CORNEA, 2-VOLUME SET.

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Front Cover Front Matter Copyright Contents Video Table of Contents Preface Acknowledgments Dedication Acknowledgments to the Founding Editors List of Contributors PART I Basic Science: Cornea, Sclera, Ocular Adnexa Anatomy, Physiology, and Pathophysiologic Responses 1 - Cornea and Sclera: Anatomy and Physiology INTRODUCTION Anatomy and Physiology Structure of the Cornea and Sclera Dimensions and Optical Properties of the Cornea Corneal Epithelium Limbal Stem Cells and Lineage of Corneal Epithelial Cells Layered Structure of the Corneal Epithelium Physiology of the Corneal Epithelium Stroma of the Cornea and Sclera Bowman Layer Structure of the Stroma Physiology of the Stroma Endothelium Descemet Membrane Endothelial Cells Physiology of the Endothelium Innervation Vascular System Oxygen and Nutrient Supply Development of the Anterior Eye Segment References 2 - The Conjunctiva: Anatomy and Physiology EMBRYOLOGY Anatomy Histology Mucocutaneous Junction Palpebral and Forniceal Conjunctiva Bulbar Conjunctiva The Corneoscleral Limbus Conjunctival Goblet Cells Substantia Propria Vascular Supply Lymphatic Drainage Nerve Supply Normal Flora Physiology of the Conjunctiva References 3 - Tear Film MACROSTRUCTURE AND FUNCTION Mucoaqueous Component Lipid Component Evaluation of the Tear Film Optical Coherence Tomography Tear Film Osmolarity Tear Film Break-up Time Tear Production Measurement Microstructure Lipidome Mucins Proteome Tear Production References 4 - Eyelids and the Corneal Surface Introduction Anatomy Eyelid Skin Eyelid Muscles: Protractors Orbicularis Oculi Eyelid Muscles: Retractors Upper Lid Retractor: Levator Palpebrae Superioris Upper Lid Retractor: Müller Muscle Upper Lid Retractor: Frontalis Lower Lid Retractors Orbital Septum Orbital Fat Tarsus Meibomian Glands Conjunctiva Lacrimal Glands Eyelid Margin Canthal Tendons Lacrimal Drainage System Vascular Supply Lymphatic Drainage Nerves Blink Reflex and Tear Flow REFERENCES 5 - A Matrix of Pathologic Responses in the Cornea ANATOMIC REGIONS OF THE CORNEA GENERAL PATHOLOGIC RESPONSES OF THE CORNEA 1. Defects and Their Repair 2. Fibrosis and Vascularization 3. Edema and Cysts 4. Inflammation and Immune Responses 5. Deposits 6. Proliferation Specific Pathologic Responses of the Cornea 1. Defects and Their Repair 2. Fibrosis and Vascularization 3. Edema and Cysts Epithelial Edema 4. Inflammation and Immune Responses 5. Deposits Elements Drugs5 Corneal Dystrophies and Degenerations 6. Proliferation 1. Defects and Their Repair 2. Fibrosis and Vascularization Subepithelial Avascular Fibrosis Subepithelial Vascular Fibrosis 3. Edema and Cysts 4. Inflammation and Immune Responses 5. Deposits 6. Proliferation of the Epithelial Basement Membrane Pathologic Responses of the Corneal Stroma 1. Defects and Their Repair 2. Fibrosis and Vascularization Stromal Fibrosis Stromal Vascularization 3. Edema and Cysts 4. Inflammation and Immune Responses 5. Deposits Topical and Systemic Drugs Ocular Diseases Systemic Diseases Deposits from Dystrophies and Degenerations of the Stroma 6. Proliferation 1. Defects (and Their Repair) Defects in the Endothelium Defects in Descemet Membrane 2. Fibrosis and Vascularization Posterior to Descemet Membrane Posterior Collagenous Layer Using the Posterior Collagenous Layer to Date the Onset of Endothelial or Descemet Membrane Disease with Transmission Electron M... 3. Edema and Cysts 4. Inflammation and Immune Responses 5. Deposits Topical and Systemic Drugs Ocular and Systemic Diseases Corneal Dystrophies and Degenerations 6. Proliferation Overview Cells of Inflammation and the Immune Response Cells of the Lymphoid System B Lymphocytes T Lymphocytes Null Lymphocytes Cells of the Myeloid System Macrophages and the Mononuclear Phagocytic System Dendritic Cells Langerhans Cells Other Cells of the Myeloid System Polymorphonuclear Leukocytes Eosinophils Basophils Mast Cells Adhesion Molecules Cytokines Chemokines Complement Mucosa-Associated Immune System Lacrimal Functional Unit Cell-Mediated Immune Response Major Histocompatibility Complex40 Antigen Presentation and T-Cell Activation40 Cell-Mediated Immune Response Humoral (Antibody-Mediated) Immune Response Immunoglobulins Characteristics of Immunoglobulins40 Anterior Chamber–Associated Immune Deviation Immune Hypersensitivity Reactions Type I Hypersensitivity Response (Atopic, Allergic Reactions) Type II (Cytotoxic) Hypersensitivity Response Type III Hypersensitivity Response (Immune Complex) Type IV (Delayed-Type Hypersensitivity) Response References PART II Examining and Imaging the Cornea and External Eye SECTION 1 Basic Evaluation of the Cornea and External Eye 6 - Examination of the Lids General Principles History of the Patient Dermatologic Examination Eyelid Position Tear Meniscus and Puncta Anterior Eyelid Posterior Eyelid Meibomian Gland Expression Mucocutaneous Junction Meibomian Gland Imaging References 7 - Slit Lamp Examination and Photography SECTION I: EXAMINATION The Instrument Forms of Illumination Direct Illumination Diffuse Illumination Indirect Illumination Proximal Illumination Sclerotic Scatter Direct and Indirect Retroillumination from the Iris Retroillumination from the fundus Transillumination of the Iris The Peripheral Cornea (Gonioscopy) Vital Dyes Section II: Photography The Instrument Preparing for Photography Focus The Photographic Format Centration Control of Artifacts The Final Image Color Balance and Sensitivity . Subject reflectivity is an element of great influence. In recording conditions by direct illumination, levels of subject refle... . When an electronic flash is used, the duration of exposure is dictated by the 1-ms duration of the flash. The intensity and sh... Direct Illumination Diffuse Illumination Broad-beam Illumination Optical Section Combined Direct Focal and Diffused Illumination Tyndall Light/Anterior Chamber Cells and Flare Specular Reflection Indirect Illumination Proximal Illumination Sclerotic Scatter Direct Retroillumination from the Iris Indirect Retroillumination from the Iris Retroillumination from the Fundus Transillumination of the Iris The Peripheral Cornea Vital Dyes . To produce photographs of this technique, the blue filter is placed over the light source, which is set to produce full broad-... Techniques Specific to Keratoconus . The Fleischer ring may be documented by using the blue filter described earlier for exciting fluorescein. In this case, the bl... . This sign is a simple, graphic means of demonstrating the abnormal corneal outline. By asking the patient to look down, the li... Further Reading References 8 - Tear Film and Corneal Diagnostic Techniques INTRODUCTION Dry Eye Questionnaires General Inspection Slit Lamp Examination Tear Stability Corneal Staining Tear Production and Volume Meibomian Gland Health Tear Clearance Tests Point-of-Care Tests Tear Osmolarity Inflammatory Biomarkers Lactoferrin Devices Used in the Assessment of Dry Eye Disease Tests of Visual Function Corneal Imaging Aesthesiometry Confocal Microscopy Conclusion References SECTION 2 Laboratory Investigations 9 - Practical Ophthalmic Microbiology for the Detection of Corneal Pathogens Introduction Corneal Specimen Collection Transport Media Mailing of Diagnostic Specimens Stains and Cytology Specimens Bacterial Laboratory Diagnosis Mycobacteria Nocardia and Actinomyces Antibiotic Susceptibility Testing Laboratory Diagnosis of Fungal Infection Laboratory Testing for Chlamydia Laboratory Diagnosis: Unusual Requests Summary References 10 - Molecular Genetics of Corneal Disease REVIEW OF GENETICS AND HUMAN DISEASE Techniques Used to Identify Disease-Causing Genes Disease-Causing Mutations Versus Non–Disease-Causing Sequence Variations Terminology Epithelial Basement Membrane Dystrophy Epithelial Recurrent Erosion Dystrophies Subepithelial Mucinous Corneal Dystrophy Meesmann Corneal Dystrophy Lisch Epithelial Corneal Dystrophy Gelatinous Drop-Like Corneal Dystrophy (GDLD) Epithelial-Stromal Tgfbi Dystrophies TGFBI Dystrophies Reis-Bücklers Corneal Dystrophy Thiel-Behnke Corneal Dystrophy Lattice Corneal Dystrophy Type 1 Granular Corneal Dystrophy Type 1 Granular Corneal Dystrophy Type 2 Stromal Dystrophies Macular Corneal Dystrophy Schnyder Corneal Dystrophy Congenital Stromal Corneal Dystrophy Fleck Corneal Dystrophy Posterior Amorphous Corneal Dystrophy Central Cloudy Dystrophy of François Pre-Descemet Corneal Dystrophy Endothelial Dystrophies Fuchs Dystrophy Posterior Polymorphous Corneal Dystrophy Congenital Hereditary Endothelial Dystrophy X-Linked Endothelial Corneal Dystrophy Other Conditions Keratoconus X-Linked Megalocornea Cornea Plana EDICT Syndrome Familial Amyloidosis, Gelosin Type Conclusion References SECTION 3 Imaging Techniques of the Cornea 11 - Keratometry and Topography Keratometry Corneal Topography Placido Disk-based Topography Grid-Style Reflection Topography Slit Scanning Tomography Scheimpflug Tomography Optical Coherence Tomography Topographic Maps Axial Curvature Map Instantaneous or Tangential Curvature Map Refractive Power Map Elevation Map Pachymetric Map Difference Map Quantitative Indices Screening With Topographic and Tomographic Indices Applications Cataract Surgery Refractive Surgery Corneal Surgery Conclusion References 12 - Corneal Shape Analysis and Biomechanical Assessment Scheimpflug Principle Three-Dimensional Image—Cornea and Lens Densitometry (Backscatter) Elevation Maps—Enhanced Elevation Cone Location Displaced Apex Syndrome Enhanced Reference Surface Comprehensive Corneal Thickness Evaluation Spatial Variation of Corneal Thickness Segmental Tomography Corneal Biomechanical Assessment Conclusion References 13 - Specular Microscopy Optical Principles of Specular Microscopy Patient Preparation Standardization of Imaging Techniques INSTRUMENTATION Qualitative Specular Microscopy Epithelium Endothelium: Miscellaneous Bright and Dark Structures Endothelium: Morphometry Quantitative Specular Microscopy Specular Microscopy in Clinical Trials and the Value of a Reading Center Clinical Applications for Specular Microscopy Cataract Extraction With Intraocular Lens Implantation Refractive Surgery and Collagen Crosslinking Photorefractive Keratectomy and Laser in Situ Keratomileusis Phakic Intraocular Lenses Intrastromal Devices Collagen Crosslinking Donor Corneas Penetrating Keratoplasty Endothelial Keratoplasty Descemet Stripping Automated Endothelial Keratoplasty Descemet Membrane Endothelial Keratoplasty Summary References 14 - Confocal Microscopy Background Historical Overview Current Confocal Systems in Clinical Use In Vivo Confocal Imaging Normal Corneal Structures Confocal Microscopy Through-Focusing Applications Conclusion References 15 - Clinical Applications of Confocal Microscopy Introduction Normal Exam Infectious Keratitis Acanthamoeba Keratitis Fungal Keratitis Corneal Dystrophies Keratoconus Endothelium Evaluation Corneal Surgery and Wound Healing Corneal Disease Corneal Nerves Dry Eye Corneal/Conjunctival Intraepithelial Neoplasia Systemic Diseases Conclusion Declaration of Interests References 16 - High-Resolution Ultrasound ULTRASOUND BIOMICROSCOPY: HIGH-RESOLUTION ULTRASOUND Examination Techniques With Ultrasound Biomicroscopy Normal Cornea Uveal Melanoma Idiopathic Iris Cyst Malignant Neoplasm of the Conjunctiva Scleral Staphyloma Pigmented Iris Lesion Shallow Anterior Chamber Iris Stromal Cyst Hyphema Low Intraocular Pressure Imaging Through Corneal Opacities Summary References 17 - Anterior Segment Optical Coherence Tomography INTRODUCTION Dewarping of Anterior Segment Optical Coherence Tomography Images Detection and Classification of Keratoconus and Other Corneal Irregularities Refractive Surgery Evaluation LASIK Flap Evaluation Refractive Enhancement LASIK Complications Small-Incision Lenticule Extraction Corneal Power Measurement and Intraocular Lens Power Calculation Corneal Opacities Planning of Phototherapeutic Keratectomy Cornea Transplant Endothelial Keratoplasty Femtosecond Enabled Keratoplasty Refractive Implants Corneal Implants Phakic Intraocular Lenses Ocular Surface and Iris Tumors Ocular Surface Tumor Iris Tumors Other Anterior Segment Optical Coherence Tomography Applications Financial Disclosure References PART III Differential Diagnosis of Selected Problems in Cornea and External Eye Disease 18 - Congenital Corneal Opacities: Diagnosis and Management BACKGROUND HISTORY AND PHYSICAL EXAMINATION SCLEROCORNEA (S TUMPED) TEARS IN THE ENDOTHELIUM AND DESCEMET MEMBRANE (S T UMPED) Congenital Glaucoma Birth Trauma CORNEAL ULCERS AND INFLAMMATION(ST U MPED) Infectious Diseases METABOLIC DISEASES (STU M PED) Mucopolysaccharidosis Gangliosidoses Fabry disease Mucolipidosis Cystinosis Tyrosinemia MISCELLANEOUS SYNDROMES Fetal Alcohol Syndrome POSTERIOR CORNEAL DEFECTS (STUM P ED) Peters Anomaly Posterior Keratoconus Congenital Anterior Staphyloma ENDOTHELIAL DYSTROPHY (STUMP E D) Congenital Hereditary Endothelial Dystrophy Posterior