Vitreoretinal interface | 490 | ||
| Topographic variations | 490 | ||
| Strength of vitreoretinal adhesion | 490 | ||
| Peripheral fundus and vitreous base | 490 | ||
| Interface along major retinal vessels | 490 | ||
| Vitreomacular interface | 490 | ||
| Vitreopapillary interface | 490 | ||
| Physiology | 495 | ||
| Biochemical | 495 | ||
| Biophysical | 495 | ||
| Age-Related Vitreous Degeneration | 496 | ||
| Liquefaction (synchysis) | 496 | ||
| Pathogenesis of vitreous liquefaction | 496 | ||
| Aging changes and vitreous biochemistry | 497 | ||
| Structural changes | 497 | ||
| Vitreous body | 497 | ||
| Aging changes at the vitreoretinal interface | 497 | ||
| Posterior vitreous detachment | 497 | ||
| Epidemiology | 498 | ||
| Symptomatic PVD | 498 | ||
| Anomalous PVD (APVD) | 498 | ||
| Vitreous effects of APVD | 498 | ||
| Peripheral retinal effects of APVD | 500 | ||
| Retinal breaks | 500 | ||
| Other sequelae | 500 | ||
| Macular effects of APVD | 500 | ||
| Vitreomacular traction | 500 | ||
| Exudative age-related macular degeneration | 500 | ||
| Cystoid macular edema | 500 | ||
| Macular cysts | 500 | ||
| Macular holes | 504 | ||
| Histopathology and pseudo-operculum | 504 | ||
| Pathogenesis | 504 | ||
| Optic disc effects | 505 | ||
| Vitreoretinal Changes after Lens Extraction | 505 | ||
| Structural | 505 | ||
| Biochemical | 506 | ||
| PVD | 506 | ||
| Inflammatory | 506 | ||
| Vitreoretinal changes after trauma | 506 | ||
| Blunt trauma | 506 | ||
| Shaken-baby syndrome | 506 | ||
| Posterior penetrating and perforating trauma | 507 | ||
| Periretinal proliferation | 507 | ||
| Premacular membranes | 507 | ||
| Retroretinal membranes | 507 | ||
| Complex membranes | 507 | ||
| References | 512 | ||
| Section 2: Basic Mechanisms of Injury in the Retina | 517 | ||
| 22 Mechanisms of Oxidative Stress in Retinal Injury | 517 | ||
| Overview of oxidative stress in the retina | 517 | ||
| Retinal diseases related to oxidative stress | 517 | ||
| Age-related macular degeneration | 517 | ||
| Diabetic retinopathy | 518 | ||
| Inherited retinal degenerations | 519 | ||
| Oxidative injury to the retina | 520 | ||
| Retinal pigment epithelium | 520 | ||
| Retinal vasculature | 520 | ||
| Photoreceptors | 521 | ||
| Mitochondria | 521 | ||
| Oxidative stress and inflammation | 522 | ||
| Retinal therapies targeting oxidative stress | 523 | ||
| Supplemental antioxidants | 523 | ||
| Dietary antioxidants | 524 | ||
| Anti-advanced glycation end-product treatment | 525 | ||
| Genetic modification | 525 | ||
| Conclusions | 525 | ||
| References | 525 | ||
| 23 Mechanisms of ER Stress in Retinal Disease | 529 | ||
| The endoplasmic reticulum | 529 | ||
| ER stress and UPR signaling | 529 | ||
| Binding protein/glucose-regulated protein 78 | 529 | ||
| PERK | 530 | ||
| IRE1 | 530 | ||
| ATF6 | 531 | ||
| ER-associated degradation | 531 | ||
| Apoptosis-inducing pathways | 532 | ||
| Retinal diseases associated with ER stress | 532 | ||
| Retinitis pigmentosa and other photoreceptor dystrophies | 532 | ||
| Rhodopsin mutations | 532 | ||
| cGMP-PDE mutations | 533 | ||
| Carbonic anhydrase mutations | 533 | ||
| LRAT mutations | 533 | ||
| Diabetic retinopathy | 533 | ||
| Macular degeneration | 534 | ||
| Early-onset macular dystrophies | 534 | ||
| Age-related macular degeneration | 534 | ||
| References | 535 | ||
| 24 Cell Death, Apoptosis, and Autophagy in Retinal Injury | 537 | ||
| Introduction | 537 | ||
| Modes of cell death | 537 | ||
| Apoptosis | 537 | ||
| Necrosis | 537 | ||
| Other | 537 | ||
| Cross-talk between cell death pathways | 538 | ||
| Is cell death a bad thing? | 538 | ||
| Autophagy and cell maintenance | 538 | ||
| AGE-related retinal cell loss | 541 | ||
| Retinal damage: death and repair | 542 | ||
| Introduction | 542 | ||
| Glaucoma and ganglion cell loss | 542 | ||
| Diabetic retinopathy | 542 | ||
| Macular degeneration | 543 | ||
| Retinal detachment | 544 | ||
| Retinal dystrophies | 544 | ||
| Light damage | 545 | ||
| Therapeutic options | 545 | ||
| Neuroprotection | 545 | ||
| Modulating autophagy | 547 | ||
| Cellular replacement | 547 | ||
| Conclusions | 548 | ||
| References | 548 | ||
| 25 Inflammatory Response and Mediators in Retinal Injury | 553 | ||
| Introduction | 553 | ||
| Retinal injury | 553 | ||
| Ischemia–hypoxia | 553 | ||
| Oxidative stress | 554 | ||
| Oxidative stress and inflammation in AMD | 555 | ||
| Microglia and AMD | 556 | ||
| Other inflammatory-related molecules and pathways | 556 | ||
| Genes and inflammation in AMD | 557 | ||
| Oxidative stress and inflammation in retinopathy of prematurity | 557 | ||
| Anti-inflammatory and antioxidant therapies in AMD and ROP | 557 | ||
| Trauma | 557 | ||
| Conclusion | 559 | ||
| References | 559 | ||
| 26 Basic Mechanisms of Pathological Retinal and Choroidal Angiogenesis | 562 | ||
| Introduction | 562 | ||
| Pathogenesis | 562 | ||
| CNV associated with AMD (Fig. 26.3) | 564 | ||
| Aging and senescence of the RPE | 564 | ||
| Drusen, basal laminar/linear deposit formation | 564 | ||
| Enzymatic and mechanical disruption of Bruch’s membrane | 564 | ||
| Complement, AMD, and CNV | 566 | ||
| Inflammation, bone marrow-derived cells, and CNV | 566 | ||
| CNV membrane formation | 566 | ||
| Cicatricial membrane formation | 566 | ||
| Neovascularization associated with diabetic retinopathy | 567 | ||
| Neovascularization associated with retinopathy of prematurity | 567 | ||
| Neovascularization in vascular occlusions | 568 | ||
| Neovascularization in uveitis | 568 | ||
| Genetic aspects of neovascularization | 568 | ||
| Age-related macular degeneration | 568 | ||
| Diabetic retinopathy | 568 | ||
| Section 1: Retinal Degenerations and Dystrophies | 761 | ||
| 40 Retinitis Pigmentosa and Allied Disorders | 761 | ||
| Early history | 761 | ||
| Typical retinitis pigmentosa | 761 | ||
| Clinical features | 761 | ||
| Nyctalopia | 761 | ||
| Visual field loss | 761 | ||
| Central vision loss | 763 | ||
| Color vision defects | 764 | ||
| Photopsia and other symptoms | 764 | ||
| Fundus appearance | 764 | ||
| Vitreous abnormalities | 769 | ||
| Anterior-segment abnormalities | 769 | ||
| Refractive status | 769 | ||
| Psychophysical findings | 769 | ||
| Perimetry | 769 | ||
| Dark adaptometry | 772 | ||
| Retinal densitometry (fundus reflectometry) | 774 | ||
| Electrophysiology | 775 | ||
| Imaging modalities in RP | 777 | ||
| Fundus photography/fluorescein angiography | 777 | ||
| Autofluorescence | 778 | ||
| Optical coherence tomography | 780 | ||
| Adaptive optics scanning laser ophthalmoscopy | 780 | ||
| Classification | 780 | ||
| Subdivision by inheritance type | 780 | ||
| Subdivision by age of onset | 780 | ||
| Subdivision by molecular defect | 781 | ||
| Subdivision by distribution of retinal involvement or fundus appearance | 782 | ||
| Sector and sectoral retinitis pigmentosa | 783 | ||
| Pericentral retinitis pigmentosa | 784 | ||
| Unilateral or extremely asymmetrical retinitis pigmentosa | 784 | ||
| Complicated retinitis pigmentosa | 787 | ||
| Systemic associations | 787 | ||
| Usher syndrome | 787 | ||
| Differential diagnosis – phenocopies of retinitis pigmentosa | 789 | ||
| Cone–rod and cone dystrophy | 789 | ||
| Leber congenital amaurosis/severe early childhood onset retinal dystrophy (SECORD) | 794 | ||
| Bardet–Biedl syndrome | 795 | ||
| Refsum syndromes | 796 | ||
| Infantile Refsum disease | 796 | ||
| Adult-onset Refsum disease | 796 | ||
| Neuronal ceroid lipofuscinosis (Batten’s disease) | 796 | ||
| Differential diagnosis: pseudoretinitis pigmentosa | 798 | ||
| Retinal inflammatory diseases | 798 | ||
| Rubella retinopathy | 798 | ||
| Syphilis | 798 | ||
| Infectious retinitis | 798 | ||
| Autoimmune paraneoplastic retinopathy | 799 | ||
| Drug toxicity (see Chapter 89, Drug toxicity) | 799 | ||
| Thioridazine | 799 | ||
| Chlorpromazine | 799 | ||
| Chloroquine | 799 | ||
| Hydroxychloroquine | 799 | ||
| Quinine | 799 | ||
| Pigmented paravenous retinochoroidal atrophy | 799 | ||
| Traumatic retinopathy | 801 | ||
| Diffuse unilateral subacute neuroretinitis | 801 | ||
| Grouped pigmentation of the retina | 801 | ||
| Basic science | 801 | ||
| Molecular biology | 801 | ||
| Molecular genetics | 801 | ||
| Autosomal dominant RP genes | 801 | ||
| Autosomal recessive RP genes | 804 | ||
| X-linked RP genes | 806 | ||
| Digenic inheritance and RP | 807 | ||
| Usher syndrome molecular genetics | 808 | ||
| Protein chemistry | 808 | ||
| Abnormal pre-mRNA splicing | 808 | ||
| RPGR interactome | 809 | ||
| Ush interactome | 809 | ||
| Bardet–Biedl syndrome and the “BBSome” | 810 | ||
| Abnormal intracellular trafficking | 810 | ||
| Cell death pathways | 810 | ||
| Cell and tissue biology | 810 | ||
| Histopathology | 810 | ||
| Photoreceptor abnormalities | 810 | ||
| Outer retinal disease | 811 | ||
| Inner retinal pathology | 811 | ||
| Cellular remodeling and vascular changes | 812 | ||
| Genetic consultation | 813 | ||
| Counseling family groups | 813 | ||
| Support services | 814 | ||
| Treatment | 815 | ||
| Cataract extraction | 815 | ||
| Macular edema | 816 | ||
| Vitamin A supplements | 817 | ||
| Docosahexaenoic acid supplements | 817 | ||
| Lutein supplements | 817 | ||
| Clinical trial of DHA supplementation | 818 | ||
| Clinical trials of lutein supplementation | 818 | ||
| Purported “cures” for RP | 818 | ||
| Future management | 819 | ||
| Gene therapy | 819 | ||
| Cell therapy (see Chapter 35, Stem cells and cellular therapy, and Chapter 125, Transplantation frontiers) | 820 | ||
| Apoptosis/neuroprotection | 821 | ||
| Electronic prosthesis (artificial retina) | 821 | ||
| Acknowledgment | 822.e1 | ||
| References | 822 | ||
| 41 Hereditary Vitreoretinal Degenerations | 836 | ||
| Snowflake vitreoretinal degeneration | 836 | ||
| General features | 836 | ||
| Clinical findings | 836 | ||
| Ocular features | 836 | ||
| Molecular genetics of SVD | 839 | ||
| Visual psychophysics | 839 | ||
| Electrophysiology | 839 | ||
| Differential diagnosis | 839 | ||
| Stickler syndrome type I | 839 | ||
| Stickler syndrome type II | 839 | ||
| Marshall syndrome | 840 | ||
| Wagner syndrome | 840 | ||
| Goldmann–Favre vitreotapetoretinal degeneration | 840 | ||
| Management | 840 | ||
| The chromosome 5Q retinopathies: wagner syndrome, jansen syndrome, erosive vitreoretinopathy, and related conditions | 840 | ||
| General features | 840 | ||
| Clinical findings | 840 | ||
| Ocular features | 840 | ||
| Visual psychophysics | 841 | ||
| Electrophysiology | 841 | ||
| Differential diagnosis | 841 | ||
| Autosomal dominant vitreoretinopathies | 841 | ||
| Snowflake vitreoretinal degeneration | 841 | ||
| Stickler syndrome | 841 | ||
| Autosomal dominant vitreoretinochoroidopathy | 842 | ||
| Autosomal recessive vitreoretinopathies | 842 | ||
| Goldmann–Favre syndrome (GFS) and enhanced S-cone syndrome | 842 | ||
| Knobloch syndrome | 842 | ||
| Management | 842 | ||
| Genetic counseling | 842 | ||
| Treatments | 842 | ||
| Chondrodysplasias associated with vitreoretinal degeneration: the stickler syndromes, marshall syndrome, kniest dysplasia, knobloch syndrome, and weissenbacher–zweymuller syndrome | 842 | ||
| General features | 842 | ||
| Clinical findings | 843 | ||
| Extraocular features | 843 | ||
| Ocular features | 843 | ||
| Differential diagnosis | 844 | ||
| Marfan syndrome | 844 | ||
| Wagner syndrome | 844 | ||
| Erosive vitroeretinopathy | 844 | ||
| Management | 844 | ||
| X-linked retinoschisis | 844 | ||
| General features | 844 | ||
| Clinical findings | 844 | ||
| Ocular features | 844 | ||
| Visual psychophysics | 845 | ||
| Optical coherence tomography | 845 | ||
| Electrophysiology | 845 | ||
| Differential diagnosis | 846 | ||
| Management | 846 | ||
| Genetic counseling | 846 | ||
| Treatment | 846 | ||
| Pharmacological treatment | 846 | ||
| Laser | 846 | ||
| Surgery | 847 | ||
| Gene therapy | 847 | ||
| Retina and/or progenitor cell transplantation | 847 | ||
| Retinal nuclear receptor (NR2E3)-related diseases: enhanced S-cone syndrome and goldmann–favre vitreotapetoretinal degeneration | 847 | ||
| General features | 847 | ||
| Clinical findings | 847 | ||
| Ocular features | 847 | ||
| Visual psychophysics | 848 | ||
| Electrophysiology | 848 | ||
| Differential diagnosis | 848 | ||
| X-linked retinoschisis | 848 | ||
| Cystoid macular edema | 848 | ||
| Management | 848 | ||
| Other vitreoretinal degenerations and vitreoretinopathies | 848 | ||
| Autosomal dominant vitreoretinochoroidopathy | 848 | ||
| Autosomal recessive inherited vitreoretinal dystrophy | 848 | ||
| Hereditary neovascular vitreoretinopathies | 848 | ||
| Autosomal dominant neovascular inflammatory vitreoretinopathy | 848 | ||
| Dominantly inherited peripheral retinal neovascularization | 849 | ||
| References | 849 | ||
| 42 Macular Dystrophies | 852 | ||
| Introduction | 852 | ||
| The initial approach to a patient with macular dystrophy | 852 | ||
| Best macular dystrophy | 855 | ||
| Clinical features of BMD | 855 | ||
| Visual function | 856 | ||
| Refractive error | 856 | ||
| Optical coherence tomography (OCT) | 859 | ||
| Fluorescein angiography and autofluorescence | 859 | ||
| Electrophysiology | 861 | ||
| Genetics | 862 | ||
| Pathophysiology and histopathology | 862 | ||
| Additional phenotypes associated with mutations in BEST1 | 863 | ||
| Autosomal dominant vitreoretinochoroidopathy (ADVIRC) | 863 | ||
| Autosomal recessive bestrophinopathy (ARB) | 863 | ||
| Treatment | 864 | ||
| Stargardt disease | 864 | ||
| Clinical features of Stargardt disease | 864 | ||
| Visual function | 868 | ||
| Fluorescein angiography and autofluorescence | 868 | ||
| Optical coherence tomography | 869 | ||
| Electrophysiology | 871 | ||
| Genetics | 874 | ||
| Pathophysiology and histopathology | 874 | ||
| Treatment | 874 | ||
| Stargardt-like dominant macular dystrophy (SLDMD) | 874 | ||
| Pathophysiology | 875 | ||
| Pattern dystrophy | 875 | ||
| Clinical features and history of specific pattern dystrophies | 876 | ||
| Butterfly-shaped pigment dystrophy | 876 | ||
| Adult-onset foveomacular vitelliform pattern dystrophy | 877 | ||
| Sjögren reticular dystrophy of the RPE | 877 | ||
| Central areolar choroidal dystrophy (central areolar retinochoroidal dystrophy) | 878 | ||
| Electrophysiology | 879 | ||
| Pathophysiology | 879 | ||
| Treatment | 880 | ||
| Sorsby fundus dystrophy | 880 | ||
| Clinical features of SFD | 880 | ||
| Genetics | 881 | ||
| Pathophysiology | 881 | ||
| Treatment | 881 | ||
| Autosomal dominant radial drusen (doyne honeycomb retinal dystrophy, malattia leventinese) | 881 | ||
| Clinical features of ADRD | 881 | ||
| Visual function and electrophysiology | 882 | ||
| Imaging | 882 | ||
| Pathophysiology and histopathology | 882 | ||
| Treatment | 883 | ||
| North carolina macular dystrophy | 883 | ||
| Spotted cystic dystrophy | 883 | ||
| Dominant cystoid macular dystrophy | 885 | ||
| Fenestrated sheen macular dystrophy (FSMD) | 885 | ||
| Glomerulonephritis type II and drusen | 885 | ||
| Acknowledgments | 886.e1 | ||
| References | 886 | ||
| 43 Hereditary Choroidal Diseases | 891 | ||
| Introduction | 891 | ||
| Choroidal atrophy phenotypes | 891 | ||
| Central areolar choroidal dystrophy | 891 | ||
| Peripapillary choroidal dystrophy | 892 | ||
| Diffuse choroidal dystrophy | 892 | ||
| Gyrate atrophy of the choroid and retina | 892 | ||
| Choroideremia | 893 | ||
| Clinical phenotypes resembling hereditary choroidal diseases | 895 | ||
| X-linked retinitis pigmentosa (XLRP) | 895 | ||
| Kearns–Sayre syndrome (KSS) | 895 | ||
| Bietti’s crystalline dystrophy | 896 | ||
| Thioridazine (Mellaril) retinal toxicity | 896 | ||
| Stargardt disease | 896 | ||
| Pattern macular dystrophy | 897 | ||
| Conclusion | 897 | ||
| References | 897 | ||
| 44 Abnormalities of Cone and Rod Function | 899 | ||
| Disorders of the cone system | 899 | ||
| Achromatopsia | 899 | ||
| Diagnosis | 899 | ||
| Molecular basis of achromatopsia | 901.e1 | ||
| Section 2: Retinal Vascular Disease | 907 | ||
| 45 The Epidemiology of Diabetic Retinopathy | 907 | ||
| Introduction | 907 | ||
| Prevalence of diabetic retinopathy | 907 | ||
| Incidence and progression of diabetic retinopathy and incidence of clinically significant macular edema | 909 | ||
| The relationship of race/ethnicity to diabetic retinopathy | 910 | ||
| Genetic factors | 911 | ||
| Sex | 911 | ||
| Age and puberty | 911 | ||
| Duration of diabetes | 911 | ||
| Age at diagnosis | 912 | ||
| Glycemia | 912 | ||
| C-peptide status | 915 | ||
| Exogenous insulin | 915 | ||
| Blood pressure | 916 | ||
| Proteinuria and diabetic nephropathy | 917 | ||
| Serum lipids and lipid lowering | 917 | ||
| Smoking | 917 | ||
| Alcohol | 918 | ||
| Body mass index (BMI) | 918 | ||
| Physical activity | 918 | ||
| Socioeconomic status | 918 | ||
| Hormone and reproductive exposures in women | 919 | ||
| Comorbidity and mortality | 919 | ||
| New medical interventions | 919 | ||
| Public health applications of epidemiological data | 919 | ||
| Conclusion | 921 | ||
| References | 921 | ||
| 46 Diabetic Retinopathy: Genetics and Etiologic Mechanisms | 925 | ||
| Introduction | 925 | ||
| Anatomic lesions | 925 | ||
| Loss of pericytes | 925 | ||
| Capillary basement membrane thickening | 926 | ||
| Microaneurysms | 926 | ||
| Capillary acellularity | 926 | ||
| Breakdown of blood–retina barrier | 927 | ||
| Biochemical mechanisms in the pathogenesis of diabetic retinopathy | 927 | ||
| The aldose reductase theory | 927 | ||
| Advanced glycation endproduct (AGE) theory | 928 | ||
| Reactive oxygen intermediates (ROI) theory | 928 | ||
| Protein kinase C (PKC) theory | 928 | ||
| Insulin receptors and glucose transporters | 929 | ||
| Genetic factors in the pathogenesis of diabetic retinopathy | 930 | ||
| Other ocular factors | 931 | ||
| Retinopathy in different forms of diabetes | 932 | ||
| Animal models in the study of diabetic retinopathy | 932 | ||
| Cell culture studies | 934 | ||
| Conclusion | 935 | ||
| Acknowledgments | 935.e1 | ||
| References | 935 | ||
| 47 Nonproliferative Diabetic Retinopathy and Diabetic Macular Edema | 940 | ||
| Natural course of nonproliferative diabetic retinopathy | 940 | ||
| Diabetes mellitus without retinopathy | 940 | ||
| Microaneurysms | 940 | ||
| Retinal vascular hyperpermeability | 940 | ||
| Diabetic macular edema | 941 | ||
| Capillary closure, microvascular remodeling, and retinal ischemia | 942 | ||
| Alterations of the vitreous gel and vitreoretinal interface | 942 | ||
| Clinical evaluation of nonproliferative diabetic retinopathy | 942 | ||
| Duration of diabetes mellitus | 942 | ||
| Hyperglycemia | 942 | ||
| Hypertension | 943 | ||
| Dyslipidemia | 944 | ||
| Other extraocular factors | 944 | ||
| Ophthalmic evaluation | 944 | ||
| Ancillary ocular imaging | 944 | ||
| Fundus photography | 944 | ||
| Fluorescein angiography | 945 | ||
| Optical coherence tomography | 945 | ||
| Funduscopic lesions of nonproliferative diabetic retinopathy | 947 | ||
| Classification of diabetic retinopathy | 948 | ||
| Clinical evaluation of diabetic macular edema | 949 | ||
| Distribution of retinal thickening and hard exudates | 949 | ||
| Magnitude of retinal thickening | 950 | ||
| Retinal microvascular alterations and vascular hyperpermeability | 950 | ||
| Traction by vitreous gel and epiretinal proliferation | 951 | ||
| Alterations in the retinal pigment epithelium | 953 | ||
| Subretinal fibrosis | 954 | ||
| Visual acuity and its correlation to retinal thickening and fluorescein leakage | 954 | ||
| Diurnal variation of DME | 955 | ||
| Management of nonproliferative diabetic retinopathy and diabetic macular edema | 955 | ||
| Modification of systemic risk factors | 955 | ||
| Retinopathy screening and surveillance | 955 | ||
| Ocular treatment for diabetic macular edema | 956 | ||
| Focal/grid laser photocoagulation | 957 | ||
| Pharmacotherapy with vascular endothelial growth factor (VEGF) antagonists | 958 | ||
| Pharmacotherapy with corticosteroids | 959 | ||
| Vitrectomy | 962 | ||
| Ocular treatment for nonproliferative diabetic retinopathy | 962 | ||
| Other systemic treatment for nonproliferative diabetic retinopathy | 963 | ||
| Conclusion | 964 | ||
| References | 964 | ||
| 48 Proliferative Diabetic Retinopathy | 969 | ||
| Pathogenesis of proliferative diabetic retinopathy | 969 | ||
| Origin and early recognition of preretinal new vessels | 970 | ||
| Natural course of proliferative diabetic retinopathy | 972 | ||
| Development and proliferation of new vessels | 972 | ||
| Contraction of the vitreous and fibrovascular proliferation | 974 | ||
| Retinal distortion and tractional detachment | 977 | ||
| Involutional or “Quiescent” Proliferative Diabetic Retinopathy | 978 | ||
| Relationship of proliferative diabetic retinopathy to type and duration of diabetes | 978 | ||
| Proliferative diabetic retinopathy and blood glucose control | 980 | ||
| Early worsening of retinopathy with improved glycemic control | 980 | ||
| Absence of proliferative diabetic retinopathy in individuals with diabetes of extreme duration | 981 | ||
| Systemic medications and proliferative diabetic retinopathy | 981 | ||
| Other risk factors for proliferative diabetic retinopathy | 981 | ||
| Management of proliferative diabetic retinopathy | 982 | ||
| Pituitary ablation | 982 | ||
| Early laser trials | 982 | ||
| Panretinal photocoagulation | 983 | ||
| Early treatment diabetic retinopathy study and the timing of treatment | 987 | ||
| Scatter photocoagulation and macular edema | 989 | ||
| Panretinal photocoagulation and advanced proliferative diabetic retinopathy | 990 | ||
| Current techniques of panretinal photocoagulation | 991 | ||
| Direct (local) treatment of NVE | 991 | ||
| Distribution and strength of panretinal photocoagulation | 991 | ||
| Pattern scanning laser delivery systems | 993 | ||
| Number of episodes used for scatter treatment | 993 | ||
| Wavelength | 993 | ||
| Regression of new vessels after initial photocoagulation and indications for retreatment | 994 | ||
| Complications of prp | 995 | ||
| Antiangiogenic therapies for proliferative diabetic retinopathy | 996 | ||
| Pharmaceutical vitreolysis for treatment of pdr | 996 | ||
| Indications for vitrectomy | 997 | ||
| Conclusion | 997 | ||
| References | 997 | ||
| 49 Hypertension | 1001 | ||
| Introduction | 1001 | ||
| Hypertensive retinopathy | 1001 | ||
| Definition and classification | 1001 | ||
| Epidemiology | 1002 | ||
| Relationship with stroke | 1003 | ||
| Relationship with coronary heart disease | 1003 | ||
| Relationship with other end-organ damage of hypertension | 1003 | ||
| Hypertensive choroidopathy | 1003 | ||
| Hypertensive optic neuropathy | 1004 | ||
| Future directions | 1004 | ||
| Conclusion | 1004 | ||
| References | 1004 | ||
| 50 Telescreening for Diabetic Retinopathy | 1006 | ||
| Introduction | 1006 | ||
| Guidelines for telescreening program | 1006 | ||
| American Telemedicine Association telehealth practice recommendations for diabetic retinopathy | 1006 | ||