Polymorphous Corneal Dystrophy Congenital Stromal Corneal Dystrophy Other Dystrophies DERMOIDS (STUMPE D) CORNEAL KELOIDS REFERENCES 19 - Peripheral Corneal Disease THE PERIPHERAL CORNEA: SUSCEPTIBILITY AND RESPONSE TO DISEASE Congenital/Developmental/Inherited Disorders of the Peripheral Cornea Inflammatory/Autoimmune Disorders of the Peripheral Cornea Neoplastic Disorders of the Peripheral Cornea Degenerative Disorders of the Peripheral Cornea Infectious Disorders of the Peripheral Cornea References 20 - Evaluation of the Corneal Ulcer History Clinical Exam Testing—Corneal Smears and Cultures Treatment Conclusion References 21 - Evaluation of Recurrent Corneal Erosions Introduction Presentation Pathophysiology Diagnosis Ancillary Diagnostic Testing References 22 - Corneal Deposits Superficial Deposits Pigmented Deposits Cornea Verticillata Striate Melanokeratosis Epithelial Iron Lines Spheroidal Degeneration Adrenochrome Deposition Nonpigmented Deposits Subepithelial Mucinous Corneal Dystrophy Coat’s White Ring Calcific Band Keratopathy Fluoroquinolone Deposits Mucin Balls Refractile/Crystalline Deposits Meesmann Dystrophy Gelatinous Drop-like Dystrophy Tyrosinemia II (Richner−Hanhart Syndrome) Intraepithelial Ointment Gout (Urate) Stromal Deposits Pigmented Deposits Phenothiazines Corneal Blood Staining Bilirubin Siderosis Nonpigmented Deposits Granular Dystrophy Macular Corneal Dystrophy Fleck Corneal Dystrophy Lipid Deposition Mucopolysaccharidoses Refractile/Crystalline Deposits Lattice Dystrophy Schnyder Central Crystalline Dystrophy Bietti Crystalline Dystrophy Immunoglobulin Deposition Cystinosis Deep Stromal Deposits Pigmented Deposits Copper Deposition Associated With Wilson Disease Chalcosis Ocular Chrysiasis Mottled Cyan Opacification of the Posterior Cornea Deep Blue Dot Degeneration Nonpigmented Deposits Cornea Farinata Pre-Descemet Corneal Dystrophy X-linked Ichthyosis Ocular Argyrosis Refractile/Crystalline Deposits Polymorphic Amyloid Degeneration References 23 - The Red Eye HISTORY Identify the Risk Factors Onset and Duration of Disease Laterality Pain Vision Loss Identifying Abnormalities in the Ocular Structures General Appearance Skin Eyelids Periorbital Masses and Globe Position Recognizing Distinctive Ocular Signs Eyelid Margins Tear Film and Tear Volume Discharge Findings of the Palpebral and Tarsal Conjunctiva Findings of the Bulbar Conjunctiva Corneal Findings Additional Testing Constructing a Differential Diagnosis Classification Schemes Conclusion References PART IV Eye Banking 24 - Eye Banking: Structure and Function Eye Banking: Past and Present Tissue Acquisition Donor Screening and Authorization Recovery Tissue Preservation and Storage Tissue Evaluation and Suitability Donor Eligibility Tissue Processing and Distribution Facilities, Equipment, Instruments, and Supplies Quality Assurance Future Considerations References 25 - Medical Standards for Eye Banking Eye Bank Association of America Medical Standards Federal and State Regulations Standards Relating to Safety of Tissue Dystrophies and Degenerations Endophthalmitis and Microbial Keratitis Rabies Hepatitis B Hepatitis C Human Immunodeficiency Virus I and II Other Infectious Agents Prion Disease Malignancies Recalls and Market Withdrawals Primary Graft Failure Mechanical and Optical Integrity Future Challenges and Opportunities References PART V The Ocular Adnexa SECTION 1 Diseases of the Lid: Anatomic Abnormalities 26 - Malposition of the Eyelids ENTROPION Congenital Entropion Involutional Entropion Cicatricial Entropion Acute Spastic Entropion Differential Diagnosis Medical Management Surgical Management Congenital Entropion Involutional Entropion Cicatricial Entropion Ectropion Congenital Ectropion Involutional Ectropion Cicatricial Ectropion Paralytic Ectropion Mechanical Ectropion Inflammatory Ectropion Medical Management Surgical Management Involutional Ectropion Congenital Ectropion Paralytic Ectropion Cicatricial Ectropion Complications Trichiasis and Distichiasis Management of Trichiasis Management of Distichiasis Floppy Eyelid Syndrome Treatment Giant Fornix Syndrome Lid Imbrication Syndrome Treatment Lagophthalmos Treatment Eyelid Retraction Treatment References SECTION 2 Diseases of the Lid: Tumors, Inflammation, and Infection 27 - Benign Lid Tumors Histology of the Eyelid Skin Approach to Diagnosis and Management Benign Tumors of the Epidermis Acrochordon Epidermal Inclusion Cyst Seborrheic Keratosis Milia Keratoacanthoma Inverted Follicular Keratosis Cutaneous Horn Linear Epidermal Nevus Nodular Elastosis With Cysts and Comedones (Favre-Racouchot Syndrome) Actinic Keratosis (Solar Keratosis) Dermoid and Epidermoid Cysts Oncocytoma Phakomatous Choristoma Benign Tumors of the Dermis Tumors Derived From Neural Tissue Neurofibroma Neurilemoma Neuroma Granular Cell Tumor Tumors Derived From Smooth Muscle Leiomyoma Tumors Derived From Vascular Tissue Nevus Flammeus Capillary Hemangioma Cherry Hemangioma Cavernous Hemangioma Varix Lymphatic Malformation Arteriovenous Malformation Pyogenic Granuloma Glomus Tumor Intravascular Papillary Endothelial Hyperplasia Angiolymphoid Hyperplasia With Eosinophilia Tumors Derived From Histiocytes Xanthelasma Xanthoma Juvenile Xanthogranuloma Tumors Derived From Fibrous Tissue Dermatofibroma Benign Tumors of the Eyelid Adnexa Tumors of Sweat Gland Origin Tumors of Eccrine Sweat Gland Origin . Syringomas are benign eccrine sweat gland tumors found most commonly in young females. Clinically, they usually appear as mult... . The eccrine spiradenoma shows differentiation toward the secretory portion of the eccrine gland. This benign tumor is an uncom... . The eccrine acrospiroma (clear cell hidradenoma) shows differentiation toward both ductal and secretory portions of the eccrin... . These cystic lesions tend to be small (1–3 mm in diameter) and multiple; commonly they occur clustered around the eyelids and ... . The pleomorphic adenoma (mixed tumor of skin, chondroid syringoma) occurs most commonly in the head and neck region and may in... Tumors of Apocrine Sweat Gland Origin . The apocrine type of hidrocystoma (cyst of Moll) is a true adenoma of the secretory cells of the glands of Moll rather than a ... . Although recognized as a tumor of sweat gland origin, the exact origin is uncertain. Solitary or multiple lesions can occur. C... . Appearing at birth or during early childhood, syringocystadenoma papilliferum occurs most commonly on the scalp or face. The l... Tumors of Hair Follicle Origin Trichoepithelioma Trichoadenoma Trichofolliculoma Trichilemmoma Pilomatricoma Tumors of Sebaceous Gland Origin Sebaceous Gland Hyperplasia Sebaceous Adenoma Benign Melanocytic Lesions Nevocellular Nevi Junctional Nevi Compound Nevi Melanoma Intradermal Nevi Variants of Nevocellular Nevi Congenital Nevus Blue Nevus Nevus of Ota Freckle Lentigo Simplex Lentigo Senilis Lentigo Maligna Conclusion References 28 - Malignant Eyelid Tumors Basal Cell Carcinoma Clinical Appearance Biologic Behavior Clinical Diagnosis Treatment Mohs Micrographic Surgery Frozen Section Control Ionizing Radiation Therapy Chemotherapy Recurrence Squamous Cell Carcinoma Premalignant Lesions Actinic Keratosis Bowen Disease Epidemiology and Differential Diagnosis Squamous Cell Carcinoma Clinical Presentation Biologic Behavior Treatment Sebaceous Cell Carcinoma Etiology Clinical Presentation Treatment Prognosis Merkel Cell Carcinoma (Trabecular Carcinoma) Normal Merkel Cell Merkel Cell Carcinomas Clinical Presentation Histopathology Treatment . Localized disease requires prompt and aggressive initial treatment for potential favorable outcome. Wide surgical excision of ... . Chemotherapy using a combination of drugs that are active against small cell carcinoma of the lung is recommended for patients... Malignant Melanoma Lentigo Maligna Melanoma Superficial Spreading Melanoma Nodular Melanoma Treatment Basal Cell Nevus Syndrome (Gorlin-Goltz Syndrome) Systemic Manifestations Ophthalmic Manifestations Treatment Xeroderma Pigmentosum Cutaneous Abnormalities Ocular Abnormalities Neurologic Abnormalities Treatment Muir-Torre Syndrome Summary References 29 - Blepharitis: Overview and Classification Classification Differential Diagnosis Management References 30 - Eyelid Infections Viral Infections Herpes Simplex Virus Varicella-Zoster Virus Molluscum Contagiosum Human Papillomavirus Bacterial Infections Hordeolum Preseptal Cellulitis Impetigo Contagiosa Rare Bacterial Infections Anthrax Syphilis Mycobacterial Infections Actinomyces Canaliculitis Fungal Infections Blastomycosis Coccidiomycosis Cryptococcosis Dermatophytosis/Tinea Palpebrum Sporotrichosis Mucormycosis Parasitic Eyelid Infections Demodicosis Phthiriasis and Pediculosis Palpebrarum Leishmaniasis Ophthalmomyiasis Externa References SECTION 3 Disorders of Tear Production and the Lacrimal System 31 - Dry Eye Overview: Classification and Treatment Algorithm DEFINITION Pathophysiology Classification Scheme Aqueous Tear-Deficient Dry Eye Sjögren Syndrome Dry Eye Non-Sjögren Syndrome Dry Eye Evaporative Dry Eye TFOS DEWS II DED Diagnostic Algorithm (Fig. 31.2) Step 1 Education Tear Supplementation Essential Fatty Acid Supplementation Step 2 Tear Conservation . Punctal occlusion is one of the most useful and practical therapies for conserving tears in patients with aqueous-deficient DE... . Moisture-conserving spectacles reduce tear evaporation by increasing humidity around the eye and can be worn during the day or... In Office Procedures . These devices have proven efficacious in the treatment of MGD and may offer benefit in patients with poor compliance of lid hy... . Microblepharoexfoliation (MBE) is an in-office procedure for mechanically debriding the lid margin of scurf, collarettes, kera... . Intense pulsed light (IPL) is effective in patients with prominent features of ocular rosacea and MGD. The mechanism of action... . Topical cyclosporine A (CsA), a calcineurin inhibitor, reduces proinflammatory cytokines (e.g., conjunctival IL-6 levels), act... . This topical immunomodulator inhibits binding between LFA-1 and ICAM-1 (intercellular adhesion molecule 1) on epithelial cells... . Corticosteroids exert their immuno Oral Macrolide or Tetracycline Antibiotic Step 3 Therapeutic Contact Lens Options Oral Secretagogues Autologous/Allogenic Serum Step 4 Other Surgical Approaches . A partial tarsorrhaphy is reserved for severe or refractory DED.35 This surgical approach reduces the area of exposed ocular s... . Salivary submandibular gland transplantation is capable of replacing deficient mucin and aqueous tears. This minimal-risk surg... . Topical acetylcysteine is mentioned in the literature as a DED treatment as early as the 1960s and is still occasionally used ... . Amniotic membrane grafts in the acute stage of Stevens-Johnson syndrome56 and ocular surface burns57 has been shown to reduce ... Conclusion References 32 - Aqueous Deficiency Dry Eye Syndrome PATHOPHYSIOLOGY Sjögren Syndrome Dry Eye Non-Sjögren Syndrome Dry Eye Secondary Lacrimal Gland Deficiencies Lacrimal Gland Infiltration Graft-Versus-Host Disease Obstruction of the Lacrimal Gland Ducts Reflex Hyposecretion Systemic Medications Diagnosis History Physical Examination Diagnostic Tests Systemic Work-Up Management References 33 - Meibomian Gland Dysfunction and Evaporative Dry Eye Normal Anatomy of the Meibomian Glands Terminology Physiology/Pathophysiology Classification of Meibomian Gland Dysfunction Diagnosis of Meibomian Gland Dysfunction Associated Conditions Animal Models of Meibomian Gland Dysfunction Lipid Composition and Behavior of Human Meibomian Secretion Role of Microorganisms Treatment Conclusion References 34 - Dacryoadenitis, Dacryocystitis, and Canaliculitis DACRYOADENITIS Dacryocystitis Canaliculitis References 35 - Epiphora Introduction Etiology Overproduction Outflow Obstruction Lacrimal Pump Failure Differential Diagnosis Evaluation Treatment Overproduction Outflow Obstruction Lacrimal Pump Failure Summary References PART VI The Conjunctiva SECTION 1 Conjunctivitis 36 - Conjunctivitis: An Overview and Classification Conjunctival Injection Conjunctivitis Morphologic Responses Papillae Follicles Membranes Cicatrizing Changes Granulomas Type of Exudate Anatomic Localization Acute Conjunctivitis Acute Papillary Conjunctivitis Acute Follicular Conjunctivitis Adenovirus Inclusion Conjunctivitis Ocular Herpes Infections RNA-Containing Viruses Poxviruses Acute Membranous Conjunctivitis Chronic Conjunctivitis Giant Papillary Conjunctivitis Vernal Keratoconjunctivitis Atopic Keratoconjunctivitis Secondary Giant Papillary Conjunctivitis Chronic Papillary Conjunctivitis Masquerade Syndrome Lacrimal Drainage System Infection Superior Limbic Keratoconjunctivitis Floppy Eyelid Syndrome Blepharoconjunctivitis Aqueous Tear Deficiency Dry Eye Mucus-Fishing Syndrome Toxic Papillary Keratoconjunctivitis Chronic Follicular Conjunctivitis Moraxella Molluscum Contagiosum Toxic Follicular Conjunctivitis Lyme Disease Chronic