| Steps of telescreening | 1007 | ||
| Technical considerations | 1008 | ||
| Image acquisition | 1008 | ||
| Compression | 1008 | ||
| Data transfer, archiving, and retrieval | 1008 | ||
| Security and documentation | 1008 | ||
| Operational considerations | 1008 | ||
| Detection of diabetic retinopathy and macular edema | 1008 | ||
| Role of the reading center to grade retinal images | 1009 | ||
| Pathways of grading | 1009 | ||
| Pathway 1: Disease/no disease grading | 1009 | ||
| Pathway 2: Full disease grading | 1009 | ||
| Arbitration grade | 1009 | ||
| Reading personnel | 1009 | ||
| Handling of ungradable images | 1009 | ||
| Quality assurance | 1009 | ||
| Evaluating telescreening programs | 1010 | ||
| Efficacy | 1010 | ||
| Patient satisfaction | 1010 | ||
| Cost-effectiveness | 1010 | ||
| Advances in telescreening | 1010 | ||
| Automated retinal image analysis | 1010 | ||
| Conclusion | 1011 | ||
| References | 1011 | ||
| 51 Retinal Artery Obstructions | 1012 | ||
| Central retinal artery obstruction | 1012 | ||
| Epidemiology | 1012 | ||
| Clinical features | 1012 | ||
| Ancillary studies | 1014 | ||
| Systemic associations | 1016 | ||
| Evaluation | 1017 | ||
| Treatment | 1018 | ||
| Branch retinal artery occlusion | 1019 | ||
| Cilioretinal artery occlusion | 1020 | ||
| Combined retinal artery and vein occlusion | 1021 | ||
| Cotton-wool spots | 1022 | ||
| References | 1023 | ||
| 52 Acquired Retinal Macroaneurysms | 1026 | ||
| Clinical description | 1026 | ||
| Diagnosis of retinal macroaneurysm | 1026 | ||
| Natural course and treatment of retinal macroaneurysms | 1027 | ||
| References | 1028 | ||
| 53 Branch Vein Occlusion | 1029 | ||
| Introduction | 1029 | ||
| Pathogenesis | 1029 | ||
| Clinical features | 1029 | ||
| Symptoms | 1029 | ||
| Signs | 1029 | ||
| Complications | 1030 | ||
| Clinical evaluation | 1031 | ||
| Clinical examination | 1031 | ||
| Fluorescein angiography | 1031 | ||
| Wide-field angiography | 1031 | ||
| Optical coherence tomography | 1031 | ||
| Diagnostic workup | 1032 | ||
| Young patient | 1032 | ||
| Older patient | 1032 | ||
| Bilateral or numerous BRVO patients | 1032 | ||
| Treatment options | 1032 | ||
| Medical treatment | 1032 | ||
| Laser treatment | 1032 | ||
| Branch Vein Occlusion Study for macular edema | 1032 | ||
| Branch Vein Occlusion Study for neovascularization | 1034 | ||
| Steroid treatment | 1035 | ||
| SCORE (triamcinolone) study | 1035 | ||
| GENEVA (dexamethasone implant) study | 1035 | ||
| Anti-VEGF treatment | 1035 | ||
| BRAVO (ranibizumab) study | 1036 | ||
| Other anti-VEGF inhibitors | 1036 | ||
| Bevacizumab | 1036 | ||
| Pegaptanib | 1036 | ||
| Aflibercept | 1036 | ||
| Experimental treatments | 1037 | ||
| FAVOR (iluvien) study | 1037 | ||
| Surgical management | 1037 | ||
| Vitrectomy with or without sheathotomy | 1037 | ||
| Follow-up | 1037 | ||
| Conclusions | 1037 | ||
| Disclosure | 1037 | ||
| References | 1038 | ||
| 54 Central Retinal Vein Occlusion | 1039 | ||
| Clinical features | 1039 | ||
| Perfusion status | 1040 | ||
| Pathogenesis | 1041 | ||
| Risk factors and associations | 1042 | ||
| Clinical evaluation | 1042 | ||
| Therapeutic options | 1043 | ||
| Treatment of macular edema | 1043 | ||
| Observation | 1043 | ||
| Corticosteroid therapy | 1043 | ||
| Intravitreal anti-VEGF therapy | 1044 | ||
| Treatment of ocular neovascularization | 1045 | ||
| Laser photocoagulation | 1045 | ||
| Medical therapy | 1045 | ||
| Treatment of systemic medical conditions | 1045 | ||
| Alternative treatments | 1046 | ||
| Chorioretinal venous anastomosis | 1046 | ||
| Tissue plasminogen activator | 1046 | ||
| Surgical treatments | 1046 | ||
| Vitrectomy | 1046 | ||
| Radial optic neurotomy | 1047 | ||
| Follow-up | 1047 | ||
| Conclusion | 1047 | ||
| References | 1047 | ||
| 55 Macular Telangiectasia | 1050 | ||
| Introduction | 1050 | ||
| History, nomenclature, and classification of macular telangiectasia | 1050 | ||
| Classification | 1050 | ||
| Yannuzzi classification | 1050 | ||
| Epidemiology | 1050 | ||
| Prevalence of disease: estimates from population-based studies | 1050 | ||
| Beaver dam eye study | 1050 | ||
| Melbourne collaborative cohort study | 1050 | ||
| Clinical presentation | 1051 | ||
| Fundus appearance | 1051 | ||
| Retinal imaging | 1053 | ||
| Fundus autofluorescence | 1053 | ||
| Fluorescein angiography | 1053 | ||
| Optical coherence tomography | 1053 | ||
| Adaptive optics imaging | 1054 | ||
| Visual function | 1054 | ||
| Microperimetry | 1055 | ||
| Staging and prognostic factors | 1055 | ||
| Genetics | 1055 | ||
| Association of systemic diseases | 1055 | ||
| Differential diagnosis | 1055 | ||
| Clinicopathological correlation | 1056 | ||
| Therapeutic options | 1056 | ||
| Summary and future research directions | 1056 | ||
| References | 1056 | ||
| 56 Coats Disease | 1058 | ||
| History | 1058 | ||
| Histopathology, etiology, and pathogenesis | 1058 | ||
| Clinical presentation | 1059 | ||
| Diagnostic testing | 1061 | ||
| Fluorescein angiography | 1063 | ||
| Computed tomography | 1063 | ||
| Magnetic resonance imaging | 1063 | ||
| Doppler ultrasonography | 1063 | ||
| Blood testing | 1063 | ||
| Differential diagnosis | 1064 | ||
| Treatment | 1065 | ||
| Ablative therapies – laser photocoagulation and cryotherapy | 1065 | ||
| Pharmacologic therapies | 1066 | ||
| Surgery | 1066 | ||
| Outcomes | 1067 | ||
| References | 1068 | ||
| 57 Hemoglobinopathies | 1071 | ||
| Prevalence | 1071 | ||
| Genetic modifiers | 1071 | ||
| Pathophysiology | 1071 | ||
| Systemic manifestations | 1072 | ||
| Ophthalmic clinical features | 1072 | ||
| Retrobulbar and orbital involvement | 1072 | ||
| Anterior-segment involvement | 1073 | ||
| Posterior-segment involvement | 1073 | ||
| Vitreoretinal interface | 1073 | ||
| Optic nerve | 1073 | ||
| Macula | 1073 | ||
| Angioid streaks | 1074 | ||
| Retinal vasculature | 1074 | ||
| Nonproliferative sickle retinopathy | 1074 | ||
| Salmon patch hemorrhages | 1074 | ||
| Iridescent spots | 1074 | ||
| Black sunburst | 1076 | ||
| Proliferative sickle retinopathy | 1076 | ||
| Goldberg stages | 1076 | ||
| Stage I | 1076 | ||
| Stage II | 1077 | ||
| Stage III | 1078 | ||
| Stage IV | 1079 | ||
| Stage V | 1079 | ||
| Alternative classification schemes | 1079 | ||
| Incidence/prevalence | 1079 | ||
| Risk factors | 1079 | ||
| Natural history | 1079 | ||
| Ophthalmic treatments | 1079 | ||
| Imaging | 1080 | ||
| Potential therapeutic options for the future | 1080 | ||
| References | 1080 | ||
| 58 Radiation Retinopathy | 1083 | ||
| Introduction | 1083 | ||
| Etiology, pathogenesis, and histopathology | 1083 | ||
| Natural history and clinical features | 1084 | ||
| Classification | 1085 | ||
| Risk factors | 1086 | ||
| Incidence and dosimetry | 1086 | ||
| Radiation type | 1086 | ||
| Treatment modality | 1086 | ||
| Total radiation dose | 1086 | ||
| Fractionation schedule | 1086 | ||
| Volume of retina irradiated | 1086 | ||
| Total elapsed time | 1086 | ||
| Differential diagnosis and diagnostic evaluation | 1086 | ||
| Prevention and treatment | 1087 | ||
| Prognosis | 1088 | ||
| Conclusion | 1088 | ||
| References | 1088 | ||
| 59 Ocular Ischemic Syndrome | 1091 | ||
| Demographics and incidence | 1091 | ||
| Etiology | 1091 | ||
| Clinical presentation | 1091 | ||
| Visual loss | 1091 | ||
| Prolonged light recovery | 1091 | ||
| Scintillating scotomas | 1092 | ||
| Amaurosis fugax | 1092 | ||
| Pain | 1092 | ||
| Visual acuity | 1092 | ||
| External collaterals | 1092 | ||
| Anterior segment changes | 1092 | ||
| Posterior segment findings | 1092 | ||
| Ancillary studies | 1093 | ||
| Fluorescein angiography | 1093 | ||
| Electroretinography | 1098 | ||
| Carotid artery imaging | 1098 | ||
| Others | 1099 | ||
| Systemic associations | 1099 | ||
| Differential diagnosis | 1099 | ||
| Treatment | 1100 | ||
| Total carotid artery obstruction | 1100 | ||
| Less than total carotid artery obstruction | 1101 | ||
| Carotid endarterectomy in general | 1101.e1 | ||
| Medical therapy | 1101 | ||
| Direct ocular therapeutic modalities | 1102 | ||
| References | 1102.e1 | ||
| 60 Coagulopathies | 1104 | ||
| General considerations | 1104 | ||
| Disseminated intravascular coagulation | 1104 | ||
| Idiopathic thrombocytopenic purpura and thrombotic thrombocytopenic purpura | 1104 | ||
| HELLP syndrome | 1105 | ||
| Ophthalmic involvement | 1105 | ||
| Conclusion | 1106 | ||
| References | 1106 | ||
| 61 Pediatric Retinal Vascular Diseases | 1108 | ||
| Retinopathy of prematurity | 1108 | ||
| Historical perspective | 1108 | ||
| Early history | 1108 | ||
| Retinopathy of prematurity and contemporary nursery practices | 1108 | ||
| The role of oxygen | 1108 | ||
| Clinical findings | 1108 | ||
| Experimental findings | 1109 | ||
| Mechanism of oxygen’s effects on the immature retina | 1109 | ||
| Primary stage of retinal vasoconstriction and vascular occlusion | 1109 | ||
| Secondary stage of retinal neovascularization | 1109 | ||
| Pathogenesis | 1111 | ||
| Normal retinal vasculogenesis | 1111 | ||
| Pathogenesis of ROP | 1112 | ||
| International classification | 1113 | ||
| Zones of involved retina | 1113 | ||
| Extent of retinopathy of prematurity | 1114 | ||
| Staging | 1114 | ||
| Stage 1: demarcation line | 1114 | ||
| Stage 2: ridge | 1114 | ||
| Stage 3: ridge with extraretinal fibrovascular proliferation | 1115 | ||
| “Plus” and “pre-plus” disease | 1115 | ||
| Zone I ROP | 1115 | ||
| Aggressive posterior ROP | 1115 | ||
| Classification of retinal detachment | 1116 | ||
| Stage 4A: extrafoveal retinal detachment | 1116 | ||
| Stage 4B: partial retinal detachment including the fovea | 1116 | ||
| Stage 5: total retinal detachment | 1116 | ||
| Other factors related to retinal detachment | 1116 | ||
| Involution of retinopathy of prematurity | 1117 | ||
| Regressed rop: retinal detachment, strabismus, and amblyopia | 1117 | ||
| History of prematurity | 1117 | ||
| Ocular findings of regressed retinopathy of prematurity | 1117 | ||
| Myopia | 1117 | ||
| Other refractive and binocular defects | 1118 | ||
| Lens and corneal changes | 1118 | ||
| Glaucoma in retinopathy of prematurity | 1118 | ||
| Glaucoma in patients with advanced retinopathy | 1118 | ||
| Angle closure glaucoma in regressed retinopathy of prematurity | 1118 | ||
| Differential diagnosis | 1118 | ||
| Risk factors | 1118 | ||
| Examination procedures in the nursery | 1119 | ||
| General aspects and timing of the examination | 1119 | ||
| Screening guidelines | 1120 | ||
| Side-effects of the examination | 1120 | ||
| Techniques of eye examination | 1120 | ||
| Informing the patient’s family | 1120 | ||
| Prophylaxis and therapy | 1121 | ||
| The role of vitamin E | 1121 | ||
| The role of light | 1121 | ||
| Cryotherapy | 1121 | ||
| The multicenter trial of cryotherapy | 1121 | ||
| Treatment | 1121 | ||
| Results | 1121 | ||
| Current concepts in management of retinopathy of prematurity | 1121 | ||
| Treatment techniques | 1121 | ||
| Cryotherapy – special considerations | 1121 | ||
| Laser – special considerations | 1121 | ||
| The Early Treatment for Retinopathy of Prematurity trial | 1122 | ||
| Retinal detachment | 1122 | ||
| The ETROP study: better outcomes, changing clinical strategy | 1123 | ||
| Anti-VEGF therapy for posterior ROP | 1123 | ||
| Other pediatric retinal vascular diseases | 1123 | ||
| Coats disease | 1123 | ||
| Persistent fetal vasculature | 1124 | ||
| Incontinentia pigmenti | 1124 | ||
| Familial exudative vitreoretinopathy and Norrie disease | 1124 | ||
| References | 1125 | ||
| 62 Telescreening for Retinopathy of Prematurity | 1129 | ||
| Limitations of traditional care | 1129 | ||
| Telemedicine as an emerging approach | 1129 | ||
| Evaluation studies | 1129 | ||
| Accuracy | 1129 | ||
| Image quality | 1131 | ||
| Cost-effectiveness, speed, and satisfaction | 1131 | ||
| Evaluation of operational ROP telemedicine programs | 1132 | ||
| Barriers and challenges | 1132 | ||
| Future directions | 1132 | ||
| Disclosure | 1132 | ||
| References | 1132 | ||
| Section 3: Choroidal Vascular/Bruch's Membrane Disease | 1134 | ||
| 63 Epidemiology and Risk Factors for Age-Related Macular Degeneration | 1134 | ||
| Classification | 1134 | ||
| Prevalence | 1134 | ||
| Incidence | 1135 | ||
| Quality of life | 1135 | ||
| Sociodemographic risk factors | 1135 | ||
| Age | 1135 | ||
| Gender | 1135 | ||
| Race/ethnicity | 1136 | ||
| Socioeconomic status | 1136 | ||
| Ocular risk factors | 1136 | ||
| Refractive error | 1136 | ||
| Iris color | 1136 | ||
| Lens opacities, cataracts, and cataract surgery | 1136 | ||
| Cup-to-disc ratio | 1136 | ||
| Behavioral and lifestyle factors | 1136 | ||
| Smoking | 1136 | ||
| Antioxidants, vitamins, and minerals | 1137 | ||
| Alcohol intake | 1137 | ||
| Obesity and physical activity | 1138 | ||
| Sunlight exposure | 1138 | ||
| Medications | 1138 | ||
| Cardiovascular-related factors | 1138 | ||
| Cardiovascular diseases | 1138 | ||
| Blood pressure and hypertension | 1138 | ||
| Cholesterol levels and dietary fat intake | 1139 | ||
| Diabetes and hyperglycemia | 1139 | ||
| Hormonal and reproductive factors | 1139 | ||
| Inflammatory factors | 1140 | ||
| Genetic factors | 1140 | ||
| Conclusion | 1141 | ||
| References | 1141 | ||
| 64 Pathogenetic Mechanisms in Age-Related Macular Degeneration | 1145 | ||
| Introduction | 1145 | ||
| Structural changes | 1145 | ||
| Choroid | 1145 | ||
| Therapeutic implications | 1145 | ||
| Bruch’s membrane | 1145 | ||
| Therapeutic implications | 1146 | ||
| The retinal pigment epithelium | 1146 | ||
| Therapeutic implications | 1147 | ||
| Outer retina | 1148 | ||
| Therapeutic implications | 1148 | ||
| Conclusion | 1148 | ||
| References | 1148 | ||
| 65 Age-Related Macular Degeneration: Non-neovascular Early AMD, Intermediate AMD, and Geographic Atrophy | 1150 | ||
| Introduction | 1150 | ||
| Aging | 1151 | ||
| The aging eye – clinical findings | 1152 | ||
| The aging eye – morphologic changes | 1152 | ||
| Photoreceptors | 1154 | ||
| Retinal pigment epithelium | 1154 | ||
| Bruch’s membrane | 1154 | ||
| Changes in hydraulic conductivity | 1155 | ||
| Choroid | 1155 | ||
| Onset and progress of age-related macular degeneration | 1156 | ||
| Clinical features in the absence of drusen | 1156 | ||
| Morphologic changes | 1156 | ||
| Basal laminar deposit – early form | 1156 | ||
| Membranous debris | 1157 | ||
| Internal to the retinal pigment epithelium basement membrane | 1157 | ||
| External to the RPE basement membrane (basal linear deposit )* | 1158 | ||
| At the apex of the retinal pigment epithelium | 1158 | ||
| Basal laminar deposit – late form (diffuse thickening of the internal aspect of Bruch’s membrane) | 1159 | ||
| Retinal pigment epithelium and photoreceptors | 1159 | ||
| Bruch’s membrane and choroid | 1160 | ||
| Drusen | 1160 | ||
| Clinical grading | 1160 | ||
| Drusen type | 1160 | ||
| Drusen size | 1160 | ||
| Extent of fundus involvement | 1160 | ||
| Drusen distribution | 1160 | ||
| Drusen symmetry | 1160 | ||
| Drusen color | 1160 | ||
| Clinical grading of AMD severity | 1160 | ||
| Grading in scientific studies | 1161 | ||
| Imaging of drusen | 1161 | ||
| Fluorescence of drusen | 1161 | ||
| Autofluorescence | 1161 | ||
| Ocular coherence tomography (OCT) | 1161 | ||
| Pathologic considerations | 1161 | ||
| Clinicopathologic classification | 1162 | ||
| Small, hard (hyalinized, nodular) drusen | 1162 | ||
| Clinical features | 1162 | ||
| Formation | 1163 | ||
| Significance | 1163 | ||
| Soft (pseudosoft) cluster-derived drusen | 1163 | ||
| True soft drusen | 1164 | ||
| Granular soft drusen (synonyms: serogranular drusen, semisolid drusen, localized detachment of the basal linear deposit) | 1164 | ||
| Soft, fluid (serous) drusen and drusenoid pigment epithelial detachments | 1165 | ||
| Disappearance of drusen following prophylactic laser photocoagulation | 1166 | ||
| Soft (membranous) drusen (localized accumulation of the basal linear deposit) | 1166 | ||
| Reticular pseudodrusen, reticular drusen, subretinal drusenoid deposits | 1168 | ||
| Regressing (fading) drusen (localized detachment of the basal laminar deposit within an area of retinal pigment epithelium and photoreceptor atrophy) | 1169 | ||
| Outcome of drusen | 1170 | ||
| Histochemistry | 1171 | ||
| Incipient atrophy (nongeographic atrophy) | 1172 | ||
| Pathology | 1172 | ||
| Choroidal perfusion in age-related macular degeneration | 1172 | ||
| Relationship to age-related macular degeneration | 1173 | ||
| Functional effects | 1173 | ||
| Prognostic value | 1174 | ||
| Geographic atrophy | 1174 | ||
| Evolution | 1174 | ||
| Drusen-unrelated atrophy | 1174 | ||
| Drusen-related atrophy | 1174 | ||
| Following pigment epithelial detachments | 1174 | ||
| Pathology | 1175 | ||
| Choroidal atrophy | 1175 | ||
| Imaging geographic atrophy | 1175 | ||
| Fluorescein angiography | 1175 | ||
| Fundus autofluorescence | 1175 | ||
| Optical coherence tomography | 1175 | ||
| Clinical significance of geographic atrophy | 1176 | ||
| Prognosis | 1176 | ||
| Geographic atrophy and choroidal neovascularization | 1177 | ||
| Intermediate stage of age-related macular degeneration and cataract | 1177 | ||
| Age-related macular degeneration and age | 1177 | ||
| Management of non-neovascular AMD | 1178 | ||
| References | 1180 | ||
| 66 Neovascular (Exudative or “Wet”) Age-Related Macular Degeneration | 1183 | ||
| Epidemiology | 1183 | ||
| Risk factors | 1183 | ||
| Clinical (including biomicroscopic) presentation | 1183 | ||
| Overview | 1183 | ||
| Retinal pigment epithelial detachments | 1184 | ||
| Breakthrough vitreous hemorrhage | 1185 | ||
| Massive subretinal hemorrhage | 1185 | ||
| Retinal pigment epithelial tears | 1185 | ||
| Disciform scars | 1185 | ||
| Fluorescein angiographic features | 1185 | ||
| Overview | 1185 | ||
| Classic choroidal neovascularization | 1186 | ||
| Occult choroidal neovascularization | 1187 | ||
| Other terms relevant to interpreting fluorescein angiography of choroidal neovascularization | 1190 | ||
| Retinal pigment epithelium detachments in age-related macular degeneration | 1190 | ||
| Other angiographic features | 1192 | ||
| Speckled hyperfluorescence | 1192 | ||
| Fading choroidal neovascularization | 1192 | ||
| Feeder vessels | 1194 | ||
| Retinal lesion anastomosis (“retinal angiomatous proliferans” or “chorioretinal anastomosis”) | 1194 | ||
| Loculated fluid | 1195 | ||
| Retinal pigment epithelial tears | 1195 | ||
| Disciform scars | 1195 | ||
| Pathogenesis | 1196 | ||
| Choroidal neovascularization | 1196 | ||
| Histopathology | 1196 | ||
| Associated factors | 1197 | ||
| Differential diagnosis | 1197 | ||
| Choroidal neovascularization | 1197 | ||
| Vitreous hemorrhage | 1198 | ||
| Natural history | 1198 | ||
| Well-defined extrafoveal and juxtafoveal choroidal neovascularization | 1198 | ||
| Subfoveal choroidal neovascularization | 1199 | ||
| Natural course of large subfoveal subretinal hemorrhage in age-related macular degeneration | 1199 | ||
| Retinal pigment epithelial tears | 1199 | ||
| Laser photocoagulation treatment | 1199 | ||
| Laser treatment of well-defined choroidal neovascular lesions | 1199 | ||
| Preparation for laser photocoagulation treatment | 1199 | ||
| Macular Photocoagulation Study photocoagulation techniques | 1200 | ||
| Evaluations following laser photocoagulation | 1201 | ||
| Complications of laser photocoagulation | 1201 | ||
| Photodynamic therapy | 1202 | ||
| Results of photodynamic therapy treatment | 1202 | ||
| Preparation for photodynamic therapy | 1202 | ||
| Follow-up after photodynamic therapy | 1203 | ||
| Pharmacologic therapy with anti-VEGF products and overall management approach to CNV in AMD | 1203 | ||
| Efficacy of ranibizumab vs PDT with verteporfin for predominantly classic subfoveal CNV lesions | 1204 | ||
| Efficacy of ranibizumab vs sham treatment for minimally classic or occult with no classic subfoveal choroidal neovascular lesions and presumed recent disease progression | 1205 | ||
| Safety of ranibizumab | 1205 | ||
| Impact of noninferiority results on frequency of treatment and the role of aflibercept or bevacizumab in place of ranibizumab | 1206 | ||
| Potential implications of anti-VEGF noninferiority trials results on clinical practice | 1207 | ||
| Follow-up after deciding to initiate anti-VEGF therapy for neovascular AMD | 1207 | ||
| Early identification of choroidal neovascularization | 1207 | ||
| Prevention of choroidal neovascularization | 1207 | ||
| Risk of fellow-eye involvement | 1207 | ||
| Additional therapies | 1208 | ||
| Submacular surgery | 1208 | ||
| Indocyanine green angiography | 1208 | ||
| Radiation therapy | 1209 | ||
| Other pharmacologic therapies and combination therapies | 1209 | ||
| Patient education and rehabilitation | 1209 | ||
| Disclosure | 1210 | ||
| References | 1210 | ||
| 67 Pharmacotherapy of Age-Related Macular Degeneration | 1213 | ||
| Introduction | 1213 | ||
| Etiologic factors | 1213 | ||
| Genetic susceptibility | 1213 | ||
| Environmental factors | 1216 | ||
| Diet | 1216 | ||
| Smoking* | 1216 | ||
| Light exposure | 1216 | ||
| Use of medications | 1216 | ||
| Systemic risk factors | 1216 | ||
| The pathophysiology of exudative amd: the crucial role of cytokines | 1217 | ||
| Definition and steps in angiogenesis | 1217 | ||
| VEGF and other positive and negative modulators of angiogenesis | 1217 | ||
| Vascular permeability | 1218 | ||
| VEGF receptors | 1219 | ||
| VEGFR-2 (KDR in humans or FLK-1 in mice) | 1219 | ||
| Other cytokines and regulators of angiogenesis | 1220 | ||
| Naturally occurring upregulators of angiogenesis | 1220 | ||
| Fibroblast growth factor and integrins | 1220 | ||
| Platelet-derived growth factor | 1221 | ||
| Angiopoietins | 1221 | ||
| Matrix metalloproteinases and tissue inhibitors of metalloproteinases | 1221 | ||
| Naturally occurring downregulators of angiogenesis | 1221 | ||
| Pigment epithelial-derived factor | 1221 | ||
| Other cytokines | 1221 | ||
| Agents currently in use or under investigation: non-neovascular AMD | 1222 | ||
| Antioxidants, vitamins, and cofactors | 1222 | ||
| Age-Related Eye Disease Study (AREDS) and related supplements | 1222 | ||
| Visual cycle inhibitors* | 1223 | ||
| Complement modulators* | 1223 | ||
| Agents currently in use or under investigation: neovascular AMD | 1224 | ||
| VEGF inhibitors | 1224 | ||
| Direct VEGF inhibitors | 1225 | ||
| Monoclonal antibody: bevacizumab (Avastin) | 1225 | ||
| Antigen binding fragment: ranibizumab (Lucentis) | 1227 | ||
| Comparison of Age-related Macular Degeneration Treatments Trial (CATT) | 1230 | ||
| Soluble receptor: aflibercept (VEGF-TRAP EYE) | 1241 | ||
| KH902 | 1242 | ||
| Adeno-associated viral vector (AAV) gene transduction | 1242 | ||
| Oligonucleotide aptamer (pegaptanib – Macugen) | 1243 | ||
| Small interfering RNA (siRNA) | 1243 | ||
| Intracellular signaling blockers | 1243 | ||
| PDGF/PDGFR inhibitors* | 1243 | ||
| TrpRS | 1244 | ||
| Protein kinase C inhibitors | 1244 | ||