Membranous Conjunctivitis Giant Fornix Syndrome Cicatrizing and Granulomatous Conjunctivitis References 37 - Bacterial Conjunctivitis Introduction Incidence Risk Factors Categories of Bacterial Conjunctivitis Acute Conjunctivitis Pathogens Signs and Symptoms Associated Extraocular Manifestations Diagnosis Management Monitoring for Resistance and Emerging Alternative Therapy Hyperacute Bacterial Conjunctivitis Neisseria gonorrhoeae Clinical Manifestations Management Microbiologic Confirmation Other Considerations in Management of Gonococcal Conjunctivitis Neisseria meningitidis Management Chronic Conjunctivitis Staphylococcal Infections Moraxella Chlamydial Infections Trachoma Inclusion Conjunctivitis Ophthalmia Neonatorum Other Conclusion References 38 - Viral Conjunctivitis DNA VIRUSES Adenoviruses Pharyngoconjunctival Fever Epidemic Keratoconjunctivitis Acute Nonspecific Follicular Conjunctivitis Chronic Conjunctivitis Laboratory Diagnosis Treatment Herpes Simplex Virus Varicella Zoster Virus Epstein-Barr Virus Cytomegalovirus Variola and Vaccinia Virus Molluscum Contagiosum Virus RNA Viruses Picornaviruses Paramyxoviruses Togaviruses Flaviviruses Filoviruses References 39 - Chlamydial Infections Introduction Biology Laboratory Tests for Chlamydia Trachoma Epidemiology Clinical Manifestations Pathophysiology Treatment Public Health Efforts Neonatal Inclusion Conjunctivitis Adult Inclusion Conjunctivitis Lymphogranuloma Venereum Chlamydia Psittaci and Chlamydia Pneumoniae Conclusion References 40 - Ophthalmia Neonatorum Pathogenesis and Assessment of Risk The Role of Sexually Transmitted Diseases Causes of Neonatal Conjunctivitis Chemical Bacterial Chlamydial Viral Fungal Laboratory Diagnosis Laboratory Diagnosis Treatment Ocular Prophylaxis References 41 - Parinaud Oculoglandular Syndrome History Directed Work-Up Individual Etiologies Cat-Scratch Disease Tularemia Tuberculosis Sporotrichosis Syphilis Other Causes References 42 - Seasonal and Perennial Allergic Conjunctivitis INTRODUCTION Clinical Findings Pathophysiology Local Treatments Systemic Treatments Future Developments Conclusion References 43 - Vernal and Atopic Keratoconjunctivitis VERNAL KERATOCONJUNCTIVITIS Definition Demographics Symptoms Signs Pathophysiology Diagnosis Treatment Atopic Keratoconjunctivitis Definition Demographics Symptoms Signs Pathophysiology Diagnosis Treatment References 44 - Giant Papillary Conjunctivitis SYMPTOMS AND SIGNS Histopathology and Immunopathology Pathophysiology Differential Diagnosis Treatment Prognosis References 45 - Mucous Membrane Pemphigoid INTRODUCTION Epidemiology Pathogenesis Diagnosis Ocular Manifestations Management of Ocular Mucous Membrane Pemphigoid Medical Management Dry Eye Disease Chronic Blepharitis and Meibomitis Immunomodulatory Surgical Treatment Epilation Lid Surgery Fornix Reconstruction Corneal Surgery Conclusion References 46 - Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis INTRODUCTION History Classification Incidence Clinical Findings Initial Presentation Eye Findings Acute Eye Findings Chronic Eye Findings Nonocular Findings Recurrent Disease Incidence of Ocular Complications Differential Diagnosis Ocular Disorders Dermatologic Manifestations Etiology Pathogenesis Histopathology Skin Eye Management Systemic Disease Ophthalmic Disease Acute Stage Chronic Stage Conclusion References 47 - Toxic Conjunctivitis Introduction Toxicity Versus Allergy Cellular Mechanisms Signs and Symptoms Diagnostic Testing Toxic Keratoconjunctivitis Related to Topical Medications Methods of Assessing Toxicity Toxicity of Specific Agents Antivirals Glaucoma Medications Antibiotics and Antifungals Anesthetics Preservatives Follicular Conjunctivitis Hurricane Keratitis Drug-induced Mucous Membrane Pemphigoid Other Causes of Toxic Keratoconjunctivitis Cosmetics and Skin Care Products Hair Care Products Tear Gas Weapons and Lacrimating Agents Therapeutic Considerations References 48 - Superior Limbic Keratoconjunctivitis Introduction and Epidemiology Clinical Presentation Etiology Histopathology Treatment Modalities Medical Treatment Surgery Differential Diagnosis Summary References 49 - Ligneous Conjunctivitis Clinical Findings EPIDEMIOLOGY Pathophysiology/Histopathology Etiology Treatment References 50 - Conjunctivochalasis INTRODUCTION Epidemiology Pathophysiology Clinical Presentation Diagnosis Management Medical Treatment Surgical Treatment Conjunctival Cauterization Conjunctival Excision Other Conclusion References SECTION 2 Conjunctival Disease: Tumors 51 - Epithelial Tumors of the Conjunctiva Benign Tumefactions Benign Hereditary Intraepithelial Dyskeratosis Pseudoepitheliomatous Hyperplasia, Large Cell Conjunctival Squamous Papilloma Inverted Conjunctival Papillomas Dacryoadenoma Sebaceous Adenoma Pterygia and Pingueculae Malignant Tumefactions Noninvasive Ocular Surface Squamous Neoplasia/Conjunctival Intraepithelial Neoplasia Invasive Ocular Surface Squamous Neoplasia Corneal Epithelial Dysmaturation and Epithelial Mucoepidermoid Carcinoma Spindle Cell Carcinoma Adenoid Squamous Carcinoma Clear Cell Squamous Cell Carcinoma Transitional Cell Carcinoma Sebaceous Cell Carcinoma Basal Cell Carcinoma References 52 - Medical and Surgical Management of Ocular Surface Squamous Neoplasia INITIAL CONSIDERATIONS Role of Optical Coherence Tomography in Diagnosis and Management of Ocular Surface Squamous Neoplasia Choice of Treatment Modality for Ocular Surface Squamous Neoplasia Medical Management of Ocular Surface Squamous Neoplasia Topical Immunomodulatory and Chemotherapeutic Agents Immunomodulators . IFN is an endogenous immunomodulatory glycoprotein released by various immune cells with antiviral, antimicrobial, and antineo... Antimetabolites . 5-FU is a pyrimidine analog that blocks DNA and RNA synthesis by inhibiting thymidylate synthase. Long used as systemic therap... . MMC is an alkylating agent derived from the actinobacterium Streptomyces caespitosus and exerts its antineoplastic effect by c... Radiotherapy Anti-Vascular Endothelial Growth Factor Surgical Management of Ocular Surface Squamous Neoplasia “No-Touch” Technique Intraoperative Adjunctive Treatments Mitomycin C Amniotic Membrane Transplant Postoperative Management and Complications Standard Postoperative Regimen Neoadjuvant Treatments Complications of Surgical Resection Role of Optical Coherence Tomography During Treatment and Surveillance of Ocular Surface Squamous Neoplasia References 53 - Melanocytic Neoplasms of the Conjunctiva INTRODUCTION CLINICAL AND HISTOPATHOLOGIC FEATURES OF CONJUNCTIVAL MELANOCYTIC LESIONS Conjunctival Nevus Conjunctival Melanosis Epithelial Congenital Melanosis Subepithelial Congenital Melanosis Secondary Acquired Melanosis Primary Acquired Melanosis Malignant Melanoma Management of Conjunctival Melanocytic Lesions Diagnosis Treatment Financial Support References 54 - Subepithelial Tumors of the Conjunctiva Congenital Lesions Choristomas Dermoid and Dermolipoma Ectopic Lacrimal Gland; Simple and Complex Choristomas Epibulbar Osseous and Neuroglial Choristomas Hamartomas Conjunctival Cysts Pyogenic Granuloma/Lobular Capillary Hemangioma Pinguecula/Pterygium Elastofibroma Oculi Nodular Fasciitis Granulomatous and Histiocytic Lesions Epibulbar Molluscum Contagiosum Keloid Amyloid Hematic Cyst Neoplastic Lesions Fibrous Histiocytoma Kaposi Sarcoma Oncocytoma (Oxyphilic Adenoma) Basal Cell Carcinoma Malignant Melanoma Lymphoid Lesions Other Subconjunctival Neoplastic Lesions References PART VII Diseases of the Cornea SECTION 1 Developmental Abnormalities of Cornea 55 - Developmental Corneal Anomalies of Size and Shape Absence of the Cornea Anomalies of Size Megalocornea Microcornea Anomalies of Shape Oval Cornea Astigmatism Sclerocornea (Cornea Plana) Posterior Keratoconus Keratoglobus Congenital Anterior Staphyloma and Keratectasia References 56 - Axenfeld-Rieger Syndrome Axenfeld-Rieger Syndrome History Clinical Features Cornea Iridocorneal Angle Iris Glaucoma Other Ocular Findings Nonocular Findings Face Teeth Umbilical Genital Others Differential Diagnosis Pathogenesis Genetics Chromosome 4q, RIEG1, PITX2 Chromosome 13q, RIEG2 Chromosome 6p, FOXC1 Genotype-Phenotype Correlations Natural History Treatment References 57 - Peters Anomaly Peters Anomaly Peters Anomaly History Clinical Features Cornea Anterior Chamber Lens Glaucoma Other Ocular Findings Nonocular Findings Differential Diagnosis Pathogenesis Genetics Natural History Treatment References SECTION 2 Corneal Manifestations of Systemic Disease and Therapies 58 - Corneal Manifestations of Metabolic Diseases INTRODUCTION Disorders of Carbohydrate Metabolism Diabetes Mellitus: Diabetic Keratopathy Lysosomal Storage Disorders Introduction Mucopolysaccharidoses Mucopolysaccharidoses I Hurler Syndrome (Mucopolysaccharidoses I-H) Scheie Syndrome (Mucopolysaccharidoses IS) Hurler Scheie (Mucopolysaccharidoses I H/S) Hunter Syndrome (Mucopolysaccharidoses II) Sanfilippo Syndrome (Mucopolysaccharidoses III) Morquio Syndrome (Mucopolysaccharidoses IV) Maroteaux-Lamy Syndrome (Mucopolysaccharidoses VI) Sly Syndrome (Mucopolysaccharidoses VII) Natowicz Syndrome (Mucopolysaccharidoses IX) Corneal Histopathology in Mucopolysaccharidoses Dyslipoproteinemias Hyperlipoproteinemias and Schnyder Corneal Dystrophy Hypolipoproteinemias Lipidoses Fabry Disease Multiple Sulfatase Deficiency Generalized Gangliosidoses Drug-induced lipidoses Chloroquine Amiodarone Other Agents Mucolipidoses Galactosialidosis Cystinosis Tyrosinemia II Alkaptonuria Amyloidosis Gout Porphyria References 59 - Skeletal and Connective Tissue Disorders With Anterior Segment Manifestations Classification of Skeletal Disorders Craniofacial Dysostosis Syndromes Crouzon Syndrome Apert Syndrome Saethre-Chotzen Syndrome Goldenhar Syndrome Ehlers-Danlos Syndrome Osteogenesis Imperfecta Kniest Dysplasia Marfan Syndrome Oculo-dento-osseous Dysplasia Sotos Syndrome (Cerebral Gigantism) Dermo-chondro-corneal Dystrophy of François Werner Syndrome Hypophosphatasia Treatment of Ocular and Corneal Abnormalities in Skeletal Disorders References 60 - Granulomatosis With Polyangiitis and Other Systemic Inflammatory Diseases Granulomatosis With Polyangiitis Epidemiology, Etiology, and Pathogenesis Systemic Manifestations 60 Ocular Manifestations Management Inflammatory Bowel Disease Epidemiology, Etiology, and Pathogenesis Systemic Manifestations Ocular Manifestations Management Whipple Disease Epidemiology, Etiology, and Pathogenesis Systemic Manifestations Ocular Manifestations Management Chronic Granulomatous Disease Epidemiology, Etiology, and Pathogenesis Systemic Manifestations Ocular Manifestations Management Kawasaki Disease Epidemiology, Etiology, and Pathogenesis Systemic Manifestations Ocular Manifestations Management References 61 - Nutritional Disorders METABOLISM OF VITAMIN A Historical Considerations Classification and Clinical Manifestations of Xerophthalmia Night Blindness (XN) Conjunctival Xerosis (X1A) and Bitot Spots (X1B) Corneal Xerosis (X2) Corneal Ulceration/Keratomalacia (X3A/X3B) Pathogenesis of Corneal Ulceration Interactions With Other Factors Epidemiology Treatment Other Nutritional Deficiencies Vitamin B (Riboflavin) Vitamin C Other Nutrients Discrete Colliquative Keratopathy Summary References 62 - Hematologic Diseases and Malignancies Basics of Hematology Anemia Iron Deficiency Anemia Systemic Manifestations Ophthalmic Manifestations Thalassemia Systemic Manifestations Ophthalmic Manifestations Sickle Cell Disease Systemic Manifestations Ophthalmic Manifestations Leukemia Acute Leukemia: Acute Myeloid Leukemia Systemic Manifestations Ophthalmic Manifestations Acute Leukemia: Acute Lymphoblastic Leukemia Systemic Manifestations Ophthalmic Manifestations Chronic Leukemia: Chronic Myeloid Leukemia Systemic Manifestations Ophthalmic Manifestations Chronic Leukemia: Chronic Lymphocytic Leukemia Systemic Manifestations Ophthalmic Manifestations Infectious Complications of Leukemia Ocular Toxicity of Leukemia Treatment Ophthalmic Diagnostic Techniques in Leukemia Ocular Treatment in Leukemia Bruton Agammaglobulinemia Plasma Cell Disorders Multiple Myeloma Systemic Manifestations Ophthalmic Manifestations Waldenström Macroglobulinemia Systemic Manifestations Ophthalmic Manifestations Monoclonal Gammopathy of Undetermined Significance Systemic Manifestations Ophthalmic Manifestations Cryoglobulinemia Systemic Manifestations Ophthalmic Manifestations Differential Diagnosis of the Crystalline Keratopathies References 63 - Endocrine Disease and the Cornea DIABETES MELLITUS Biochemistry Animal Studies Morphology Clinical Correlations Parathyroid Disease Hypoparathyroidism Hyperparathyroidism Thyroid Disease Multiple Endocrine Neoplasia References 64 - Dermatologic Disorders and the Cornea Psoriasis Seborrheic Dermatitis Rosacea Contact Dermatitis Atopic Dermatitis Pityriasis Rubra Pilaris Impetigo Immunobullous Disorders Pemphigus Pemphigus Vulgaris Pemphigus Foliaceus Paraneoplastic Pemphigus Pemphigoid Bullous Pemphigoid Mucous Membrane Pemphigoid Drug-Induced Ocular Pemphigoid Linear IgA Bullous Dermatosis Epidermolysis Bullosa Acquisita Genetic Disorders Acrodermatitis Enteropathica Darier Disease (Keratosis Follicularis) Dyskeratosis Congenita Ectodermal Dysplasias Ehlers-Danlos Syndrome Epidermolysis Bullosa Focal Dermal Hypoplasia (Goltz Syndrome) Ichthyosis Incontinentia Pigmenti (Bloch-Sulzberger Syndrome) Keratosis Follicularis Spinulosa Decalvans Melkersson-Rosenthal Syndrome Richner-Hanhart Syndrome Rothmund-Thomson Syndrome Werner Syndrome Xeroderma Pigmentosum Miscellaneous Disorders Acanthosis Nigricans Epidermal Nevus Syndrome Hydroa Vacciniforme Juvenile Xanthogranuloma Kaposi Sarcoma Malignant Atrophic Papulosis (Degos Disease) Summary References 65 - Infectious Disease: Ocular Manifestations EMERGING INFECTIONS Ebola Virus Disease Background Epidemiology Transmission Systemic Disease Ocular Disease Diagnosis Treatment and Prevention Chikungunya Background Epidemiology Transmission Systemic Disease Ocular Disease Diagnosis Treatment and Prevention West Nile Virus Background Epidemiology Transmission Systemic Disease Ocular Disease Diagnosis Treatment and Prevention Zika Virus Disease Background Epidemiology Transmission Systemic Disease Ocular Disease Diagnosis Treatment and Prevention Established Infections Hansen Disease (Leprosy) Background Epidemiology Transmission Systemic Disease Ocular Disease . Nerve involvement is more common in the tuberculoid group, and one of the principal nerves affected is the facial nerve on one... . Superciliary and ciliary madarosis are common and a contributory finding for diagnosis as they usually occur relatively early ... . Scleritis, episcleritis, and uveitis can be a presenting complaint in perhaps as many as one out of five patients. These can b... . Intraocular inflammation can be seen in those (especially lepromatous) patients who have recently been treated or sometimes wi... Diagnosis Treatment and Prevention Brucellosis Background Epidemiology Transmission Systemic Disease Ocular Disease Diagnosis Treatment and Prevention Onchocerciasis Background Epidemiology Transmission Ocular Disease . Microfilariae can and do enter the cornea, probably via the conjunctiva, corneal nerves, or perhaps the sclera. The organism c... . There are at least two forms of uveitis including anterior iridocyclitis and a posterior chorioretinitis. The microfilariae ca... . The second form of uveitis is a chorioretinitis, although there are many potential presentations of this form. The organism pr... Diagnosis Treatment and Prevention . Although ivermectin is a relatively safe drug, there can be some problems with coinfecting organisms that should be recognized... Lyme Disease Background Epidemiology Transmission Systemic Disease Ocular Disease Diagnosis Treatment and Prevention Tuberculosis Background Epidemiology Transmission Systemic Disease Ocular Disease Diagnosis Treatment and Prevention References 66 - Corneal and External Ocular Infections in Acquired Immunodeficiency Syndrome Viral Infections Human Immunodeficiency Virus Cytomegalovirus Varicella-Zoster Virus Herpes Zoster Ophthalmicus Varicella-Zoster Virus Keratitis Herpes Simplex Virus Molluscum Contagiosum Virus Other Viruses Bacterial Infections Fungal Infections Parasitic Infections Summary References 67 - Ocular Graft-Versus-Host Disease INTRODUCTION Pathophysiology of Acute and Chronic Graft-Versus-Host Disease Ocular Graft-Versus-Host Disease Conjunctival Disease Keratoconjunctivitis Sicca/Dry Eye Syndrome Meibomian Gland Dysfunction Other Findings of Ocular Graft-Versus-Host Disease Diagnostic Criteria of Ocular Graft-Versus-Host Disease Increase Lubrication Control and Immunomodulate Inflammation Manage Meibomian Gland Dysfunction Protect the Ocular Surface Consider Surgery (With Caution) References 68 - Corneal Manifestations of Local and Systemic Therapies Topical Ophthalmic Medications Topical Preservatives Topical Anesthetics Topical Antimicrobials Topical Antiinflammatories Antiglaucoma Medications Surgical Solutions Surgical Antiseptics Irrigating Solutions, Viscoelastics, Intracameral Injections, Capsular Dyes Topical Chemotherapeutic Agents Systemic Therapies Systemic Chemotherapeutics Other Systemic Medications Corneal Deposits Conclusion References SECTION 3 Corneal Dystrophies, Ectatic Disorders, and Degenerations 69 - Corneal Dystrophy Classification History Corneal Dystrophy Definition Corneal Dystrophy Classification in the Literature Shortcomings of Corneal Dystrophy Nomenclature and Classification The Impact of Genotyping on Corneal Dystrophy Nomenclature The International Committee for Classification of Corneal Dystrophies The IC3D Classification—an Anatomically Based Classification System The IC3D Category System to Denote the Evolution of a Corneal Dystrophy The IC3D Templates Changes from 2008 to 2015 IC3D Summary References 70 - Anterior Corneal Dystrophies EPITHELIAL CORNEAL DYSTROPHIES Meesmann Corneal Dystrophy (IC3D, MECD, Category 1) Lisch Epithelial Corneal Dystrophy (IC3D, LECD, Category 2) Gelatinous Drop-Like Corneal Dystrophy (IC3D, GDLD, Category 1) Epithelial Basement Membrane Dystrophy (IC3D, EBMD, Some Category 1) Corneal Dystrophies of Bowman Layer References 71 - The Stromal Dystrophies Granular Corneal Dystrophy Granular Corneal Dystrophy, Type 1 Clinical Features Epidemiology and Heritability Histopathology Management Granular Corneal Dystrophy, Type 2 Clinical Features Epidemiology and Heritability Histopathology Management Lattice Corneal Dystrophy Lattice Corneal Dystrophy: Classic and Variant Clinical Features Epidemiology and Heritability Histopathology Management Macular Corneal Dystrophy Clinical Features Epidemiology and Heritability Histopathology Management Schnyder Corneal Dystrophy Clinical Features Epidemiology and Heritability Histopathology Management Fleck Corneal Dystrophy Clinical Features Epidemiology and Heritability Histopathology Management Central Cloudy Dystrophy of François Clinical Features Epidemiology and Heritability Histopathology Management Posterior Amorphous Corneal Dystrophy Clinical Features Epidemiology and Heritability Histopathology Management Congenital Stromal Corneal Dystrophy Clinical Features Epidemiology and Heritability Histopathology Management Pre-Descemet Corneal Dystrophy Clinical Features Epidemiology and Heritability Histopathology Management References 72 - Descemet Membrane and Endothelial Dystrophies Posterior Polymorphous Corneal Dystrophy Introduction Molecular Genetics Clinical Presentation and Course Differential Diagnosis Prognosis and Management Light Microscopy Electron Microscopy Fuchs Endothelial Corneal Dystrophy Introduction Molecular Genetics Early-Onset FECD Late-Onset FECD Clinical Presentation and Course Nonguttate Endothelial Dystrophy Differential Diagnosis Prognosis and Management Histopathology Light Microscopy Electron Microscopy Congenital Hereditary Endothelial Dystrophy Molecular Genetics Differential Diagnosis Clinical Presentation and Course Prognosis and Management Electron Microscopy X-Linked Endothelial Corneal Dystrophy Molecular Genetics Clinical Presentation and Course Differential Diagnosis Prognosis and Management Microscopy References 73 - Noninflammatory Ectatic Disorders KERATOCONUS Definition Prevalence, Distribution, and Course Associated Disease Systemic Disease Ocular Disease Etiology Biochemistry Heredity Pathology Diagnosis Corneal Imaging Overview Corneal Power Corneal Elevation and Thickness Biomechanical Measurement Treatment Contact Lens Intrastromal Ring Segments Corneal Crosslinking Keratoplasty Bowman Layer Transplantation Post-Keratorefractive Surgery Corneal Ectasia Pellucid Marginal Degeneration Keratoglobus Posterior Keratoconus References 74 - Iridocorneal Endothelial Syndrome HISTORY AND BACKGROUND Clinical Features Essential Iris Atrophy Chandler Syndrome Cogan-Reese Syndrome Iris Nevus Syndrome Etiology Clinical Course Differential Diagnosis Management Summary References 75 - Corneal and Conjunctival Degenerations Corneal Degenerations Arcus Senilis Limbal Girdle (of Vogt) Iron Deposits Calcific Degeneration Crocodile Shagreen Cornea Farinata Senile Furrow Hassall-Henle Bodies Lipid Degeneration Spheroidal Degeneration (Climatic Droplet Keratopathy) Climatic Proteoglycan Stromal Keratopathy Amyloid Degeneration Salzmann Nodular Degeneration Peripheral Hypertrophic Subepithelial Corneal Degeneration Corneal Keloids Terrien Marginal Corneal Degeneration Epithelial Basement Membrane Degeneration Conjunctival Degenerations Pingueculae Pterygium Concretions References SECTION 4 Corneal Infections 76 - Bacterial Keratitis INTRODUCTION EPIDEMIOLOGY HOST DEFENSE AND RISK FACTORS Defense of the Ocular Surface External Risk Factors Contact Lens Use Ocular Surface Abnormalities PATHOGENESIS Bacterial Adherence Bacterial Invasion Corneal Inflammation and Tissue Damage NATURAL HISTORY PRESENTATION DIFFERENTIAL DIAGNOSIS CLINICAL EXAMINATION SPECIFIC BACTERIAL ULCERS Staphylococci Streptococci Nocardia Nontuberculous Mycobacteria Pseudomonas Neisseria Bacillus Infectious Crystalline Keratopathy LABORATORY INVESTIGATION Cultures and Smears Cultures Indications for Re-Culture Stains Antimicrobial Susceptibility Testing OTHER DIAGNOSTIC TOOLS Corneal Biopsy Impression Cytology SPECIFIC THERAPEUTIC AGENTS AND RESISTANCE PATTERNS Cephalosporins Glycopeptides Aminoglycosides Macrolides Fluoroquinolones Sulfonamide and Trimethoprim Anti-Collagenase Antiseptics STRATEGIES FOR INITIAL MANAGEMENT Culture-Guided Approach Empiric Approach Case-Based Approach MODIFICATION OF THERAPY Review After 1 Week Corticosteroid Therapy THERAPY FOR COMPLICATED CASES Cyanoacrylate Tissue Adhesives Therapeutic Contact Lenses Amniotic Membrane SURGICAL MANAGEMENT Conjunctival Flap Keratoplasty Collagen Crosslinking PREVENTION AND EARLY DETECTION COUNSELING AND REFERRAL REFERENCES 77 - Nontuberculous Mycobacteria Keratitis OVERVIEW Classification Risk Factors Clinical Features Laboratory Diagnosis Medical Treatment Surgical Treatment References 78 - Herpes Simplex Keratitis Viral Structure and Classification Epidemiology Pathogenesis Latency and Reactivation Immune Defense Mechanisms Clinical Manifestations Congenital and Neonatal Ocular Herpes Primary Ocular Herpes Recurrent Ocular Herpes Blepharitis Conjunctivitis Keratitis Iridocyclitis Diagnosis Management Herpetic Eye Disease Study Medical Management Antiviral Agents Corticosteroids Recommendations for Clinical Manifestations Antiviral Prophylaxis Surgical Therapy References 79 - Herpes Zoster Keratitis EPIDEMIOLOGY Clinical Disorders Herpes Zoster Ophthalmicus Ophthalmic Manifestations Neurologic Manifestations Diagnosis of Herpes Zoster Ophthalmicus Therapy of Varicella-Zoster Virus Infection Medical Treatment Surgical Treatment Varicella-Zoster Virus Vaccination References 80 - Less Common Viral Corneal Infections DNA VIRUSES Poxviridae Variola (Smallpox) Vaccinia Herpesviridae Cytomegalovirus RNA Viruses Picornaviridae Acute Hemorrhagic Conjunctivitis Togaviridae Rubella (German Measles) Virus Acquired Rubella Congenital Rubella Chikungunya Flaviviridae Zika West Nile Paramyxoviridae Rubeola (Measles) Mumps Newcastle Disease Orthomyxoviridae Influenza Filoviridae Ebola References 81 - Fungal Keratitis Introduction Pathogenesis Epidemiology Risk Factors Clinical Features Laboratory Diagnosis Medical Therapy Polyenes Azoles Echinocandins Crosslinking Surgical Therapy Prognosis Conclusion Acknowledgment References 82 - Acanthamoeba and Other Parasitic Corneal Infections Acanthamoeba Keratitis Introduction Incidence Risk Factors Pathogenesis Clinical Features Diagnosis Treatment Medical Surgical Role of Corticosteroids Other Parasitic Corneal Infections Non-Acanthamoeba Amoebic Keratitis Microsporidia Parasitic Keratitis of Primarily Endogenous Origin Onchocerciasis Leishmaniasis Trypanosomiasis Pythium Insidiosum References 83 - Corneal Diseases in the Developing World GLOBAL DATA ON CORNEAL BLINDNESS Causes of Corneal Blindness Xerophthalmia Clinical Features Management Trachoma Clinical Diagnosis Management Onchocerciasis Clinical Features Management Leprosy Clinical Features Management Ophthalmia Neonatorum Corneal Trauma Infectious Keratitis Traditional Eye Medicines Keratoconus Corneal Dystrophies Keratoplasty Models for TACKLING Corneal Blindness Conclusion References SECTION 5 Interstitial Keratitis 84 - Syphilitic Keratitis EPIDEMIOLOGY Pathogenesis Ocular Infection in Early Syphilis Corneal Immunity During Late Syphilis Stromal Keratitis of Congenital Syphilis Context Corneal Inflammation Corneal Neovascularization Bilateral Keratitis Anterior Uveitis Posterior Uveitis Recurrent Ocular Inflammation Otosyphilis and Neurosyphilis Stromal Keratitis of Acquired Syphilis Context Corneal Inflammation Syphilitic Keratopathy Corneal Opacity Ghost Vessels Thickened Descemet Membrane Iris Atrophy and Secondary Glaucoma Laboratory Investigation Serologic Testing of