| Complement inhibitors* | 1244 | ||
| Naturally occurring inhibitory polypeptides and inducible cleavage products | 1245 | ||
| Pigment epithelial-derived factor | 1245 | ||
| Pathologically expressed circulating molecules | 1245 | ||
| Angiostatin and endostatin | 1245 | ||
| Extracellular matrix modulators | 1245 | ||
| Integrin antagonists | 1246 | ||
| MMP inhibitors | 1246 | ||
| Other molecules | 1246 | ||
| Squalamine | 1247 | ||
| The role of steroids and other immunomodulators | 1247 | ||
| Steroids | 1247 | ||
| Rapamycin | 1247 | ||
| Photodynamic Therapy | 1248 | ||
| Pharmacology of photodynamic sensitizers | 1248 | ||
| Verteporfin (Visudyne) | 1248 | ||
| Combination therapies* | 1249 | ||
| Conclusion | 1250 | ||
| References | 1250 | ||
| 68 Myopic Macular Degeneration | 1256 | ||
| Epidemiology | 1256 | ||
| Pathogenesis | 1256 | ||
| Histopathology | 1258 | ||
| The sclera | 1258 | ||
| Choroid and retinal pigment epithelium | 1258 | ||
| Animal models | 1259 | ||
| Features of the myopic fundus | 1259 | ||
| Myopic conus | 1259 | ||
| Posterior staphyloma | 1260 | ||
| Myopic chorioretinal atrophy | 1262 | ||
| Lacquer cracks | 1262 | ||
| Myopic chorodial neovascularization | 1262 | ||
| Myopic macular retinoschisis or myopic foveoschisis | 1263 | ||
| Natural course and treatment of myopic CNV | 1263 | ||
| Conclusion | 1264 | ||
| References | 1264 | ||
| 69 Angioid Streaks | 1267 | ||
| Introduction | 1267 | ||
| Histopathology | 1267 | ||
| Systemic associations | 1267 | ||
| Ocular manifestations and clinical course | 1268 | ||
| Ocular imaging and diagnosis | 1268 | ||
| Fluorescein angiography (FA) | 1268 | ||
| Fundus autofluorescence (FAF) | 1268 | ||
| Indocyanine green angiography (ICGA) | 1268 | ||
| Spectral domain optical coherence tomography (SD-OCT) | 1269 | ||
| Therapy | 1269 | ||
| Laser photocoagulation | 1269 | ||
| Transpupillary thermotherapy | 1270 | ||
| Photodynamic therapy | 1270 | ||
| Macular translocation | 1270 | ||
| Anti-VEGF treatment | 1271 | ||
| Bevacizumab | 1271 | ||
| Ranibizumab | 1272 | ||
| Combination therapy | 1272 | ||
| Conclusion | 1272 | ||
| References | 1272 | ||
| 70 Ocular Histoplasmosis | 1274 | ||
| Historical perspective | 1274 | ||
| Clinical features of ocular histoplasmosis | 1274 | ||
| Differential diagnosis | 1274 | ||
| Relationship of ocular disease to systemic infection | 1277 | ||
| Clinical features of systemic infection | 1277 | ||
| Epidemiology of ocular histoplasmosis | 1277 | ||
| Geographic distribution of H. capsulatum in the USA | 1277 | ||
| Prevalence and incidence | 1277 | ||
| Age | 1277 | ||
| Gender and race | 1278 | ||
| Histocompatibility antigens and genetic predisposition | 1278 | ||
| Pathogenesis | 1278 | ||
| Natural history of ocular histoplasmosis and public health implications | 1278 | ||
| Treatment | 1278 | ||
| Laser photocoagulation | 1278 | ||
| Photodynamic therapy | 1280 | ||
| Anti-VEGF therapy | 1280 | ||
| Combination therapy | 1281 | ||
| Intravitreal triamcinolone | 1281 | ||
| Submacular surgery and macular translocation | 1281 | ||
| References | 1282 | ||
| 71 Polypoidal Choroidal Vasculopathy | 1285 | ||
| Introduction | 1285 | ||
| Pathogenesis | 1285 | ||
| Clinical features | 1286 | ||
| Demographics | 1286 | ||
| Clinical findings | 1286 | ||
| Angiographic features | 1287 | ||
| Classification | 1288 | ||
| Differential diagnosis | 1288 | ||
| Neovascular age-related macular degeneration | 1288 | ||
| Central serous chorioretinopathy | 1288 | ||
| Treatment | 1289 | ||
| Thermal laser photocoagulation | 1289 | ||
| Photodynamic therapy | 1289 | ||
| Anti-VEGF therapy | 1289 | ||
| Combination therapy | 1290 | ||
| References | 1290 | ||
| 72 Central Serous Chorioretinopathy | 1291 | ||
| Introduction | 1291 | ||
| Pathogenesis, predisposition, and risk factors | 1291 | ||
| Clinical features | 1292 | ||
| Demographics | 1292 | ||
| Symptoms | 1292 | ||
| Signs | 1292 | ||
| Investigations | 1292 | ||
| Fluorescein angiography | 1292 | ||
| Indocyanine green angiography | 1293 | ||
| Optical coherence tomography | 1293 | ||
| Fundus autofluorescence | 1294 | ||
| Multifocal electroretinography | 1294 | ||
| Microperimetry | 1295 | ||
| Natural history | 1295 | ||
| Differential diagnosis | 1298 | ||
| Optic disc pit | 1298 | ||
| Age-related macular degeneration | 1298 | ||
| Polypoidal choroidal vasculopathy | 1298 | ||
| Inflammatory and infectious diseases | 1300 | ||
| Autoimmune and vascular disorders | 1300 | ||
| Intraocular tumors | 1300 | ||
| Treatment | 1300 | ||
| Observation in most cases | 1300 | ||
| Treatment for selected cases | 1301 | ||
| Safety-enhanced photodynamic therapy in selected cases | 1301 | ||
| PDT with verteporfin | 1301 | ||
| Conventional PDT with normal dosage and fluence | 1301 | ||
| Safety-enhanced PDT with reduced verteporfin dosage | 1301 | ||
| Safety-enhanced PDT with reduced laser fluence | 1302 | ||
| Historical thermal (argon) laser photocoagulation and micropulsed diode laser | 1302 | ||
| Transpupillary thermotherapy | 1302 | ||
| Intravitreal anti-VEGF therapy with or without adjuvant PDT | 1302 | ||
| Anticorticosteroid treatment | 1302 | ||
| Management of special variants of CSC | 1303 | ||
| Bullous CSC and its putative management | 1303 | ||
| Other potential but still exploratory systemic therapies | 1303 | ||
| Systemic acetazolamide | 1303 | ||
| Antiadrenergic blockage and beta-blockers | 1303 | ||
| Aspirin, finasteride, anti-Helicobacter pylori treatment | 1303 | ||
| Conclusion | 1303 | ||
| References | 1303 | ||
| 73 Uveal Effusion Syndrome and Hypotony Maculopathy | 1306 | ||
| Uveal effusion syndrome | 1306 | ||
| Introduction | 1306 | ||
| Pathophysiology of ciliochoroidal effusions | 1306 | ||
| General mechanisms | 1306 | ||
| Idiopathic and nanophthalmic uveal effusion | 1307 | ||
| Clinical features | 1307 | ||
| Diagnostic studies | 1309 | ||
| Ophthalmic ultrasound | 1309 | ||
| Angiography and optical coherence tomography | 1309 | ||
| Differential diagnosis | 1309 | ||
| Congenital and acquired scleropathies | 1309 | ||
| Hydrodynamic effusions | 1310 | ||
| Inflammatory factors | 1310 | ||
| Neoplastic effusions | 1310 | ||
| Treatment of idiopathic uveal effusion syndrome | 1311 | ||
| Scleral thinning procedures | 1311 | ||
| Pars plana vitrectomy | 1311 | ||
| Vortex vein decompression | 1311 | ||
| Conclusion | 1311 | ||
| Hypotony maculopathy | 1312 | ||
| Introduction | 1312 | ||
| Clinical features | 1312 | ||
| Diagnosis | 1313 | ||
| Fluorescein angiography | 1313 | ||
| Ocular ultrasound | 1313 | ||
| Optical coherence tomography | 1313 | ||
| Pathogenesis | 1314 | ||
| Hypotony | 1314 | ||
| Decreased aqueous production | 1314 | ||
| Increased aqueous outflow | 1314 | ||
| Mechanism of maculopathy | 1315 | ||
| Mechanism of optic disc edema | 1315 | ||
| Differential diagnosis | 1315 | ||
| Idiopathic chorioretinal folds | 1315 | ||
| Retrobulbar mass lesions | 1315 | ||
| Scleral inflammation | 1315 | ||
| Scleral buckle | 1315 | ||
| Choroidal tumors | 1315 | ||
| Choroidal neovascularization | 1315 | ||
| Focal chorioretinal scars | 1315 | ||
| Optic nerve head disorders | 1315 | ||
| Retinal folds | 1315 | ||
| Treatment | 1315 | ||
| Conclusion | 1316 | ||
| References | 1316 | ||
| Section 4: Inflammatory Disease/Uveitis | 1318 | ||
| Inflammation | 1318 | ||
| 74 Sympathetic Ophthalmia | 1318 | ||
| Introduction | 1318 | ||
| Epidemiology | 1318 | ||
| Pathogenesis | 1318 | ||
| Immunopathology | 1319 | ||
| Clinical findings | 1320 | ||
| Diagnosis | 1321 | ||
| Differential diagnosis | 1321 | ||
| Course and complications | 1321 | ||
| Therapy | 1323 | ||
| Prevention | 1323 | ||
| Prognosis | 1324 | ||
| References | 1324 | ||
| 75 Vogt–Koyanagi–Harada Disease | 1326 | ||
| Introduction and historical aspects | 1326 | ||
| Epidemiology | 1326 | ||
| Clinical description | 1326 | ||
| The prodromal stage | 1327 | ||
| The acute uveitic stage | 1327 | ||
| The chronic uveitic stage | 1327 | ||
| The chronic recurrent stage | 1331 | ||
| Frequency of distinguishing clinical features | 1331 | ||
| Pathology and pathogenesis | 1331 | ||
| Investigations | 1333 | ||
| Imaging studies | 1333 | ||
| Lumbar puncture | 1333 | ||
| Differential diagnosis | 1334 | ||
| Treatment | 1334 | ||
| Complications and management | 1334 | ||
| Prognosis | 1335 | ||
| Conclusion | 1335 | ||
| References | 1335 | ||
| 76 White Spot Syndromes and Related Diseases | 1337 | ||
| Introduction | 1337 | ||
| Birdshot chorioretinopathy | 1337 | ||
| Clinical course | 1337 | ||
| Clinical symptoms | 1337 | ||
| Epidemiology | 1337 | ||
| Fundus findings | 1338 | ||
| Other ocular findings | 1338 | ||
| Clinical course and prognosis | 1338 | ||
| Imaging | 1338 | ||
| Fluorescein angiography | 1338 | ||
| Indocyanine green angiography | 1339 | ||
| Optical coherence tomography | 1339 | ||
| Fundus autofluorescence | 1340 | ||
| Electrophysiology | 1340 | ||
| Electroretinogram | 1340 | ||
| Electrooculogram | 1340 | ||
| Visual field testing | 1340 | ||
| Systemic associations | 1340 | ||
| Pathogenesis | 1340 | ||
| Differential diagnosis | 1341 | ||
| Management/treatment | 1341 | ||
| Immunosuppressive therapy | 1341 | ||
| Summary | 1341 | ||
| Placoid diseases | 1341 | ||
| Acute posterior multifocal placoid pigment epitheliopathy | 1341 | ||
| Clinical course | 1341 | ||
| Clinical symptoms | 1341 | ||
| Epidemiology | 1341 | ||
| Fundus findings | 1342 | ||
| Other ocular findings | 1342 | ||
| Clinical course and prognosis | 1342 | ||
| Imaging | 1342 | ||
| Fluorescein angiography | 1342 | ||
| Indocyanine green angiography | 1343 | ||
| Optical coherence tomography | 1344 | ||
| Fundus autofluorescence | 1344 | ||
| Electrophysiology | 1344 | ||
| Systemic associations | 1345 | ||
| Pathogenesis | 1345 | ||
| Differential diagnosis | 1345 | ||
| Management/treatment | 1345 | ||
| Summary | 1345 | ||
| Serpiginous choroiditis | 1346 | ||
| Clinical course | 1346 | ||
| Clinical symptoms | 1346 | ||
| Epidemiology | 1346 | ||
| Fundus findings | 1346 | ||
| Other ocular findings | 1346 | ||
| Clinical course and prognosis | 1346 | ||
| Imaging | 1347 | ||
| Fluorescein angiography | 1347 | ||
| Indocyanine green angiography | 1347 | ||
| Optical coherence tomography | 1347 | ||
| Fundus autofluorescence | 1347 | ||
| Electrophysiology | 1348 | ||
| Perimetry | 1349 | ||
| Systemic associations | 1349 | ||
| Pathogenesis | 1349 | ||
| Differential diagnosis | 1349 | ||
| Management/treatment | 1350 | ||
| Summary | 1350 | ||
| Relentless placoid chorioretinitis | 1350 | ||
| Clinical course | 1350 | ||
| Clinical symptoms | 1350 | ||
| Epidemiology | 1350 | ||
| Fundus findings | 1351 | ||
| Other ocular findings | 1351 | ||
| Clinical course and prognosis | 1351 | ||
| Imaging | 1352 | ||
| Fluorescein angiography | 1352 | ||
| Indocyanine green angiography | 1352 | ||
| Optical coherence tomography | 1352 | ||
| Fundus autofluorescence | 1352 | ||
| Electrophysiology | 1352 | ||
| Systemic associations | 1352 | ||
| Pathogenesis | 1352 | ||
| Differential diagnosis | 1352 | ||
| Management/treatment | 1353 | ||
| Summary | 1353 | ||
| Persistent placoid maculopathy | 1353 | ||
| Clinical course | 1353 | ||
| Clinical symptoms | 1353 | ||
| Epidemiology | 1353 | ||
| Fundus findings | 1353 | ||
| Other ocular findings | 1353 | ||
| Clinical course and prognosis | 1353 | ||
| Imaging | 1353 | ||
| Fluorescein angiography | 1353 | ||
| Indocyanine green angiography | 1353 | ||
| Optical coherence tomography | 1355 | ||
| Fundus autofluorescence | 1355 | ||
| Electrophysiology | 1355 | ||
| Systemic associations | 1355 | ||
| Pathogenesis | 1355 | ||
| Differential diagnosis | 1355 | ||
| Management/treatment | 1355 | ||
| Summary | 1355 | ||
| Multifocal choroiditis and panuveitis, punctate inner choroidopathy, and progressive subretinal fibrosis and uveitis syndrome | 1355 | ||
| Multifocal choroiditis and panuveitis | 1355 | ||
| Clinical course | 1355 | ||
| Clinical symptoms | 1355 | ||
| Epidemiology | 1355 | ||
| Fundus findings | 1355 | ||
| Other ocular findings | 1356 | ||
| Clinical course and prognosis | 1357 | ||
| Imaging | 1358 | ||
| Fluorescein angiography | 1358 | ||
| Indocyanine green angiography | 1358 | ||
| Optical coherence tomography | 1358 | ||
| Fundus autofluorescence | 1359 | ||
| Electrophysiology testing | 1359 | ||
| Visual field testing | 1359 | ||
| Systemic associations | 1359 | ||
| Punctate inner choroidopathy | 1359 | ||
| Clinical course | 1359 | ||
| Clinical symptoms | 1359 | ||
| Epidemiology | 1359 | ||
| Fundus findings | 1360 | ||
| Other ocular findings | 1360 | ||
| Clinical course and prognosis | 1360 | ||
| Imaging | 1361 | ||
| Fluorescein angiography | 1361 | ||
| Indocyanine green angiography | 1361 | ||
| Optical coherence tomography | 1361 | ||
| Electrophysiology | 1362 | ||
| Visual field testing | 1362 | ||
| Pathogenesis | 1362 | ||
| Differential diagnosis | 1362 | ||
| Differentiation of MFC and POHS | 1362 | ||
| Differentiation of MFC and PIC | 1362 | ||
| Section 5: Miscellaneous | 1532 | ||
| 89 Drug Toxicity of the Posterior Segment | 1532 | ||
| Disruption of the retina and retinal pigment epithelium | 1532 | ||
| Phenothiazines | 1532 | ||
| Thioridazine | 1532 | ||
| Chlorpromazine | 1533 | ||
| Chloroquine derivatives | 1533 | ||
| Chloroquine | 1533 | ||
| Hydroxychloroquine | 1535 | ||
| Quinine sulfate | 1536 | ||
| Clofazimine | 1537 | ||
| Dideoxyinosine (DDI) | 1537 | ||
| Deferoxamine | 1537 | ||
| Corticosteroid preparations | 1537 | ||
| Cisplatin and BCNU (carmustine) | 1537 | ||
| Miscellaneous agents | 1538 | ||
| Vascular damage | 1539 | ||
| Quinine sulfate | 1539 | ||
| Cisplatin and BCNU (carmustine) | 1539 | ||
| Talc | 1539 | ||
| Oral contraceptives | 1539 | ||
| Aminoglycoside antibiotics | 1540 | ||
| Interferon | 1541 | ||
| Miscellaneous agents | 1541 | ||
| Cystoid macular edema | 1541 | ||
| Epinephrine | 1541 | ||
| Nicotinic acid | 1541 | ||
| Latanoprost | 1541 | ||
| Paclitaxel/docetaxel | 1541 | ||
| Retinal folds | 1541 | ||
| Sulfa antibiotics, acetazolamide, chlorthalidone, disothiazide, ethoxyzolamide, hydrochlorothiazide, metronidazole, sulphonamide, topiramate, triamterene | 1541 | ||
| Crystalline retinopathy | 1543 | ||
| Tamoxifen | 1543 | ||
| Canthaxanthine | 1548 | ||
| Methoxyflurane | 1548 | ||
| Talc | 1548 | ||
| Miscellaneous agents | 1548 | ||
| Uveitis | 1549 | ||
| Rifabutin | 1549 | ||
| Cidofovir | 1549 | ||
| Latanoprost | 1549 | ||
| Miscellaneous | 1549 | ||
| Cardiac glycosides | 1549 | ||
| Methanol | 1549 | ||
| Vigabatrin | 1549 | ||
| Sildenafil, tadalafil, vardenafil | 1549 | ||
| Summary | 1550 | ||
| References | 1550 | ||
| 90 Photic Retinal Injuries:Mechanisms, Hazards, and Prevention | 1555 | ||
| Photomechanical effects | 1555 | ||
| Photomechanical mechanisms | 1555 | ||
| Photomechanical retinal injuries | 1555 | ||
| Photothermal effects | 1556 | ||
| Photothermal mechanisms | 1556 | ||
| Photothermal retinal injuries | 1556 | ||
| Operating room or medical office injuries | 1556 | ||
| Slit-lamp photocoagulators | 1556 | ||
| Laser pointers and other consumer laser devices | 1556 | ||
| Photochemical effects | 1556 | ||
| Photochemical mechanisms | 1557 | ||
| Photochemical retinal injuries | 1557 | ||
| Solar and welder’s maculopathy | 1557 | ||
| Operating microscope and endoilluminator injuries | 1558 | ||
| Ophthalmoscope and fundus camera exposure | 1560 | ||
| Environmental issues | 1560 | ||
| Light and macular degeneration | 1560 | ||
| Sunglasses | 1560 | ||
| Safety standards | 1560 | ||
| Practical considerations | 1561 | ||
| Conclusion | 1561 | ||
| References | 1561 | ||
| 91 Traumatic Chorioretinopathies | 1564 | ||
| Epidemiology | 1564 | ||
| Chorioretinopathies from direct ocular injuries | 1564 | ||
| Commotio retinae | 1564 | ||
| Retinal concussion | 1565 | ||
| Retinal contusion | 1565 | ||
| Choroidal rupture | 1565 | ||
| Traumatic macular hole | 1565 | ||
| Traumatic chorioretinal rupture | 1566 | ||
| Traumatic retinal pigment epithelial tears | 1566 | ||
| Traumatic retinal tears and detachments | 1566 | ||
| Retinal dialyses | 1567 | ||
| Optic nerve avulsion | 1567 | ||
| Chorioretinopathies from indirect ocular injuries | 1568 | ||
| Purtscher’s retinopathy | 1568 | ||
| Terson’s syndrome | 1568 | ||
| Shaken-baby syndrome | 1568 | ||
| Valsalva retinopathy | 1569 | ||
| Fat embolism syndrome | 1569 | ||
| Whiplash retinopathy | 1569 | ||
| Acknowledgments | 1569.e1 | ||
| References | 1569 | ||
| 92 Pregnancy-Related Diseases | 1571 | ||
| Retinal and choroidal disorders arising in pregnancy | 1571 | ||
| Pre-eclampsia and eclampsia | 1571 | ||
| Retinopathy in pre-eclampsia and eclampsia | 1571 | ||
| Choroidopathy in pre-eclampsia and eclampsia | 1572 | ||
| Other ocular changes seen in pre-eclampsia and eclampsia | 1572 | ||
| Central serous chorioretinopathy | 1572 | ||
| Occlusive vascular disorders | 1573 | ||
| Retinal artery occlusion | 1573 | ||
| Retinal vein occlusion | 1573 | ||
| Disseminated intravascular coagulopathy | 1573 | ||
| Thrombotic thrombocytopenic purpura | 1573 | ||
| Amniotic fluid embolism | 1574 | ||
| Uveal melanoma | 1574 | ||
| Other changes arising in pregnancy | 1574 | ||
| Pre-existing conditions | 1574 | ||
| Diabetic retinopathy | 1574 | ||
| Progression of diabetic retinopathy during pregnancy | 1575 | ||
| Short- and long-term effects of pregnancy on diabetic retinopathy | 1575 | ||
| The role of baseline retinopathy status, duration of diabetes, and metabolic control | 1576 | ||
| No initial retinopathy | 1576 | ||
| Mild nonproliferative diabetic retinopathy | 1576 | ||
| Moderate to severe nonproliferative diabetic retinopathy | 1576 | ||
| Proliferative retinopathy | 1577 | ||
| Diabetic macular edema in pregnancy | 1577 | ||
| Other risk factors for progression of diabetic retinopathy during pregnancy | 1578 | ||
| Diabetic retinopathy and maternal and fetal wellbeing | 1578 | ||
| Toxoplasmic retinochoroiditis | 1578 | ||
| Noninfectious uveitis | 1578 | ||
| Other retinal disorders | 1579 | ||
| Diagnostic testing and therapy | 1579 | ||
| Photodynamic therapy | 1579 | ||
| Anti-VEGF therapy | 1579 | ||
| Conclusion | 1579 | ||
| References | 1580 | ||
| 93 Optic Disc Anomalies, Pits, and Associated Serous Macular Detachment | 1583 | ||
| Optic disc anomalies | 1583 | ||
| Megalopapilla | 1583 | ||
| Aplasia | 1583 | ||
| Hypoplasia | 1583 | ||
| Cavities in the optic nerve head | 1583 | ||
| Anatomy | 1583 | ||
| Optic disc pits | 1583 | ||
| Visual defects | 1584 | ||
| Associated retinal changes | 1584 | ||
| Macular detachment | 1585 | ||
| Appearance of maculopathy | 1585 | ||
| Course of associated serous macular detachment | 1585 | ||
| Theories of pathophysiology | 1586 | ||
| Prognosis | 1586 | ||
| Treatment | 1586 | ||
| References | 1587 | ||
| 94 Retina-Related Clinical Trials: A Resource Bibliography | 1589 | ||
| Diabetic retinopathy | 1589 | ||
| Diabetic Retinopathy Study (DRS) | 1589 | ||
| UK Multicentre Controlled Study | 1590 | ||
| Diabetic Retinopathy Vitrectomy Study (DRVS) | 1590 | ||
| Early Treatment Diabetic Retinopathy Study (ETDRS) | 1590 | ||
| Sorbinil Retinopathy Trial (SRT) | 1592 | ||
| Krypton Argon Regression of Neovascularization Study (KARNS) | 1592 | ||
| Diabetes Control and Complications Trial (DCCT) | 1592 | ||
| UK Prospective Diabetes Study (UKPDS) | 1594 | ||
| Diabetic Retinopathy Clinical Research Network (DRCR.net) | 1595 | ||
| Other diabetic retinopathy trials | 1596 | ||
| Vein occlusions | 1597 | ||
| Cochrane Systematic Review | 1597 | ||
| Branch Vein Occlusion Study (BVOS) | 1597 | ||
| Central Vein Occlusion Study (CVOS) | 1597 | ||
| Standard Care vs COrticosteroid for REtinal Vein Occlusion Study (SCORE) | 1597 | ||
| Other clinical trials for vein occlusion | 1598 | ||
| Age-related macular degeneration and other conditions associated with choroidal neovascularization | 1598 | ||
| Treatment trials | 1598 | ||
| Macular Photocoagulation Study (MPS) | 1598 | ||
| Other trials of laser treatment of choroidal neovascularization and a Cochrane systematic review | 1600 | ||
| Trials of photodynamic therapy with verteporfin (Visudyne) and a Cochrane systematic review | 1601 | ||
| Submacular Surgery Trials (SST) | 1602 | ||
| Trials of radiotherapy for choroidal neovascularization and a Cochrane systematic review | 1603 | ||
| Trials of anti-VEGF therapy for choroidal neovascularization | 1604 | ||
| V.I.S.I.O.N. | 1604 | ||
| MARINA | 1604 | ||
| ANCHOR | 1604 | ||
| FOCUS | 1604 | ||
| ABC Trial | 1604 | ||
| PIER and other randomized trials of ranibizumab for neovascular age-related macular degeneration | 1605 | ||
| Comparison of Age-related Macular Degeneration Treatments Trial (CATT) | 1605 | ||
| Trials of other pharmacologic treatments for choroidal neovascularization | 1605 | ||
| Prevention trials | 1605 | ||
| Age-Related Eye Disease Study (AREDS and AREDS 2) | 1605 | ||
| Complications of AMD Prevention Trial (CAPT) | 1606 | ||
| Other trials of potential preventive treatment for age-related macular degeneration and a Cochrane systematic review | 1607 | ||
| Retinopathy of prematurity | 1608 | ||
| Multicenter Trial of Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) | 1608 | ||
| Multicenter Study of Light Reduction in Retinopathy of Prematurity (LIGHT-ROP) | 1610 | ||
| Supplemental Therapeutic Oxygen for Prethreshold Retinopathy of Prematurity (STOP-ROP) | 1610 | ||
| Early Treatment of Retinopathy for Prematurity (ETROP or EARLY-ROP) | 1610 | ||
| Other retinal and retina-related conditions | 1610 | ||
| Collaborative Ocular Melanoma Study (COMS) | 1610 | ||
| Studies of the Ocular Complications of AIDS (SOCA) | 1611 | ||
| Trials of treatment of posterior uveitis | 1612 | ||
| Silicone Study | 1613 | ||
| Macular hole trials | 1613 | ||
| Retinitis pigmentosa | 1613 | ||
| 1 Surgical Retina | 1615 | ||
| Section 1: The Pathophysiology of Retinal Detachment and Associated Problems | 1616 | ||
| 95 Pathogenetic Mechanisms of Retinal Detachment | 1616 | ||
| Major types | 1616 | ||
| Rhegmatogenous retinal detachment | 1616 | ||
| Traction retinal detachment | 1618 | ||
| Combined tractional and rhegmatogenous retinal detachment | 1618 | ||
| Exudative and hemorrhagic retinal detachment | 1619 | ||
| Conclusion | 1620 | ||
| References | 1620 | ||
| 96 Nonrhegmatogenous Retinal Detachment | 1622 | ||
| Introduction | 1622 | ||
| Pathophysiology | 1622 | ||
| Idiopathic | 1622 | ||
| Central serous chorioretinopathy | 1622 | ||
| Bullous retinal detachment | 1622 | ||
| Chronic CSCR | 1624 | ||
| Uveal effusion syndrome | 1624 | ||
| Vascular | 1625 | ||
| Coats disease | 1625 | ||
| Accelerated hypertension and pregnancy-induced hypertension | 1626 | ||
| Pregnancy-induced hypertension | 1626 | ||
| Diabetic retinopathy | 1627 | ||
| Vascular occlusive diseases | 1627 | ||
| Collagen vascular diseases | 1628 | ||
| Inflammatory and infectious | 1628 | ||
| Vogt–Koyanagi–Harada syndrome | 1628 | ||
| Sympathetic ophthalmia (SO) | 1629 | ||