Stromal Keratitis Serologic Testing of Stromal Keratopathy Serologic Screening of Corneal Donors Management Antiinflammatory Therapy Antibacterial Therapy Keratoplasty References 85 - Nonsyphilitic Interstitial Keratitis NONSYPHILITIC BACTERIAL INFECTIONS ASSOCIATED WITH INTERSTITIAL KERATITIS Mycobacteria Infections Tuberculosis Other Bacterial Infections Lyme Disease (Borreliosis) Acanthamoeba Keratitis Onchocerciasis Leishmaniasis Trypanosomiasis Microsporidiosis Viral Infections Herpes Simplex Virus Infection Varicella-Zoster Virus Infection Other Viral Infections Cogan Syndrome Background Typical Cogan Syndrome Atypical Cogan Syndrome Epidemiology Clinical Manifestations Etiology and Pathogenesis Differential Diagnosis Laboratory Findings Treatment References SECTION 6 Noninfectious Keratopathy 86 - Filamentary Keratitis INTRODUCTION BACKGROUND PATHOPHYSIOLOGY ASSOCIATED CONDITIONS CLINICAL MANIFESTATIONS TREATMENT SUMMARY REFERENCES 87 - Superficial Punctate Keratitis of Thygeson History Epidemiology Disease Course and Clinical Manifestations Differential Diagnosis Treatment Pathogenesis References 88 - Neurotrophic Keratitis Clinical Causes Biochemical Basis Clinical Findings Histopathologic Findings Clinical Evaluation Management Summary References 89 - Factitious Keratoconjunctivitis Introduction Factitious Disorders Defined Munchausen Syndrome (Chronic Factitious Disorder) Diagnosis of Factitious Keratoconjunctivitis Treatment of Factitious Disease Malingering Mucus Fishing Syndrome Inadvertent or Unintentionally Self-Induced Trauma Self-Induced Trauma and Psychosis Neurologic Disorders Self-Inflicted Injuries in Children Topical Anesthetic Abuse History Local Effects on the Corneal Surface Clinical Presentation Diagnosis Treatment Conclusion References SECTION 7 Immunologic Disorders of the Cornea 90 - Corneal Disease in Rheumatoid Arthritis Introduction Pathogenesis Clinical Manifestations Articular Manifestations Extra articular Manifestations Rheumatoid Nodules Vasculitis Ocular Manifestations Keratoconjunctivitis Sicca Scleritis Keratitis Additional Ocular Disease Treatment Systemic Disease Ocular Disease Keratoconjunctivitis Sicca Keratitis Scleritis . Systemic medical therapy for RA-associated nonnecrotizing scleritis and keratitis is successful in most cases.45 Initial treat... Necrotizing Scleritis and Peripheral Ulcerative Keratitis Effectiveness of Systemic Immunosuppressive Therapy in Ocular Inflammation Surgical Management Conclusion References 91 - Corneal Disease Associated With Nonrheumatoid Collagen-Vascular Disease The Collagenoses Systemic Lupus Erythematosus Systemic Sclerosis Mixed Connective Tissue Disease Polymyositis and Dermatomyositis Relapsing Polychondritis The Vasculitides Granulomatosis With Polyangiitis Polyarteritis Nodosa Churg-Strauss Syndrome Cogan Syndrome Behçet Disease Giant Cell Arteritis Conclusion References 92 - Phlyctenular Keratoconjunctivitis and Marginal Staphylococcal Keratitis Phlyctenular Keratoconjunctivitis Background and History Etiology and Epidemiology Pathogenesis Clinical Features Differential Diagnosis Diagnostic Evaluation Treatment Staphylococcal Marginal Keratitis Background Pathogenesis Clinical Features Differential Diagnosis Diagnostic Evaluation Treatment References 93 - Mooren Ulcer Introduction Epidemiology Etiology Pathogenesis Clinical Features Symptoms Signs Differential Diagnosis Diagnosis Treatment Systemic Immunosuppression Other Agents Additional Surgery Prognosis Summary References SECTION 8 Corneal Trauma 94 - Mechanical Injury History Examination Abrading Injuries Epithelial Abrasions Etiology Clinical Signs and Findings Treatment Healing Complications Stromal Abrasions Etiology Clinical Signs and Findings Treatment Complications Blunt Trauma Contusion Injuries Clinical Signs and Findings Diffuse Endotheliopathy Endothelial Rings Stromal Injuries and Fractures Obstetric Injuries Pathogenesis Clinical Signs Management Injuries Caused by Radiant Energy Ultraviolet Radiation Corneal Effects of Ultraviolet Irradiation Clinical Signs and Findings Infrared Radiation Thermal Burns Clinical Signs and Findings Management Foreign Body Injuries Clinical Signs and Findings Stings Bee and Wasp Stings Jellyfish Stings References 95 - Chemical and Thermal Injuries of the Eye Introduction Epidemiology of Chemical Eye Injuries Etiology of Chemical Injury: Causative Agents Sequelae Classification Emergency Management Irrigation Aqueous Humor Replacement Acute Management Control of Inflammation Topical Corticosteroids Prevention of Stromal Breakdown Tetracyclines Ascorbate Citrate Promotion of Reepithelialization and Repair Amniotic Membrane Transplant Tenonplasty Flaps Preservative-Free Lubricants Biologic Fluids Preservative-Free Antibiotics Management of Ocular Exposure Management of Intraocular Pressure Antihypertensives Miscellaneous Cycloplegic Agents Management of Symblepharon Formation Emergency Surgery Tissue Glue and Tectonic Keratoplasty Chronic Phase Rehabilitation of the Ocular Surface Reconstruction of the Adnexa Management of Limbal Stem Cell Deficiency Unilateral Stem Cell Deficiency . In conjunctival limbal autograft (CLAU), a relatively large free conjunctival limbal lenticule is harvested from the donor eye... . Pellegrini et al. first described the technique of treating unilateral LSCD with an ex vivo autologous cultured limbal epithel... . Simple limbal epithelial transplantation (SLET) is a recently developed technique for the treatment of unilateral LSCD.45 Brie... Bilateral Limbal Deficiency . Bilateral limbal deficiency secondary to ocular chemical burns pose a unique set of challenges due to the lack of a source of ... . The risk of allograft rejection and the requirement for systemic immunosuppression in allogenic LSCT inspired the development ... Limbal Stem Cell Transplantation and Keratoplasty Keratoprosthesis Glaucoma Management Future Therapies Vascular Endothelial Growth Factor Inhibitors Stem Cell–Based Therapies Ocular Thermal Burns Pathophysiology Clinical Evaluation Clinical Management Conclusion Acknowledgments References 96 - Corneal Complications of Intraocular Surgery Epithelial Complications Thermal Burns Descemet Membrane Tear/Detachment Infectious Keratitis Endothelial Injury Incidence Pathogenesis Brown-Mclean Syndrome Toxic Anterior Segment Syndrome Specific Etiologic Factors in Aphakic Bullous Keratopathy and Pseudophakic Bullous Keratopathy Preoperative Factors: Preexisting Disease Intraoperative Factors Postoperative Causes of Corneal Edema Treatment Future Considerations References 97 - External Eye Manifestations of Biological and Chemical Warfare BACKGROUND MECHANISM OF ATTACK WARNING SIGNS BIOLOGICAL DISEASES Anthrax Microbiology/Epidemiology Clinical Manifestations Diagnosis Treatment Prevention Botulism Microbiology Clinical Manifestations Diagnosis Treatment Smallpox Microbiology Epidemiology Clinical Manifestations Diagnosis Treatment and vaccination Tularemia Microbiology and Epidemiology Clinical Manifestations Diagnosis Treatment Viral Hemorrhagic Fever Microbiology and Epidemiology Clinical Manifestations Diagnosis Treatment CHEMICAL AGENTS Nerve Agents Ophthalmic Manifestations Vesicants Clinical Manifestations Treatment REFERENCES SECTION 9 Contact Lenses 98 - Contact Lens Applications in Corneal Disease Introduction Soft Lens Innovations Hydrogel Lenses Silicone Hydrogel Lenses Rigid Lens Innovations Contact Lenses As Bandage Lenses Collagen Shields Therapeutic Uses for Contact Lenses Contact Lenses for Drug Delivery Irregular Cornea and Ectasia Conventional Soft Spherical and Soft Toric Lenses Keratoconus Design Specialty Soft Lenses Conventional RGP Lenses Keratoconus Design Specialty RGP Lenses Ultralarge and Intralimbal RGP Designs Hybrid Lenses Piggyback Lenses Mini-scleral Lenses, Scleral Lenses, and Prosthetic Replacement of the Ocular Surface Ecosystem Treatment Scleral Lens and Prosthetic Replacement of the Ocular Surface Ecosystem Treatment as Alternative to Keratoplasty Wavefront Customized Contact Lens Designs Contact Lens Correction for Vision Correction After Surgery Contact Lenses After Corneal Collagen Crosslinking for Keratoconus Contact Lens Correction After Corneal RING Segments Contact Lens Correction After Penetrating Keratoplasty Contact Lens Correction After Refractive Surgery Radial Keratotomy Photorefractive Keratectomy and Laser In Situ Keratomileusis Prosthetic Contact Lenses Tinted Contact Lenses Summary References 99 - Complications of Contact Lens Wear INTRODUCTION Patient History Abrasions Infiltrates Infections Fungal Infections Acanthamoeba Contact Lens-Induced Keratopathy Giant Papillary Conjunctivitis and Other Allergies Ocular Allergies Ptosis Dry Eyes Meibomian Gland Dysfunction Hypoxia Corneal Warpage Conclusion References PART VIII The Sclera and Anterior Uvea SECTION 1 Disorders of the Sclera 100 - Episcleritis ANATOMY Incidence Clinical Manifestations History Physical Examination Course Differential Diagnosis Pathology Imaging Angiography Complications Etiology Laboratory Evaluation Treatment References 101 - Scleritis Immune-Mediated Scleritis Epidemiology Pathogenesis and Risk Factors Clinical Findings Associated Systemic Diseases Complications Signs Aiding in Differential Diagnosis Laboratory Investigations Therapy Infectious Scleritis Clinical Findings Sclerokeratitis Panophthalmitis Scleritis After Scleral Buckling Surgery132,133 Post-pterygium Excision Scleritis Other Postsurgical Infectious Scleritis Herpetic Scleritis Syphilitic Scleritis Mycobacterium Tuberculosis Scleritis108,155–157 Signs Aiding in Differential Diagnosis Laboratory Investigations Therapy References SECTION 2 Anterior Uveitis 102 - Classification and Diagnosis of Anterior Uveitis DIAGNOSIS Symptoms of Anterior Uveitis History Demographic Information Past Medical History and Review of Systems Social and Family History Physical Examination External Pupils Sclera and Conjunctiva Cornea Anterior Chamber, Anterior Chamber Angle, and Iris Lens and Anterior Vitreous Intraocular Pressure Retina and Optic Nerve Classification Response to Therapy Diagnostic Evaluation Treatment Summary References 103 - Idiopathic Anterior Uveitis Classification of Uveitis History of Uveitis Ocular Examination Differential Diagnoses of Anterior Uveitis Laboratory Evaluation Treatment Of Anterior Uveitis Topical Corticosteroids Topical Cycloplegics Systemic Corticosteroids Systemic Immunosuppressive Drugs Biologic Agents Treatment Of Complications summary References 104 - HLA-B27–Associated Uveitis EPIDEMIOLOGY Clinical Features Etiology Hla-B27 and its Association With Spondyloarthropathies Treatment Complications and Prognosis References 105 - Sarcoidosis Ocular Manifestations Eyelids Lacrimal Gland Lacrimal Drainage System Orbit Conjunctiva Cornea Iris Anterior Uveitis Intermediate and Posterior Uveitis Optic Nerve Diagnosis Course and Management References 106 - Behçet Disease Systemic Manifestations of Behçet Disease Ocular Manifestations of Behçet Disease Anterior Segment Findings Posterior Segment Findings Conventional Therapy Biologic Therapy References 107 - Fuchs Uveitis Syndrome Clinical Features Etiology Differential Diagnosis Treatment Prognosis References 108 - Juvenile Idiopathic Arthritis-Associated Uveitis Epidemiology Clinical Features Ocular Clinical Features Etiology Treatment of Ocular Disease Screening Conclusion References PART IX Keratoplasty SECTION 1 Overview 109 - The Evolution of Contemporary Keratoplasty EARLY CONTRIBUTIONS TO CORNEAL TRANSPLANTATION Modern Techniques of Corneal Transplantation The Evolution From Penetrating Keratoplasty Back to Lamellar Keratoplasty Ultrathin Descemet Stripping Automated Endothelial Keratoplasty Nanothin Descemet Stripping Automated Endothelial Keratoplasty Deep Anterior Lamellar Keratoplasty Use of the Femtosecond Laser Ocular Surface Transplantation Conclusion References 110 - Preoperative Considerations and Decision-Making in Keratoplasty GENERAL CONSIDERATIONS Ocular Surface Reconstruction Procedures Anterior Lamellar Keratoplasty Procedures Indications Contraindications Advantages of Anterior Lamellar Keratoplasty Disadvantages of Anterior Lamellar Keratoplasty Surgical Techniques Posterior Lamellar Keratoplasty Procedures Indications Contraindications Advantages of Endothelial Keratoplasty Compared to Penetrating Keratoplasty Disadvantages of Endothelial Keratoplasty Surgical Technique Penetrating Keratoplasty Procedures Indications for Penetrating Keratoplasty Relative Contraindications for Penetrating Keratoplasty Advantages of Penetrating Keratoplasty Disadvantages of Penetrating Keratoplasty Surgical Techniques Permanent Keratoprosthesis Surgery Indications Relative Contraindications Advantages of Permanent Keratoprosthesis Surgery (KPro) Disadvantages of Permanent Keratoprosthesis (KPro) Surgical Technique Summary References SECTION 2 Penetrating Keratoplasty Procedures 111 - Penetrating Keratoplasty: The Fundamentals Introduction