| Posterior scleritis | 1629 | ||
| Infections associated with exudative detachment | 1630 | ||
| Bacterial infection | 1630 | ||
| Fungal infection | 1630 | ||
| Viral infection | 1630 | ||
| Degenerative | 1630 | ||
| Age-related macular degeneration and polypoidal choroidal vasculopathy | 1630 | ||
| Tumor and malignancy | 1631 | ||
| Choroidal hemangioma | 1631 | ||
| Choroidal melanoma | 1632 | ||
| Metastatic tumors | 1633 | ||
| Lymphoma | 1634 | ||
| Leukemia | 1634 | ||
| Disc anomalies | 1635 | ||
| Optic nerve pit | 1635 | ||
| Morning glory syndrome | 1636 | ||
| Other conditions | 1637 | ||
| Postsurgical exudative retinal detachment | 1637 | ||
| Disseminated intravascular coagulopathy | 1637 | ||
| Post-organ transplantation or hemodialysis exudative detachment | 1637 | ||
| Miscellaneous | 1637 | ||
| Conclusion | 1638 | ||
| References | 1638 | ||
| 97 Pathogenesis of Proliferative Vitreoretinopathy | 1640 | ||
| Introduction | 1640 | ||
| Cellular basis of PVR | 1641 | ||
| Composition of membranes | 1641 | ||
| RPE cells | 1641 | ||
| Glial cells | 1641 | ||
| Blood-borne cells | 1641 | ||
| Stimulation of cellular proliferation and migration | 1642 | ||
| Blood components | 1642 | ||
| Platelet-derived growth factor (PDGF) | 1642 | ||
| Transforming growth factor-β | 1642 | ||
| Monocyte chemotactic protein-1 (MCP1) | 1642 | ||
| Basic fibroblast growth factor (bFGF) | 1642 | ||
| Hepatocyte growth factor (HGF) | 1642 | ||
| Connective tissue growth factor (CTGF) | 1643 | ||
| Epidermal growth factor (EGF) | 1643 | ||
| Vascular endothelial growth factor (VEGF) | 1643 | ||
| Cytokines | 1643 | ||
| Extracellular matrix remodeling | 1643 | ||
| Biomarkers | 1644 | ||
| Conclusion | 1644 | ||
| References | 1645 | ||
| 98 Pathophysiology of Ocular Trauma | 1647 | ||
| Introduction | 1647 | ||
| Anatomic change | 1647 | ||
| Histopathologic findings | 1648 | ||
| Experimental models | 1648 | ||
| Wound healing and traumatic proliferative vitreoretinopathy | 1649 | ||
| Cellular constituents | 1649 | ||
| Growth factors | 1650 | ||
| Extracellular matrix | 1651 | ||
| Special conditions | 1652 | ||
| Traumatic endophthalmitis | 1652 | ||
| Intraocular foreign body | 1652 | ||
| Combat ocular injury | 1652 | ||
| Therapeutic aspects | 1652 | ||
| Surgical approach | 1652 | ||
| Pharmacologic approach | 1652 | ||
| Conclusion | 1653 | ||
| References | 1653 | ||
| Section 2: Retinal Reattachment: General Surgical Principles and Techniques | 1656 | ||
| 99 The Effects and Action of Scleral Buckles in the Treatment of Retinal Detachment | 1656 | ||
| Effects of scleral buckles on the geometry of the eye | 1656 | ||
| Axial length changes after scleral buckles | 1656 | ||
| Refractive errors caused by scleral buckles | 1657 | ||
| Astigmatic errors | 1657 | ||
| Spherical equivalent errors | 1658 | ||
| Scleral chord versus scleral arc length | 1658 | ||
| Effects on the internal geometry of the eye | 1659 | ||
| Volume changes in the eye after scleral buckles | 1659 | ||
| Scleral buckles and ocular rigidity | 1660 | ||
| Scleral buckles and ocular blood flow | 1661 | ||
| Effects of scleral buckles on the RPE and retina | 1661 | ||
| An overview of forces acting on the retina | 1661 | ||
| Forces that lead to retinal tears and detachments | 1661 | ||
| Vitreous traction | 1662 | ||
| Fluid movement and retinal breaks | 1662 | ||
| Epiretinal membranes, cellular proliferation, and retinal breaks | 1663 | ||
| Forces that promote attachment of the retina | 1663 | ||
| Physiologic adhesion between retina and RPE | 1664 | ||
| Thermal chorioretinal adhesions | 1664 | ||
| Scleral buckles and vitreous traction | 1664 | ||
| Scleral buckles and traction on the retinal surface | 1665 | ||
| Scleral buckles and fluid movement | 1666 | ||
| Conclusion | 1667 | ||
| References | 1667 | ||
| 100 Techniques of Scleral Buckling | 1669 | ||
| Introduction | 1669 | ||
| Surgical anatomy | 1669 | ||
| Coats of the eye | 1669 | ||
| Extraocular muscles | 1669 | ||
| Choroidal vasculature | 1670 | ||
| Innervation | 1670 | ||
| Preoperative assessment | 1670 | ||
| Finding the retinal break | 1670 | ||
| Lincoff’s rules | 1671 | ||
| Scheduling surgery | 1671 | ||
| Preparation for surgery | 1672 | ||
| Anesthesia | 1672 | ||
| Positioning the head for surgery | 1672 | ||
| Preparation and draping | 1672 | ||
| Surgical steps | 1672 | ||
| Conjunctival peritomy | 1672 | ||
| Slinging rectus muscles | 1672 | ||
| Reoperations | 1672 | ||
| Examination under anesthesia and break localization | 1674 | ||
| Retinopexy | 1674 | ||
| Cryotherapy | 1675 | ||
| Diode laser | 1676 | ||
| Photocoagulation | 1676 | ||
| Choice of retinopexy technique | 1676 | ||
| Choice of scleral explant | 1677 | ||
| Scleral sutures | 1680 | ||
| Tying the sutures | 1682 | ||
| Subretinal fluid drainage | 1683 | ||
| Indications for drainage | 1683 | ||
| Technique of drainage | 1684 | ||
| Timing | 1684 | ||
| Location of drain sites | 1684 | ||
| Drainage techniques | 1684 | ||
| Cut down techniques | 1684 | ||
| Single-stage techniques | 1685 | ||
| Comparison of techniques | 1685 | ||
| After drainage | 1685 | ||
| Air injection | 1686 | ||
| Encirclement | 1686 | ||
| Final examination of the retina | 1687 | ||
| Closure | 1687 | ||
| Documentation | 1687 | ||
| Outcomes | 1688 | ||
| Postoperative complications | 1689 | ||
| Recurrent retinal detachment | 1689 | ||
| Glaucoma | 1691 | ||
| Epiretinal membranes | 1691 | ||
| Extrusion/infection | 1691 | ||
| Band migration | 1693 | ||
| Diplopia | 1693 | ||
| Anterior segment ischemia | 1693 | ||
| References | 1694 | ||
| 101 Principles and Techniques of Vitreoretinal Surgery | 1696 | ||
| Vitreoretinal surgical anatomy | 1696 | ||
| Mechanics of vitreoretinal surgery | 1696 | ||
| Peeling | 1696 | ||
| Shear | 1697 | ||
| Fatigue failure | 1697 | ||
| Infusion system management | 1697 | ||
| Vitreous cutter considerations | 1699 | ||
| Control systems | 1699 | ||
| Microscope requirements | 1699 | ||
| Tool ergonomics | 1699 | ||
| Surgical steps | 1699 | ||
| Transconjunctival, small gauge vitrectomy | 1699 | ||
| Sclerotomies | 1700 | ||
| Vitreous removal | 1701 | ||
| Lens management | 1702 | ||
| Epiretinal membrane management | 1704 | ||
| Management of subretinal proliferation | 1705 | ||
| Extrusion techniques | 1705 | ||
| Interfacial surface tension management | 1706 | ||
| Fluid–air exchange | 1707 | ||
| Air–gas exchange | 1707 | ||
| Liquid perfluorocarbon | 1707 | ||
| Air–silicone exchange | 1708 | ||
| Perfluorocarbon–silicone oil exchange | 1709 | ||
| Retinectomy | 1709 | ||
| Hemostasis | 1709 | ||
| Retinopexy | 1710 | ||
| Panretinal photocoagulation | 1710 | ||
| Subconjunctival pharmacotherapeutics | 1710 | ||
| Surgical algorithms | 1710 | ||
| Conclusion | 1710 | ||
| References | 1710 | ||
| 102 Primary Vitrectomy in Rhegmatogenous Retinal Detachment | 1712 | ||
| Introduction | 1712 | ||
| Pathogenesis of Rhegmatogenous Retinal Detachment | 1712 | ||
| Categories of Rhegmatogenous Retinal Detachment | 1712 | ||
| Patient selection for primary vitrectomy | 1713 | ||
| Principles of vitrectomy | 1713 | ||
| Surgical techniques | 1713 | ||
| Create three ports through the pars plana | 1713 | ||
| Core vitrectomy | 1713 | ||
| Peripheral vitrectomy | 1714 | ||
| Fluid–air exchange | 1715 | ||
| Photocoagulation/cryopexy of the retinal tear | 1715 | ||
| Tamponade | 1715 | ||
| Positioning after surgery | 1715 | ||
| Vitrectomy with encircling buckling | 1715 | ||
| Vitrectomy with phacoemulsification and intraocular lens implantation | 1715 | ||
| Sutureless microincision vitrectomy surgery | 1715 | ||
| Surgical outcomes | 1717 | ||
| Prognostic factors | 1719 | ||
| Complications | 1719 | ||
| Perspectives | 1719 | ||
| References | 1719 | ||
| 103 Pneumatic Retinopexy | 1721 | ||
| Introduction | 1721 | ||
| History | 1721 | ||
| Basic principles | 1721 | ||
| Intraocular gases | 1721 | ||
| Retina–gas interface | 1722 | ||
| Case selection | 1722 | ||
| Extent of breaks | 1722 | ||
| Inferior breaks | 1722 | ||
| Proliferative vitreoretinopathy | 1722 | ||
| Inability to maintain positioning | 1722 | ||
| Glaucoma | 1723 | ||
| Cloudy media | 1723 | ||
| Lattice degeneration | 1723 | ||
| Aphakia and pseudophakia | 1723 | ||
| Posterior vitreous detachment | 1723 | ||
| Cases where pneumatic retinopexy presents a particular advantage | 1723 | ||
| Surgical technique | 1723 | ||
| Anesthesia | 1723 | ||
| One-session versus two-session procedure | 1723 | ||
| Cryopexy versus laser | 1724 | ||
| Applying retinopexy | 1724 | ||
| Amount and type of gas to inject | 1724 | ||
| Sterilization of the ocular surface | 1725 | ||
| Preparation of the gas | 1725 | ||
| Performing a paracentesis | 1725 | ||
| Injection of gas | 1726 | ||
| Assessing intraocular pressure | 1727 | ||
| Instructing the patient | 1727 | ||
| Special procedures | 1727 | ||
| Fish eggs | 1727 | ||
| Gas entrapment at the injection site | 1728 | ||
| Steamroller | 1728 | ||
| Summary of procedure | 1728 | ||
| Postoperative management | 1729 | ||
| Results | 1730 | ||
| Comparison of pneumatic retinopexy with scleral buckle | 1730 | ||
| Comparison of pneumatic retinopexy with vitrectomy | 1730 | ||
| Complications | 1731 | ||
| Proliferative vitreoretinopathy | 1731 | ||
| New or missed retinal breaks | 1731 | ||
| Subretinal gas | 1731 | ||
| Acceptance of Pneumatic Retinopexy | 1731 | ||
| Algorithm for choosing Pneumatic Retinopexy versus other procedures for retinal detachment | 1732 | ||
| Conclusion | 1732 | ||
| References | 1733 | ||
| 104 Special Adjuncts to Treatment | 1735 | ||
| Special adjunct to treatment | 1735 | ||
| Introduction | 1735 | ||
| Physical properties of intraocular gases | 1735 | ||
| Functions of gas | 1738 | ||
| Internal tamponade | 1738 | ||
| Unfolding and folding of the retina | 1738 | ||
| Postoperative visualization | 1738 | ||
| Replace globe volume | 1738 | ||
| Dynamics of the gas bubble inside the eye | 1739 | ||
| Different phases of gas resorption | 1739 | ||
| Special considerations when under general anesthesia | 1740 | ||
| Response to changes in altitude | 1740 | ||
| Preparation for injection | 1740 | ||
| Clinical applications and surgical techniques | 1740 | ||
| In vitrectomy for retinal detachments | 1741 | ||
| In pneumatic retinopexy | 1742 | ||
| In scleral buckling for retinal detachments | 1742 | ||
| In macular hole surgery | 1743 | ||
| In displacement of subretinal blood | 1744 | ||
| In postvitrectomy gas exchange | 1744 | ||
| Postoperative care | 1744 | ||
| Head posture after intraocular gas injection | 1744 | ||
| Fundal exam in the postoperative period | 1745 | ||
| Intraocular pressure measurements | 1745 | ||
| Laser photocoagulation | 1745 | ||
| Vision change after surgery | 1745 | ||
| Changes in altitude | 1745 | ||
| Complications and management | 1745 | ||
| Cataract formation | 1745 | ||
| Raised intraocular pressure | 1745 | ||
| Hypotony | 1746 | ||
| Subretinal gas | 1746 | ||
| Gas in the anterior chamber and corneal decompensation | 1746 | ||
| Intraocular lens capture | 1746 | ||
| Conclusion | 1746 | ||
| Perfluorocarbon liquid in vitreoretinal surgery | 1746 | ||
| Introduction | 1746 | ||
| Types and properties of perfluorocarbon liquid | 1746 | ||
| Technique of perfluorocarbon liquid injection | 1747 | ||
| Technique of perfluorocarbon liquid removal | 1747 | ||
| Indications for use | 1748 | ||
| Proliferative vitreoretinopathy | 1748 | ||
| Vitreous base shaving | 1749 | ||
| Giant tears | 1749 | ||