Factors in Patient Selection Age Mental Impairment Coexisting Ocular Disease Establish Realistic Expectations Surgical Setting and Anesthesia Preoperative Preparations Infection Control Intraocular Pressure Control Lens Management Donor Corneal Tissue Management Anticipated Suprachoroidal Hemorrhage Insertion of Lid Speculum Placement of Scleral Fixation Ring Marking of Host Cornea Trephination of Donor Cornea Trephination of Host Cornea Placement of Viscoelastic Material in the Anterior Chamber Placement of the Donor Corneal Tissue in the Host Bed Placement of Four Interrupted Radial 10-0 Nylon Cardinal Sutures Complete Suturing Surgical Tips on Wound Closure Readjustment of Sutures to Minimize Astigmatism Administering Medications Postoperative Suture Adjustment Special Circumstances The Postrefractive Surgery Patient Rotating Autograft Autologous Contralateral Autograft Maximizing Penetrating Keratoplasty Outcomes References 112 - Intraoperative Complications of Penetrating Keratoplasty Complications Related to Anesthesia Scleral Perforation with Fixation Sutures Complications Related to Trephination Improper Trephination Size Eccentric Trephination Damaged Donor Button Retained Descemet Membrane Iris–lens Damage Torn Posterior Capsule Expulsive Suprachoroidal Hemorrhage References 113 - Femtosecond-Assisted Penetrating Keratoplasty Introduction History Laboratory Research Laser Platforms Preoperative Evaluation Posterior Side Bridge Trephination Geometry Surgical Methodology Early Outcomes Deep Anterior Lamellar Keratoplasty Anterior Lamellar Keratoplasty Endothelial Keratoplasty References SECTION 3 Penetrating Keratoplasty: Postoperative Management 114 - Postoperative Management IMMEDIATE POSTOPERATIVE CARE First 24 Hours EARLY POSTOPERATIVE CARE One to Seven Days POSTOPERATIVE CARE One to Twelve Weeks Beyond 3 Months POSTOPERATIVE CARE IN INFANTS AND CHILDREN EARLY POSTOPERATIVE COMPLICATIONS WOUND LEAKS AND WOUND DISPLACEMENT PERSISTENT EPITHELIAL DEFECTS FILAMENTARY KERATITIS SUTURE-RELATED COMPLICATIONS Suture Exposure Suture-Related Infection Suture-Related Immune Infiltrates Kaye Dots ELEVATED INTRAOCULAR PRESSURE POSTOPERATIVE INFLAMMATION ANTERIOR SYNECHIA PUPILLARY BLOCK CHOROIDAL DETACHMENT AND CHOROIDAL HEMORRHAGE HYPHEMA FIXED DILATED PUPIL POSTOPERATIVE INFECTION PRIMARY GRAFT FAILURE EPITHELIAL DOWNGROWTH History Incidence and Etiology Pathogenesis Diagnosis Glaucoma and Epithelial Downgrowth Treatment FIBROUS INGROWTH Incidence and Etiology Pathogenesis Diagnosis Treatment DIFFERENTIAL DIAGNOSIS MANAGEMENT OF POSTKERATOPLASTY ASTIGMATISM PREOPERATIVE FACTORS INTRAOPERATIVE FACTORS Graft Diameter Tissue Alignment Trephination Factors Scleral Support Ring POSTOPERATIVE FACTORS Relaxing Incisions Astigmatic Keratotomy Wedge Resections Laser In Situ Keratomileusis Photorefractive Keratectomy CONCLUSION REFERENCES 115 - Diagnosis and Management of Corneal Allograft Rejection CLINICAL ASPECTS OF CORNEAL ALLOGRAFT REJECTION HISTORICAL PERSPECTIVE Recognition of Risk Factors Differential Diagnosis of Corneal Allograft Rejection Treatment of Corneal Allograft Rejection Prevention of Corneal Allograft Rejection Management Strategy References 116 - Infections After Penetrating Keratoplasty MICROBIAL KERATITIS Pathogenesis Pathogens Clinical Approach Therapeutic Approach Outcomes Suture Abscess Pathogenesis Clinical Approach Therapeutic Approach Infectious Crystalline Keratopathy Historical Background Pathogenesis Clinical Approach Therapeutic Approach Outcomes Endophthalmitis Incidence Pathogenesis Pathogens Clinical Approach Therapeutic Approach Outcomes Factors Associated With Recurrence Herpes Simplex Virus Fungal Acanthamoeba Newly Acquired Herpetic Keratitis After Keratoplasty Transmission of Unusual Infections Rabies Creutzfeldt-Jakob Disease Hepatitis Virus Human Immunodeficiency Virus Conclusion References 117 - Glaucoma After Corneal Transplantation Incidence Risk Factors The Prekeratoplasty Evaluation Clinical Presentation Glaucoma and Graft Failure Mechanisms for Glaucoma Post Penetrating Keratoplasty Deep Anterior Lamellar Keratoplasty Endothelial Keratoplasty Keratoprosthesis Management of Postkeratoplasty Glaucoma Medical Management Surgical Management Laser Iridoplasty and Trabeculoplasty . The adjunctive use of mitomycin C (MMC) during trabeculectomy to suppress episcleral fibrosis appears to increase the success ... . Many keratoplasty patients have comorbid conditions that preclude trabeculectomy. For example, chemical injury, ocular surface... . Comorbidities, including the presence of cicatricial disease or conjunctival scarring from prior procedures, may make some ker... Summary References SECTION 4 Anterior Lamellar Keratoplasty 118 - Indications for Anterior Lamellar Keratoplasty Introduction Epikeratophakia Anterior Lamellar Keratoplasty Superficial Anterior Lamellar Keratoplasty Deep Anterior Lamellar Keratoplasty Deep Anterior Lamellar Keratoplasty Indications Keratoconus Keratoglobus Pellucid Marginal Degeneration Herpes Simplex Virus-Related Scar Leukoma Resulting From Thermal/Chemical Injury Corneal Perforating Wounds References 119 - Historical Development of Anterior Lamellar Keratoplasty References 120 - Techniques of Anterior Lamellar Keratoplasty Introduction Surgical Anatomy of the Corneal Stroma Descemetic DALK and Predescemetic DALK Classification Pre-Descemet Layer, Big Bubble Type 1, Type 2, and Type 3 Clinical and Surgical Implications New Acronyms: Deep Anterior Lamellar Keratoplasty, Subtotal Anterior Lamellar Keratoplasty, and Total Anterior Lamellar Keratopl... Dalk Surgical Techniques Most Prevalent Techniques Peeling Off27,28 Layer-By-Layer Manual Dissection29 Hydrodissection32 Viscoelastic Dissection11,12,33 Air Big Bubble13 ?Bubble Test (See Video 120.6)34 New Opening of the Bubble35,36 ?Cannula Big Bubble (See Video 120.6)37,38 Air-Visco Bubble Technique7,37–39 Donor Preparation Suturing Techniques New Devices References 121 - Intraoperative and Postoperative Complications of Anterior Lamellar Keratoplasty INDICATIONS FOR ANTERIOR LAMELLAR KERATOPLASTY SURGICAL INSTRUMENTS TO PREVENT INTRAOPERATIVE COMPLICATIONS Intraoperative Complications Perforations and Ruptures of Descemet Membrane Trephination Stromal Dissection Suture Needles Management of Descemet Membrane Ruptures Postoperative Complications Pseudo-Anterior Chambers Pupillary Block and Fixed Dilated Pupil (Urrets-Zavalia Syndrome) Sclerocorneal Inflammation Stromal Rejection Postoperative Astigmatism References 122 - Recovery Techniques in Deep Anterior Lamellar Keratoplasty INTRODUCTION Standard Techniques And Learning Curves Repeated Air Injections Air-Visco Bubble Technique Manual Dissection (See Videos 120.2 and 120.3) Avoidance of Descemet Membrane Ruptures Cannula Versus Needle Big Bubble Bubble Test (See Video 120.6) New Opening of the Bubble Repair of Descemet Membrane Ruptures Descemet Membrane Ruptures in Total Anterior Lamellar Keratoplasty Descemet Membrane Ruptures in Subtotal Anterior Lamellar Keratoplasty (Video 122.1) Descemet Membrane Ruptures in Deep Anterior Lamellar Keratoplasty Excessive trephination and perforation Postoperative Traumatic Descemet Membrane Disinsertion (Video 122.5) References 123 - Outcomes of Anterior Lamellar Keratoplasty INTRODUCTION Visual Outcomes Refractive Outcomes Graft Survival Resistance to Trauma Surgical Planning Summary References SECTION 5 Complex Keratoplasty 124 - Pediatric Keratoplasty INTRODUCTION Corneal Grafts and the Pediatric Patient Amblyopia and Neural Plasticity Infant and Toddler Development Emmetropization Indications Preoperative Evaluation Ocular Management Options Pediatric Keratoplasty Counseling Parents and Grandparents Scheduling Surgery Operative Technique Anesthesia Penetrating Keratoplasty Rotational Penetrating Autokeratoplasty Deep Anterior Lamellar Keratoplasty Endothelial Keratoplasty Selective Endothelial Removal Concomitant Procedures Postoperative Evaluation Early Postoperative Complications Suture Removal Graft Rejection Glaucoma Strabismus Optical Correction and Amblyopia Therapy Graft Survival Risk Factors for Failure Visual Outcome The Future References 125 - Keratoplasty in Herpes Simplex Infection INDICATIONS AND TIMING FOR CORNEAL GRAFTING PREOPERATIVE MEASURES Control of Inflammation Vascularization Corneal Sensation Antiviral Prophylaxis Donor Tissue Requirements Operative Technique Graft Size Perforated Eyes Suture The Triple Procedure Anterior Lamellar Keratoplasty Femtosecond Laser Boston Keratoprosthesis Postoperative Management Use of Corticosteroids Suture Removal Persistent Epithelial Defects Recurrence of Herpes Simplex Virus Keratitis Allograft Rejection Secondary Graft Failure Glaucoma Wound Dehiscence Secondary Infections Postkeratoplasty Refractive Errors References 126 - Immunologically High-Risk Penetrating Keratoplasty and Large-Diameter Corneal Grafts INTRODUCTION Immunology of Graft Rejection Current View of the Immune Response Histocompatibility Antigens Immune Status of the Cornea Allograft Rejection RISK FACTORS FOR IMMUNEREJECTION Corneal Vascularization Prior Graft Rejection and Graft Loss Graft Diameter and Eccentric Grafts Anterior Synechiae Previous Intraocular Surgery Herpes Simplex and Herpes Zoster Keratitis History of Anterior Segment Inflammatory Disease Ocular Surface Disease Young Age Glaucoma Preoperative Considerations Controlling Ocular Inflammation Rehabilitating the Ocular Surface Operative Considerations Tissue Matching Surgical Technique Postoperative Considerations Immunosuppression Corticosteroids Calcineurin Inhibitors Antimetabolites Monoclonal Antibodies Blocking the Activation and Action of T-Cells Monoclonal Antibodies to T-cell Antigens Co-stimulatory Blockade Inhibiting the Antigen-Presenting Cells Inhibiting Immune Access to the Graft Reduction of Corneal Neovascularization References SECTION 6 Endothelial Keratoplasty 127 - Historical Development of Endothelial Keratoplasty INTRODUCTION Early Posterior Lamellar Keratoplasty THE FLAP TECHNIQUE OF POSTERIOR LAMELLAR KERATOPLASTY THE LIMBAL APPROACH TO ENDOTHELIAL REPLACEMENT Deep Lamellar Endothelial Keratoplasty Advances in Eye Bank Tissue Preparation References 128 - Indications and Decision-Making for Endothelial Keratoplasty Indications for Endothelial Keratoplasty Advantages of Endothelial Keratoplasty Patient Selection Vision Age Duration of Corneal Edema Lens Considerations Considerations in Complex Eyes Conclusion References 129 - Eye Bank Preparation of Tissue for Endothelial Keratoplasty INTRODUCTION Issues to Consider Techniques Summary References 130 - Surgical Techniques for Descemet Stripping Automated Endothelial Keratoplasty INTRAOPERATIVE CONSIDERATIONS Wound Creation Recipient Preparation Techniques to Improve Donor Adherence Donor Insertion Techniques Donor Apposition Techniques Early Postoperative Management Summary References 131 - Ultrathin Descemet Membrane Endothelial Keratoplasty INTRODUCTION Indications Preoperative Planning Surgical Technique Donor Preparation Recipient Preparation and Graft Delivery Postoperative Care Results Visual Outcomes Refractive Outcomes Endothelial Cell Density Graft Thickness Graft Survival Complications Conclusion References 132 - Nanothin Descemet Stripping Automated Endothelial Keratoplasty Introduction ULTRATHIN DESCEMET STRIPPING AUTOMATED ENDOTHELIAL KERATOPLASTY AND ITS OUTCOMES NANOTHIN DESCEMET STRIPPING AUTOMATED ENDOTHELIAL KERATOPLASTY NANOTHIN DESCEMET STRIPPING AUTOMATED ENDOTHELIAL KERATOPLASTY OUTCOMES ADVANTAGES OF NANOTHIN DESCEMET STRIPPING AUTOMATED ENDOTHELIAL KERATOPLASTY References 133 - Intraoperative and Postoperative Complications of Descemet Stripping Automated Endothelial Keratoplasty COMPLICATIONS OF DONOR TISSUE PREPARATION Intraoperative Complications Donor Lenticule Problems Air Management Bleeding Lens or Lens Implant Problems Postoperative Complications Graft Complications Air-Related Complications Interface Problems Infectious Complications Iatrogenic Glaucoma Persistent Corneal Edema References 134 - Outcomes of Descemet Stripping Automated Endothelial Keratoplasty INTRODUCTION Results Descemet Stripping Automated Endothelial Keratoplasty Complications Visual Recovery After Descemet Stripping Automated Endothelial Keratoplasty Astigmatism Spherical Equivalent Donor Endothelial Survival Summary References 135 - Surgical Technique for Descemet Membrane Endothelial Keratoplasty Introduction Injectors Recipient Preparation Wound Creation Endothelium–Descemet Membrane Resection Pupillary Modulation Peripheral Iridotomy Graft Orientation Orientation Before Insertion Orientation After Insertion Changing Orientation After Insertion Unscrolling and Centering Fluid-Assisted Unscrolling Bubble-Assisted Unscrolling Chamber-Assisted Unscrolling Centering and Centration Sulfur Hexafluoride Versus Room Air References 136 - Intraoperative and Postoperative Complications of Descemet Membrane Endothelial Keratoplasty INTRODUCTION Intraoperative