| Ocular trauma | 1751 | ||
| Dislocated lens | 1752 | ||
| Suprachoroidal hemorrhage | 1752 | ||
| Other indications | 1753 | ||
| Complications and management | 1753 | ||
| Subretinal PFCL | 1753 | ||
| Intraocular toxicity | 1754 | ||
| PFCL in the anterior chamber | 1754 | ||
| Conclusion | 1754 | ||
| Silicone oil in vitreoretinal surgery | 1755 | ||
| Introduction | 1755 | ||
| Background | 1755 | ||
| Chemical properties of silicone oil | 1755 | ||
| Physical properties of silicone oil | 1756 | ||
| Specific gravity | 1756 | ||
| Buoyancy | 1756 | ||
| Surface tension and interfacial tension | 1756 | ||
| Viscosity | 1757 | ||
| Indications | 1758 | ||
| Retinal detachments with proliferative vitreoretinopathy | 1758 | ||
| Giant retinal tears | 1758 | ||
| Severe proliferative diabetic retinopathy | 1758 | ||
| Macular hole | 1759 | ||
| Viral retinitis | 1759 | ||
| Complicated pediatric retinal detachments | 1760 | ||
| Retinal detachments associated with choroidal coloboma | 1760 | ||
| Trauma | 1760 | ||
| Endophthalmitis | 1760 | ||
| Surgical techniques of silicone oil infusion | 1760 | ||
| General considerations | 1760 | ||
| Considerations of lens status | 1761 | ||
| Silicone oil infusion in small gauge vitrectomy systems | 1761 | ||
| Air–silicone oil exchange | 1761 | ||
| Perfluorocarbon liquid–silicone oil exchange | 1762 | ||
| Complications | 1763 | ||
| Silicone oil in the anterior chamber | 1763 | ||
| Glaucoma | 1763 | ||
| Chronic hypotony | 1765 | ||
| Cataract formation | 1766 | ||
| Recurrent retinal detachment | 1767 | ||
| Emulsification | 1767 | ||
| Keratopathy | 1768 | ||
| Unexplained visual loss following silicone oil tamponade | 1768 | ||
| Silicone oil removal | 1768 | ||
| Permanent silicone oil tamponade | 1769 | ||
| Conclusion | 1769 | ||
| The concept of heavy tamponade | 1769 | ||
| Background | 1769 | ||
| Newer generation of heavy tamponades | 1770 | ||
| Technique | 1771 | ||
| Complications | 1772 | ||
| Corneal toxicity | 1772 | ||
| Cataract formation | 1773 | ||
| Intraocular inflammation | 1773 | ||
| Emulsification | 1773 | ||
| Sticky silicone oil | 1773 | ||
| Adherent silicone oil on intraocular lens | 1774 | ||
| Hypotony | 1774 | ||
| Raised intraocular pressure | 1774 | ||
| Redetachment and proliferative vitreoretinopathy | 1774 | ||
| Conclusion | 1774 | ||
| Drugs for the prevention of proliferative vitreoretinopathy | 1775 | ||
| Introduction | 1775 | ||
| Pharmacological agents that have been tested in clinical trials | 1775 | ||
| Corticosteroids | 1775 | ||
| Fluoropyrimidines | 1775 | ||
| Daunorubicin | 1776 | ||
| Retinoids | 1777 | ||
| Heparin and low-molecular-weight heparin | 1777 | ||
| Chemicals that are yet to be tested in clinical trials | 1777 | ||
| Summary | 1778 | ||
| References | 1778 | ||
| 105 Optimal Procedures for Retinal Detachment Repair | 1784 | ||
| 2 Tumors of the Retina, Choroid, and Vitreous | 2103 | ||
| Section 1: Tumors of the Retina | 2104 | ||
| 128 Retinoblastoma | 2104 | ||
| Introduction | 2104 | ||
| Clinical advances | 2104 | ||
| Basic science advances | 2104 | ||
| Genetics of retinoblastoma | 2104 | ||
| Clinical genetics | 2104 | ||
| Genetic terminology | 2105 | ||
| Molecular genetics of retinoblastoma | 2105 | ||
| The RB1 gene | 2106 | ||
| Low penetrance retinoblastoma | 2107 | ||
| RB1 gene mutations in other tumors | 2107 | ||
| The role of the retinoblastoma protein in tumor suppression | 2108 | ||
| The retinoblastoma protein | 2108 | ||
| The RB1 tumor suppressor pathway | 2109 | ||
| The RB-E2F regulatory network | 2110 | ||
| Molecular pathogenesis of retinoblastoma | 2110 | ||
| Retinoblastoma: the disease | 2111 | ||
| Terminology | 2111 | ||
| Overview of retinoblastoma | 2111 | ||
| Epidemiology | 2111 | ||
| Natural history of intraocular retinoblastoma | 2112 | ||
| Classification of intraocular retinoblastoma | 2114 | ||
| Reese–Ellsworth classification | 2114 | ||
| International Classification for Intraocular Retinoblastoma | 2114 | ||
| Disease prognosis | 2116 | ||
| Retinoblastoma survival rates | 2116 | ||
| Factors affecting survival | 2116 | ||
| Factors affecting salvage of eye and vision | 2117 | ||
| Diagnosis of retinoblastoma | 2117 | ||
| Signs and symptoms | 2117 | ||
| Diseases simulating retinoblastoma (pseudoretinoblastoma) | 2118 | ||
| Meeting the family and making a diagnosis | 2118 | ||
| Diagnostic workup | 2120 | ||
| Metastatic workup | 2120 | ||
| Staging examination under anesthesia | 2120 | ||
| The approach to the child with cancer | 2122 | ||
| Treat the child, not only the eye | 2122 | ||
| Treatment methods and techniques | 2123 | ||
| Developing a customized treatment plan | 2123 | ||
| Unilateral nonheritable retinoblastoma | 2123 | ||
| Bilateral retinoblastoma: symmetrical disease | 2123 | ||
| Advanced intraocular disease (groups D and E) | 2123 | ||
| Systemic intravenous chemotherapy | 2123 | ||
| Terminology | 2123 | ||
| Pre-1989 chemotherapy for extraocular disease | 2124 | ||
| Background of the currently used chemotherapy regimen | 2124 | ||
| Primary systemic chemotherapy | 2124 | ||
| Subtenon carboplatin | 2124 | ||
| Complications of primary systemic chemotherapy | 2125 | ||
| Heat and chemotherapy | 2125 | ||
| Intra-arterial (IA) chemotherapy | 2126 | ||
| Focal consolidation | 2126 | ||
| Laser | 2126 | ||
| Cryotherapy | 2128 | ||
| Radiation therapy | 2128 | ||
| External beam radiotherapy (teletherapy) | 2128 | ||
| Intensity modulated radiation therapy | 2129 | ||
| Proton beam radiotherapy | 2130 | ||
| Brachytherapy | 2130 | ||
| Enucleation | 2131 | ||
| Preoperative preparation for enucleation | 2132 | ||
| Surgical technique | 2132 | ||
| Tumor harvesting | 2134 | ||
| Surgical closure | 2134 | ||
| Postoperative care following enucleation | 2134 | ||
| Retrolaminar optic nerve involvement | 2135 | ||
| Post-treatment assessment and follow-up | 2135 | ||
| Regression patterns following treatment | 2135 | ||
| New tumors or tumor recurrences during post-treatment follow-up | 2136 | ||
| Late effects of treatment | 2136 | ||
| Bony hypoplasia of the midface (orbit) | 2137 | ||
| Radiation cataract | 2137 | ||
| Radiation retinopathy | 2137 | ||
| Neurocognitive deficits | 2138 | ||
| Second malignant neoplasms | 2138 | ||
| Visual acuity outcome | 2138 | ||
| Metastatic retinoblastoma | 2139 | ||
| Risk factors | 2139 | ||
| Survival | 2139 | ||
| Metastatic workup | 2139 | ||
| Extraocular (orbital) retinoblastoma | 2139 | ||
| Multicenter trials: Children’s Oncology Group | 2140 | ||
| Atypical retinoblastoma | 2140 | ||
| Retinoma (retinocytoma) | 2140 | ||
| Diffuse infiltrating retinoblastoma | 2141 | ||
| Retinoblastoma in older children | 2142 | ||
| Iatrogenic extraocular extension of tumor | 2142 | ||
| Trilateral retinoblastoma (primitive neural ectodermal tumors, pinealoma) | 2143 | ||
| References | 2144 | ||
| 129 Cavernous Hemangioma | 2150 | ||
| Introduction | 2150 | ||
| Clinical findings | 2150 | ||
| Differential diagnosis | 2150 | ||
| Ancillary studies | 2151 | ||
| Natural history | 2151 | ||
| Treatment | 2151 | ||
| Pathology | 2152 | ||
| Systemic and familial involvement | 2153 | ||
| Genetics | 2154 | ||
| Salient features | 2154 | ||
| References | 2154 | ||
| 130 Capillary Hemangioblastoma of the Retina and von Hippel–Lindau Disease | 2156 | ||
| Introduction | 2156 | ||
| Von hippel–lindau disease | 2156 | ||
| History | 2156 | ||
| Genetic associations | 2156 | ||
| Clinical presentation | 2156 | ||
| Clinical diagnosis | 2157 | ||
| Epidemiology of ocular lesions of von hippel–lindau disease | 2158 | ||
| Causes of vision loss | 2159 | ||
| Pathology of ocular lesions | 2159 | ||
| Treatment | 2160 | ||
| Anti-angiogenic treatments | 2160 | ||
| Screening and genetic testing for von hippel–lindau disease | 2161 | ||
| Conclusion | 2161 | ||
| References | 2161 | ||
| 131 Tuberous Sclerosis and the Eye | 2164 | ||
| Introduction | 2164 | ||
| History, diagnosis, and genetic basis | 2164 | ||
| Systemic manifestations | 2164 | ||
| Neurological | 2164 | ||
| Seizures | 2164 | ||
| Cognitive and behavioral disability | 2164 | ||
| Skin features | 2165 | ||
| Visceral features | 2166 | ||
| Skeletal features | 2166 | ||
| Ocular manifestations | 2166 | ||
| Retinal manifestations | 2166 | ||
| Optic nerve phakomas | 2167 | ||
| Ocular adnexal lesions | 2167 | ||
| Differential diagnosis | 2167 | ||
| Genetics | 2168 | ||
| References | 2169 | ||
| 132 Phakomatoses | 2170 | ||
| Introduction | 2170 | ||
| Definition of hamartia, hamartoma, chorista, choristoma | 2170 | ||
| Neurofibromatosis (von recklinghausen syndrome) | 2170 | ||
| Neurofibromatosis type 1 | 2171 | ||
| General considerations | 2171 | ||
| Ophthalmologic features | 2171 | ||
| Dermatologic features | 2171 | ||
| Central nervous system features | 2172 | ||
| Other features | 2173 | ||
| Management | 2173 | ||
| Neurofibromatosis type 2 | 2173 | ||
| General considerations | 2173 | ||
| Ophthalmologic features | 2173 | ||
| Dermatologic features | 2173 | ||
| Central nervous system features | 2173 | ||
| Other features | 2174 | ||
| Management | 2174 | ||
| Encephalofacial hemangiomatosis (sturge–weber syndrome) | 2174 | ||
| General considerations | 2174 | ||
| Ophthalmologic features | 2174 | ||
| Dermatologic features | 2175 | ||
| Central nervous system features | 2175 | ||
| Other features | 2176 | ||
| Management | 2176 | ||
| Racemose hemangiomatosis (wyburn-mason syndrome) | 2176 | ||
| General considerations | 2176 | ||
| Ophthalmologic features | 2176 | ||
| Dermatologic features | 2177 | ||
| Central nervous system features | 2177 | ||
| Other features | 2177 | ||
| Management | 2177 | ||
| Retinal cavernous hemangiomatosis | 2177 | ||
| General considerations | 2177 | ||
| Ophthalmologic features | 2177 | ||
| Index | i1 | ||
| A | i1 | ||
| B | i3 | ||
| C | i4 | ||
| D | i8 | ||
| E | i9 | ||
| F | i11 | ||
| G | i12 | ||
| H | i13 | ||
| I | i14 | ||
| J | i15 | ||
| K | i15 | ||
| L | i15 | ||
| M | i16 | ||
| N | i18 | ||
| O | i19 | ||
| P | i20 | ||
| Q | i23 | ||
| R | i23 | ||
| S | i27 | ||
| T | i30 | ||
| U | i31 | ||
| V | i32 | ||
| W | i34 | ||
| X | i34 | ||
| Z | i34 | ||
| Complications in Vitreoretinal Surgery | e1 | ||
| Always Measure Prior to Trochar Insertion | e1 | ||
| Suprachoroidal Infusion | e1 | ||
| Subretinal Insertion of Endo-illuminator | e1 | ||
| Dislocated IOL and Capsular Tension Ring | e2 | ||
| Iatrogenic Breaks during the Induction of Posterior Vitreous Detachment | e2 | ||
| Iatrogenic Macular Hole during VMT Surgery | e2 | ||
| Iatrogenic Breaks during the Delamination of Diabetic Traction Retinal Detachment | e2 | ||
| Point Pressure Hemostasis during Diabetic Vitrectomy | e2 | ||
| Iatrogenic Retinal Break during ERM Peeling | e3 | ||
| Subretinal Brilliant Blue | e3 | ||
| Peripheral Retinal Detachment during Macular Hole Surgery | e3 | ||
| Subretinal Hemorrhage | e3 | ||
| Macular Fold | e3 | ||
| Subretinal Perfluorocarbon | e4 | ||
| Subretinal Perfluorocarbon Injection during En Bloc Perfluorodissection | e4 | ||
| Intraocular Foreign Body Dislodged on the Macula | e4 | ||
| Suprachoroidal Hemorrhage | e4 | ||
| Hemorrhagic Choroidal Detachment after “One Stitch” Vitrectomy Surgery | e4 | ||
| Dislocated Phakic IOL | e5 | ||
| Dislocation of the Tip of the Soft Tip Cannula | e5 | ||
| Iatrogenic Peripheral Retinal Breaks during IOFB Extraction | e5 | ||
| Peri-silicone Proliferation | e5 | ||
| Miragel Buckle Removal | e5 | ||
| Subconjunctival Silicone Oil Removal | e6 |