Complications Donor Tissue Damage During Graft Preparation Poor Visualization of Donor Tissue Difficulty Handling and Unfolding Donor Tissue Upside-Down Donor Graft Other Complications Postoperative Complications Donor Dislocation Primary Graft Failure Graft Rejection Pupillary Block Glaucoma Endothelial Cell Loss Other Complications Conclusion References 137 - Outcomes of Descemet Membrane Endothelial Keratoplasty Introduction Descemet Membrane Endothelial Keratoplasty After Vitrectomy VISUAL FUNCTION AFTER DESCEMET MEMBRANE ENDOTHELIAL KERATOPLASTY Visual Acuity Hyperopic Shift MORPHOLOGY AFTER DESCEMET MEMBRANE ENDOTHELIAL KERATOPLASTY Restoration of the Normal Anatomy Morphology and Optical Properties of the Anterior Corneal Surface Optical Properties of the Posterior Corneal Surface Morphology and Function of the Corneal Endothelium DESCEMET MEMBRANE ENDOTHELIAL KERATOPLASTY OUTCOMES: PREOPERATIVE CONSIDERATIONS Graft Preparation Donor Tissue Selection Donor Preparation Surgeon Prepared Descemet Membrane Endothelial Keratoplasty Tissue Descemet Membrane Endothelial Keratoplasty With Precut Tissue Descemet Membrane Endothelial Keratoplasty With Preloaded Tissue Donor Insertion and Manipulation Descemetorhexis Sizing DESCEMET MEMBRANE ENDOTHELIAL KERATOPLASTY OUTCOMES: PERIOPERATIVE CONSIDERATIONS Descemetorhexis Sizing Air Versus Gas Tamponade POSTOPERATIVE MANAGEMENT AND COMPLICATIONS Incomplete Graft Adhesion: Rebubbling Pupillary Block Cystoid Macular Edema Cataract Progression Graft Failure Graft Rejection Steroid-Induced Secondary Glaucoma After Descemet Membrane Endothelial Keratoplasty DESCEMET MEMBRANE ENDOTHELIAL KERATOPLASTY OUTCOMES: COMPARISON WITH OTHER TECHNIQUES OF POSTERIOR LAMELLAR SURGERY Ultrathin (UT)-DSAEK Versus DMEK DMEK Versus Nanothin (NT)- DSAEK DESCEMET MEMBRANE ENDOTHELIAL KERATOPLASTY IN SPECIAL SITUATIONS Descemet Membrane Endothelial Keratoplasty as a Combined Procedure Phakic Descemet Membrane Endothelial Keratoplasty DMEK Under Failed PK Repeat DMEK After Failed DMEK DMEK After Failed DSAEK Descemet Membrane Endothelial Keratoplasty in Complex Eyes (Posttrabeculectomy/Filtering Tubes Postvitrectomy) Descemet Membrane Endothelial Keratoplasty After Glaucoma Filtration Surgery References 138 - Complex Endothelial Keratoplasty INTRODUCTION Lens Abnormalities Aphakia Dislocated Intraocular Lens Anterior Chamber Abnormalities Excessively Shallow or Crowded Excessively Deep Anterior Chamber Iris Abnormalities Pupillary Abnormalities and Large Iris Defects Traumatic Aniridia Irido-Corneal Endothelial Syndrome Previous Glaucoma Surgery Previous Failed Graft Penetrating Keratoplasty Failed Descemet Stripping Endothelial Keratoplasty Failed Descemet Membrane Endothelial Keratoplasty Summary References 139 - Cultivated Corneal Endothelial Cell Transplantation Concept Corneal Endothelial Cell Biology Cultured Human Corneal Endothelial Cells-Sheet Transplantation Cultured Human Corneal Endothelial Cells-Injection Therapy Topical Treatment of Corneal Endothelial Disease References PART X Therapeutic Procedures SECTION 1 Corneal Surgery 140 - Management of Corneal Perforations Etiology Terminology Preoperative Management Treatment Options Tissue Adhesives Cyanoacrylate Glue Method of Application Postoperative Management Complications Penetrating Keratoplasty Patch Graft Amniotic Membrane Transplantation Medical and Nonconventional Management Prevention of Corneal Perforation Bandage Soft Contact Lens Conjunctival Flap Tarsorrhaphy Amniotic Membrane Transplantation Miscellaneous Conclusion References 141 - Therapeutic Keratoplasty THERAPEUTIC KERATOPLASTY FOR INFECTIOUS KERATITIS Therapeutic Keratoplasty for Bacterial Infections Therapeutic Keratoplasty for Fungal Keratitis Therapeutic Keratoplasty for Acanthamoeba Keratitis Therapeutic Keratoplasty for Viral Keratitis Presurgical Evaluation Antimicrobial Therapy Donor Material Surgical Technique Therapeutic Penetrating Keratoplasty Therapeutic Lamellar Keratoplasty References 142 - Surgical Management of Superficial Corneal and Conjunctival Disease INTRODUCTION Superficial Corneal Diseases Recurrent Corneal Erosions Anterior Stromal Micropuncture Epithelial Debridement (Superficial Keratectomy,Diamond Burr, PTK) Band Keratopathy Partial Limbal Stem Cell Deficiency Salzmann Nodular Degeneration Superficial Conjunctival Diseases Superior Limbic Keratoconjunctivitis Conjunctivochalasis Molluscum Contagiosum Conclusion References 143 - Excimer Laser Phototherapeutic Keratectomy INTRODUCTION INDICATIONS FOR PHOTOTHERAPEUTIC KERATECTOMY Contraindications Preoperative Evaluation Ophthalmic History Medical History Examination Ancillary Testing Patient Selection Techniques General Smooth Opacities Rough or Elevated Opacities Deep Irregular Opacities Recurrent Erosions Antihyperopia Treatment Masking Agents Mitomycin C Postoperative Management Results Side Effects and Complications Future Directions Summary References SECTION 2 Conjunctival Surgery 144 - Management of Primary Pterygium INTRODUCTION DEFINITION AND MORPHOLOGY EPIDEMIOLOGY OF PTERYGIUM Risk Factors Environmental Factors Genetic Factors PATHOGENESIS OF PTERYGIUM Degenerative Versus Proliferative Disorder Limbal Stem Cell Deficiency and Epithelial Abnormalities in Pterygium THERAPEUTIC OPTIONS FOR PTERYGIUM Bare Sclera Excision Excision With Conjunctival Closure/Transposition Bare Scleral Excision With Adjunctive Medical Therapies Ocular Surface Transplantation Techniques Conjunctival Autografting Other Adjuvants in Pterygium Management Tissue Adhesives in Pterygium Surgery Surgical Technique of Pterygium Excision and Conjunctival Autografting Conjunctival-limbal Autograft Transplantation COMPLICATIONS OF CONJUNCTIVAL AUTOGRAFTING Causes of Recurrence After Conjunctival Autografting Amniotic Membrane Transplantation Surgical Technique of Amniotic Membrane Transplantation Lamellar Keratoplasty CONCLUSION REFERENCES 145 - Management of Recurrent Pterygium Introduction Aims and Definitions Risk Factors for Pterygium Recurrence Dry Eye Syndrome Timing of Surgery Surgical Technique Previous Surgical Procedures Other Considerations Medial Rectus Author’s Technique Femtosecond Laser-Assisted Pterygium Surgery FURTHER Considerations Conclusion References 146 - Conjunctival Flaps INTRODUCTION Indications Persistent Corneal Epithelial Defect Unresponsive Ulcerative Microbial Keratitis Corneal Thinning and Perforation Corneal Limbal Disease Scleral Necrosis Glaucoma Surgery Complications Surface Preparation for a Cosmetic Scleral Shell Lacrimal Punctal Occlusion Disadvantages Surgical Techniques Total Conjunctival Flap Removal of Corneal Epithelium Mobilization of the Conjunctival Flap Conjunctival Flap Suture Bipedicle Bridge Flap Single Pedicle Flap Advancement Flap Complications Intraoperative Complications Buttonhole Formation Dissection of an Inadequate Flap Excessive Hemorrhage Postoperative Complications Retraction of the Flap Ptosis Cystic Flap Opacification and Vascularization References 147 - Indications for and Uses of Amniotic Membrane: Clinic THE AMNIOTIC MEMBRANE: HISTOLOGY, BIOLOGIC PROPERTIES, AND CLINICAL EFFECTS Preserving The Amniotic Membrane Sutureless, Cryopreserved Amniotic Membrane Dehydrated Amniotic Membrane Amniotic Membrane as a Patch in Corneal Surface Disease Amniotic Membrane and Limbal Stem Cell Deficiency Complications References 148 - Indications for and Uses of Amniotic Membrane: Operating Room THE AMNIOTIC MEMBRANE: HISTOLOGY, BIOLOGIC PROPERTIES, AND CLINICAL EFFECTS PRESERVATION METHODS OF AMNIOTIC MEMBRANE: TISSUE BANK TECHNIQUES FOR AMNIOTIC MEMBRANE IMPLANTATION IN THE OPERATING ROOM (VIDEO 148.1) AMNIOTIC MEMBRANE TRANSPLANTATION IN OCULAR SURFACE DISEASES ?Amniotic Membrane Grafts in Conjunctival Reconstruction (Video 148.2) ?Amniotic Membrane Grafts in Corneal Surface Reconstruction (See Video 148.2) ?Amniotic Membrane Patch in Corneal Surface Reconstruction (See Video 148.2) ?Amniotic Membrane and Limbal Stem Cell Deficiency (See Videos 148.2 and 148.7) Amniotic Membrane as a Substrate for Culturing Epithelial Stem Cell of the Sclero-corneal Limbus Complications References SECTION 3 Anterior Segment Surgery 149 - Surgical Management and Rehabilitation of Anterior Segment Trauma Patient Evaluation History Examination Slit Lamp Examination Imaging Tissue Adhesive Acute Surgical Management Anesthesia and Preparation of the Eye Surgical Timing and Goals Corneal Laceration Repair . In closing a jagged incision, each linear aspect of the incision is closed individually. It is important to avoid closing the ... . A variation of the mattress suture technique has been suggested by Eisner to close a stellate laceration (Fig. 149.12). A purs... . In the face of tissue loss that cannot be sealed with tissue glue, a lamellar corneal autograft may be used (Fig. 149.13).49–5... Corneoscleral Lacerations Iris Trauma Iridodialysis Traumatic Iris Defects Lens Trauma, Dislocation Anterior Segment Intraocular Foreign Bodies Postoperative Care Suture Removal Postoperative Complications Visual Rehabilitation Conclusion References 150 - Iris Reconstruction Surgery Iris Suture Techniques Sliding Knot Intracameral Suture Pupil Cerclage Procedure Iridodialysis Repair Iris Relaxing Incisions Iris Oversewing of Transillumination Defects Scissors Sculpting Vitrector Sculpting Iris Cautery Sculpting Iris Prostheses Large-incision, Rigid Diaphragm Devices Rigid Small-incision Devices Flexible Small-incision Iris Prostheses Pupillary Aperture: Pupil Versus Entrance Pupil Complications Summary References 151 - Management of Scleral Perforation GENERAL MANAGEMENT STRATEGIES Anesthesia for Scleral Perforations Surgical Repair of Scleral Perforations Preoperative Considerations Operative Considerations Conjunctiva and Tenon Capsule Tarsoconjunctival Flap Autologous Sclera Homologous Sclera Donor Cornea Fascia lata Periosteum Split-Thickness Dermal Graft Amniotic Membrane Other Materials Summary References PART XI Collagen Crosslinking 152 - Collagen Crosslinking for Keratoconus INTRODUCTION Mechanism of Action Indications Contraindications Surgical Procedure Dresden Protocol Alternative Crosslinking Techniques Transepithelial Crosslinking High Fluence Crosslinking Crosslinking Combined With Topography-Guided Photorefractive Keratectomy Crosslinking With Riboflavin Placed Through a Corneal Pocket Typical Postoperative Findings Cellular and Ultrastructural Findings After Crosslinking Biomechanical Changes Complications Clinical Outcomes Standard Crosslinking Transepithelial Collagen Crosslinking High Fluence Crosslinking Crosslinking Combined With Topography-Guided Photorefractive Keratectomy Future Directions Conclusion References 153 - Collagen Crosslinking for Postrefractive Ectasia INTRODUCTION Pathophysiology The Crosslinking Procedure Clinical Outcomes Visual Acuity Topographic Outcomes Postoperative Timecourse Biomechanical Effects of Corneal Collagen Crosslinking Complications Postoperative Haze Corneal Pachymetry Predictors of Outcomes Future of Corneal Collagen Crosslinking Financial Disclosure References 154 - Collagen Crosslinking for Infectious Keratitis BACTERIAL SUSCEPTIBILITY In Vitro Experimental Assays Fungal Susceptibility Candida albicans Fusarium solani In Vitro Experimental Essays In Vivo Experimental Studies Acanthamoeba Susceptibility In Vitro Experimental Essays In Vivo Experimental Studies PACK-Corneal Crosslinking Technique Irradiation Zone Special Conditions Safety Indications Outcomes Conclusion References PART XII Keratoprosthesis 155 - Indications for Keratoprosthesis Introduction Boston Keratoprosthesis Osteo-Odonto-Keratoprosthesis Other Keratoprosthesis Devices Moscow Eye Microsurgery Complex in Russia Keratoprosthesis KeraKlear, Pintucci, AlphaCor, Auro Keratoprosthesis Summary References 156 - Boston Keratoprosthesis Type 1 Surgical Technique Preoperative Evaluation Design Postoperative Management References 157 - Postoperative Management of Boston Keratoprosthesis Type 1 AVOIDANCE OF COMPLICATIONS Postoperative Patient Evaluation Postoperative Treatment Antibiotics Corticosteroids Glaucoma Management Soft Contact Lens Conclusion References 158 - Complications of Boston Keratoprosthesis Type 1 RETROPROSTHETIC MEMBRANE Glaucoma Endophthalmitis Posterior Segment Complications Infectious Keratitis Keratolysis or Corneal Melt Extrusion or Keratoprosthesis Loss References 159 - Outcomes of Boston Keratoprosthesis Type 1 Introduction Visual Acuity and Prosthesis Retention Postoperative Complications Retroprosthetic Membranes Glaucoma Infectious Keratitis Endophthalmitis Sterile Vitritis Retinal Detachment Corneal Melt Primary Implantation of Keratoprosthesis Conclusion References 160 - Boston Keratoprosthesis Type 2 Surgical Techniques, Complications, and Outcomes Background and Design Surgical Planning Surgical Technique Eyelid and Ocular Surface Preparation Assembly of the Boston Keratoprosthesis Type 2 Implantation of the Keratoprosthesis Closure of Eyelids Postoperative Care Outcomes and Complications Conclusion References 161 - Osteo-Odonto-Keratoprosthesis Introduction Preoperative Assessment Indications for Osteo-odonto-keratoprosthesis Preoperative Evaluation Surgical Technique Results and Complications Visual Results and Stability Complications Conclusion References 162 - Contemporary Approaches to the Biosynthetic Cornea: Overview and Classification ALPHACOR Keraklear Tissue Engineering Future Directions Conclusion References PART XIII Ocular Surface Transplantation 163 - Classification and Staging of Ocular Surface Disease Introduction Classification of Ocular Surface Disease Eyelids and Eyelashes Tear Film Conjunctiva Limbal Stem Cell Deficiency Congenital Traumatic Autoimmune Disorders Staging of Ocular Surface Disease References 164 - Conjunctival Limbal Autograft 1 INTRODUCTION Indications and Patient Selection PREOPERATIVE EVALUATION AND OCULAR SURFACE OPTIMIZATION Surgical Procedure Recipient Eye (Fig. 164.1A–C, Video 164.1) Donor Eye (Fig. 164.2, Video 164.2) Placement of the Donor Grafts (see Fig. 164.1D–F) Postoperative Management Outcomes and Complications Donor Eyes Complications Surgical Alternatives Acknowledgment References 165 - Living-Related Conjunctival Limbal Allograft Introduction Indications/Contraindications Preoperative Considerations Surgical Procedure Harvesting the LR-CLAL Donor Segment (Figs. 165.1 and 165.2, Video 165.1 Preparation of the Recipient Eye (see Figs. 165.1 and 165.3, Video 165.2) Placement of the Donor Tissue Postoperative Management Rejection and Failure OCULAR SURFACE STABILITY AND VISUAL ACUITY OUTCOMES References 166 - Keratolimbal Allograft INDICATIONS AND CONTRAINDICATIONS Preoperative Considerations Surgical Technique Preparation of the Recipient Eye Donor Tissue Preparation Technique 1 Donor Tissue Preparation Technique 2 Postoperative Considerations References 167 - Cultivated Limbal Epithelial Transplant INTRODUCTION Stem Cell Niche History Origin of Cells Culture Process Surgical Procedure Clet in Children Postoperative Medication Follow Up Results Table (LSCD Review) Outcomes Complications Advantages and Disadvantages of Clet Future Directions of Clet References 168 - Simple Limbal Epithelial Transplantation Introduction Technique Technique Modifications Results Complications and Risk Factors for Failure Other Indications for Slet Conclusion References 169 - Postoperative Management of Ocular Surface Reconstruction INTRODUCTION PREOPERATIVE SCREENING AND DONOR SELECTION—THE CINCINNATI PROTOCOL Donor Selection Preoperative Screening Preventing Immune Rejection Overview Definition and Clinical Signs of Rejection Immunosuppressive Agents Immunosuppression Regimen Evaluation of Patients Prior to Starting Immunosuppression Glucocorticosteroids Mode of Action Topical Steroids Systemic Steroids Calcineurin Inhibitors Cyclosporine A Tacrolimus (FK-506) Antimetabolites Azathioprine Mycophenolate Mofetil Other Immunosuppressive Agents Sirolimus (Rapamycin) Biologic Drugs Prophylactic Antimicrobial Therapy Risk of Malignancy Ocular Surface Rehabilitation Postoperative Infectious Keratitis References 170 - Corneal Transplantation in Ocular Surface Disease INTRODUCTION Review of the Literature TREATMENT PARADIGM FOR KERATOPLASTY IN PATIENTS WITH SEVERE OCULAR SURFACE DISEASE Summary References 171 - Outcomes of Ocular Surface Transplantation INTRODUCTION Outcome Measures Autograft Outcomes Allogeneic Outcomes Keratoplasty Outcomes Rejection Outcomes Ocular Hypertension/Glaucoma Infectious Keratitis (Fig. 171.2) Conclusion References PART XIV Refractive Surgery SECTION 1 Preoperative Considerations 172 - Decision-Making in Refractive Surgery Excimer Laser Vision Correction Patient Age Refractive Error Corneal Thickness Corneal Topography Keratometry Pupil Size Thin-Flap Lasik Surface Ablation Small-Incision Lenticule Extraction Conductive Keratoplasty Arcuate Transverse Keratotomy Phakic Intraocular Lenses Intraocular Lens After Phacoemulsification Retreatments And Sequential Procedures Intracorneal Inlays Treatment of Presbyopia References 173 - Patient Evaluation and Selection in Refractive Surgery Introduction History Ocular Herpes Simplex Virus (HSV) Radial Keratotomy Strabismus Monocular Patients Keloid Formation Contact Lenses, Presbyopia, and Monovision Pregnancy Participation in High-Risk Activities Medicines Medical Conditions Examination Visual Acuity Refraction Computed Tomography Pupil Examination Slit Lamp Examination Fundus Examination Tonometry Pachymetry Anterior Chamber Depth Dry Eye Testing Ocular Dominance Determination Wavefront Testing Informed Consent References 174 - Topographic Analysis in Keratorefractive Surgery Introduction Background Keratometry Keratoscopy Videokeratoscopy Computer-Based Videokeratoscopes Color LED Ray Tracing Topography Elevation-Based Topography Systems Clinical Applications Contact Lens-Induced Warpage Keratoconus and Keratoconus Suspects Displaced Apex Syndrome Postoperative Evaluations Contoured Ablation Patterns Future Horizons References SECTION 2 Laser Vision Correction 175 - Excimer Laser Surface Treatment: Photorefractive Keratectomy Excimer Laser Surface Treatment Photorefractive Keratectomy Preoperative Medications Stromal Ablation Postoperative Management Medications and Pain Management Epithelial Healing Dry Eye Visual Recovery Results Myopic Photorefractive Keratectomy Hyperopic Photorefractive Keratectomy Ablation Profiles Wavefront-Optimized Photorefractive Keratectomy Wavefront-Guided Photorefractive Keratectomy Topography-Guided Photorefractive Keratectomy Epithelial Mapping Complications Haze Background and Clinical Presentation Risk Factors Haze Prophylaxis Treatment Irregular Astigmatism Decentered Ablation Central Island Ectatic Disorders Management of Irregular Astigmatism Delayed Epithelial Healing Corneal Infiltrates and Infectious Keratitis Regression of Treatment Effect Conclusion References 176 - LASIK Technique INTRODUCTION The Evolution of Lasik Advantages of the Femtosecond Laser Flap Creation Versus the Microkeratome Lasik Evaluation LASIK Versus Photorefractive Keratectomy Parameters for Treatment Contact Lens Discontinuation Protocol Medications Dry eyes Methicillin-Resistant Staphylococcus aureus (Donnenfeld) Protocol Other Ocular Conditions Preoperative Planning Wavefront-Adjusted Manifest Refraction Age Adjustments Coupling Monovision Corneal Thickness Flap Size Flap Shape Pocket Position Preoperative Preparation Sedation Triage Preoperative Ocular Medications Technique LASIK Psychology Care of the Corneal Epithelium Refractive Surgery Lasers for LASIK in the United States Calibration Single-Use Disposable Instruments Sterile Versus Clean Procedure Safety Checks Laser Positioning Eye Preparation Iris Registration Docking Soft Docking and Gas Escape Flap Position Refinement Loss of Suction During Flap Creation Gas Breakthrough Lifting the Flap Opaque Bubble Layer Laser Ablation Flap Replacement Flap Alignment Immediate Postoperative Care Lasik Results Lasik Enhancements The Future of Lasik LASIK Xtra ReLEx SMILE References 177 - LASIK for Myopia HISTORY Lasik Excimer Lasers Patient Selection, Indications, Limitations, and Contraindications LASIK Contraindications Microkeratomes and Femtosecond Lasers Operative Technique Postoperative Care Intraoperative Complications Complications Ablation Complications Keratectasia Results LASIK Enhancements Lasik in Complex Cases LASIK After Radial Keratotomy LASIK After Photorefractive Keratectomy LASIK After Penetrating Keratoplasty Lasik After Smile Lasik After Intraocular Lenses Intraocular Lens Calculations After Lasik Summary References 178 - LASIK for Hyperopia Introduction Physiologic Aspects of Hyperopia Corneal Surgery for Hyperopia Preoperative Considerations and Patient Selection Indications for Lasik for Hyperopia Primary Hyperopia Secondary Hyperopia Technique and Instrumentation for LASIK for Hyperopia Technique Results Complications Conclusion References 179 - LASIK Complications INTRODUCTION Intraoperative Lasik Complications Femtosecond Laser Flap Complications Suction Loss Clinical Management Vertical Gas Breakthrough Clinical Management Prevention Anterior Chamber Gas Bubbles Clinical Management Prevention Opaque Bubble Layer Clinical Management Prevention Microkeratome Complications Laser-Related Complications Clinical Management Prevention Early Postoperative Complications Flap Striae Clinical Management Prevention Dislodged Flap Clinical Management Prevention Infection Clinical Management Prevention Diffuse Lamellar Keratitis Clinical Management Prevention Transient Light Sensitivity Syndrome Rainbow Glare Clinical Management Prevention Late Postoperative Lasik Complications Epithelial Ingrowth Clinical Management Prevention Pressure-Induced Stromal Keratitis Clinical Management Prevention Errors in Refractive Outcome Clinical Management Prevention Postoperative Keratectasia Following LASIK Clinical Management Prevention Night-Vision Disturbances: Halos and Glare Clinical Management Prevention Decentration Clinical Management Prevention Irregular Astigmatism Clinical Management Prevention Post-LASIK Dry Eyes and Neuropathic Pain Management Prevention References 180 - Corneal Ectasia: Prevention, Detection, and Management Prevention Clinical Correlation Detection Topography Tomography Ocular Coherence Tomography Wavefront Aberrometry Corneal Hysteresis Management Contact Lenses Intracorneal Ring Segments Corneal Collagen Crosslinking Clinical Correlation Corneal Transplantation References 181 - Small-Incision Lenticule Extraction Introduction Basic Science Biomechanical Stability Corneal Denervation Wound Healing and Inflammatory Reactions Clinical Assessments and Outcomes Efficacy, Predictability, Stability, and Safety Higher Order Aberrations Contrast Sensitivity Corneal Sensation Visual Quality Complications Intraoperative Complications Postoperative Complications Conclusions and Future Prospects References SECTION 3 Additional Refractive Procedures 182 - Topography-Guided Photorefractive Keratectomy Keratoconus, Pellucid Marginal Degeneration, Or Ectasia After Radial Keratotomy After Lasik, PRK, or Smile Other Conditions Caution In Interpreting Topographic Maps Summary References 183 - Intrastromal Corneal Ring Segments A BRIEF HISTORY OF THE TECHNIQUE Mechanism of Action Market Availability Indications and Contraindications Indications Contraindications Surgical Planning Surgical Procedure Manual Dissection Femtosecond Laser-Assisted Dissection Intrastromal Segments and Combined Techniques Complications References 184 - Combined Corneal Refractive Techniques Corneal Crosslinking in Combination With a Nonexcimer Laser Ablation Technique Corneal Crosslinking and Intrastromal Corneal Rings Corneal Crosslinking and Phakic Intraocular Lens Intraocular Lenses and Corneal Relaxing Incision Excimer Laser Ablation and Corneal Crosslinking Photorefractive Keratectomy and Corneal Crosslinking LASIK and Corneal Crosslinking Excimer Laser Ablation and Intraocular Lenses LASIK/Photorefractive Keratectomy and Phakic Intraocular Lenses LASIK/Photorefractive Keratectomy and Pseudophakic Intraocular Lenses Excimer Laser Ablation and Small-Incision Lenticule Extraction LASIK/Photorefractive Keratectomy and SMILE Triple Procedure Intrastromal Corneal Rings, Corneal Crosslinking, and Phakic Intraocular Lenses Intrastromal Corneal Rings, PhotorefractiveKeratectomy, and Corneal Crosslinking Intrastromal Corneal Rings, Corneal Crosslinking, and Toric Phakic Intraocular Lenses Four-Stage Procedure Intrastromal Corneal Rings, Corneal Crosslinking, Phakic Intraocular Lenses, and Photorefractive Keratectomy References 185 - Incisional Keratotomy Introduction Patient Selection Preoperative Planning Manual Peripheral Corneal Relaxing Incision Equipment Manual Peripheral Corneal Relaxing Incision Results Femtosecond Corneal Relaxing Incisions Femtosecond Laser Corneal Relaxing Incisions Nomograms Femtosecond Laser Corneal Relaxing Incisions Equipment and Surgical Techniques Femtosecond Laser Corneal Relaxing Incisions Results Femtosecond Laser Anterior Penetrating Corneal Relaxing Incisions Femtosecond Laser Intrastromal Corneal Relaxing Incisions Femtosecond Corneal Relaxing Incisions Complications Overcorrection Visual Loss Anterior Gas Breakthrough Suction Loss Alignment Manual Alignment Imaging and Guidance System Alignment Intraoperative Wavefront Aberrometry Alignment Post-Penetrating Keratoplasty Astigmatism After PRK or LASIK Summary References 186 - Phakic Intraocular Lenses INTRODUCTION ANGLE-SUPPORTED ANTERIOR CHAMBER PHAKIC INTRAOCULAR LENSES ANTERIOR CHAMBER IRIS-FIXATED PHAKIC INTRAOCULAR LENSES Posterior Chamber Phakic Intraocular Lenses Bioptics Acknowledgment References 187 - Corneal Inlay Surgery for Presbyopia: Techniques and Complications INTRODUCTION Small-Aperture Inlays: Lessons Learned Myopia Ocular Surface Deep Implantation Surgical Techniques Postoperative Management Conclusion Acknowledgments References Index