- •Contents
- •General Introduction
- •Objectives
- •Anatomy
- •Eyelids
- •Conjunctiva
- •Lacrimal Functional Unit
- •The Tear Film
- •Cornea
- •Sclera
- •2 Examination Techniques for the External Eye and Cornea
- •Evaluation of Vision in the Patient With an Abnormal Cornea
- •External Examination
- •Slit-Lamp Biomicroscopy
- •Direct Illumination Methods
- •Indirect Illumination Methods
- •Clinical Use
- •Stains
- •Fluorescein
- •Rose Bengal and Lissamine Green
- •Evaluation of the Tear Film and Tests of Tear Production
- •Tear Composition Assays
- •Imaging Technologies
- •Impression Cytology
- •Corneal Pachymetry
- •Measurement of Corneal Biomechanics
- •Measurement of Corneal Curvature
- •Zones of the Cornea
- •Shape, Curvature, and Power
- •Keratometry
- •Computerized Corneal Topography
- •Corneal Tomography
- •Indications
- •Ultrasound Biomicroscopy
- •Anterior Segment Optical Coherence Tomography
- •Confocal Microscopy
- •External and Slit-Lamp Photography
- •Specular Microscopy
- •Anterior Segment Fluorescein Angiography
- •Esthesiometry
- •Retinoscopy
- •3 Clinical Approach to Ocular Surface Disorders
- •Common Clinical Findings
- •Conjunctival Signs
- •Corneal Signs
- •Clinical Approach to Dry Eye
- •Mechanisms of Dry Eye
- •Aqueous Tear Deficiency
- •Evaporative Dry Eye
- •Treatment of Dry Eye
- •Rosacea
- •Seborrheic Blepharitis
- •Staphylococcal Blepharitis
- •Hordeola and Chalazia
- •Exposure Keratopathy
- •Floppy Eyelid Syndrome
- •Superior Limbic Keratoconjunctivitis
- •Recurrent Corneal Erosion
- •Neurotrophic Keratopathy and Persistent Corneal Epithelial Defects
- •Trichiasis and Distichiasis
- •Factitious Ocular Surface Disorders
- •Dellen
- •Limbal Stem Cell Deficiency
- •Sjögren Syndrome
- •Ichthyosis
- •Ectodermal Dysplasia
- •Xeroderma Pigmentosum
- •Vitamin A Deficiency
- •4 Infectious Diseases of the External Eye: Basic Concepts and Viral Infections
- •Defense Mechanisms of the External Eye
- •Normal Ocular Flora
- •Pathogenesis of Ocular Infections
- •Virulence
- •Inoculum
- •Host Defense
- •Ocular Microbiology
- •Diagnostic Laboratory Techniques
- •Specimen Collection
- •Staining Methods
- •Virology and Viral Infections
- •DNA Viruses: Herpesviruses
- •Herpes Simplex Eye Diseases
- •Varicella-Zoster Virus Dermatoblepharitis, Conjunctivitis, and Keratitis
- •Epstein-Barr Virus Dacryoadenitis, Conjunctivitis, and Keratitis
- •Cytomegalovirus Keratitis and Anterior Uveitis
- •DNA Viruses: Adenoviruses
- •DNA Viruses: Poxviruses
- •Molluscum Contagiosum
- •Vaccinia
- •DNA Viruses: Papovaviruses
- •RNA Viruses
- •Bacteriology
- •Gram-positive Cocci
- •Gram-negative Cocci
- •Gram-positive Rods
- •Gram-negative Rods
- •Gram-positive Filaments
- •Chlamydia Species
- •Spirochetes
- •Mycology
- •Yeasts
- •Septate Filamentous Fungi
- •Nonseptate Filamentous Fungi
- •Parasitology
- •Protozoa
- •Helminths
- •Arthropods
- •Prions
- •Staphylococcal Blepharitis
- •Fungal and Parasitic Infections of the Eyelid Margin
- •Bacterial Conjunctivitis in Children and Adults
- •Parinaud Oculoglandular Syndrome
- •Microbial and Parasitic Infections of the Cornea and Sclera
- •Contact Lens–Related Infectious Keratitis
- •Bacterial Keratitis
- •Atypical Mycobacteria
- •Fungal Keratitis
- •Acanthamoeba Keratitis
- •Corneal Stromal Inflammation Associated With Systemic Infections
- •Microsporidiosis
- •Loiasis
- •Microbial Scleritis
- •6 Ocular Immunology
- •Overview of the Ocular Surface Immune Response
- •Tear Film
- •Immunoregulation of the Ocular Surface
- •Angiogenesis and Lymphangiogenesis in the Cornea
- •Tissue-Specific Patterns of Immune-Mediated Ocular Disease
- •Conjunctiva
- •Cornea
- •Sclera
- •Diagnostic Approach to Immune-Mediated Ocular Disorders
- •Immune-Mediated Diseases of the Eyelid
- •Contact Dermatoblepharitis
- •Atopic Dermatitis
- •Immune-Mediated Disorders of the Conjunctiva
- •Hay Fever Conjunctivitis and Perennial Allergic Conjunctivitis
- •Vernal Keratoconjunctivitis
- •Atopic Keratoconjunctivitis
- •Ligneous Conjunctivitis
- •Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
- •Mucous Membrane Pemphigoid
- •Ocular Graft-vs-Host Disease
- •Other Immune-Mediated Diseases of the Skin and Mucous Membranes
- •Immune-Mediated Diseases of the Cornea
- •Thygeson Superficial Punctate Keratitis
- •Interstitial Keratitis Associated With Infectious Diseases
- •Reactive Arthritis
- •Cogan Syndrome
- •Marginal Corneal Infiltrates Associated With Blepharoconjunctivitis
- •Peripheral Ulcerative Keratitis Associated With Systemic Immune-Mediated Diseases
- •Mooren Ulcer
- •Corneal Transplant Rejection
- •Immune-Mediated Diseases of the Episclera and Sclera
- •Episcleritis
- •Scleritis
- •8 Clinical Approach to Neoplastic Disorders of the Conjunctiva and Cornea
- •Approach to the Patient With a Neoplastic Conjunctival Lesion
- •Management of Patients With Conjunctival Tumors
- •Surgical Treatment
- •Topical Chemotherapy
- •Tumors of Epithelial Origin
- •Benign Epithelial Tumors
- •Preinvasive Epithelial Lesions
- •Malignant Epithelial Lesions
- •Management of Atypical Epithelial Tumors
- •Other Malignant Epithelial Lesions
- •Glandular Tumors of the Conjunctiva
- •Oncocytoma
- •Sebaceous Gland Carcinoma
- •Tumors of Neuroectodermal Origin
- •Benign Pigmented Lesions
- •Preinvasive Pigmented Lesions
- •Malignant Pigmented Lesions
- •Neurogenic and Smooth-Muscle Tumors
- •Vascular and Mesenchymal Tumors
- •Benign Tumors
- •Malignant Tumors
- •Lymphatic and Lymphocytic Tumors
- •Lymphangiectasia and Lymphangioma
- •Lymphoid Hyperplasia
- •Lymphoma
- •Metastatic Tumors
- •9 Basic and Clinical Concepts of Congenital Anomalies of the Cornea, Sclera, and Globe
- •Developmental Anomalies of the Globe and Sclera
- •Cryptophthalmos
- •Microphthalmos
- •Nanophthalmos
- •Blue Sclera
- •Developmental Anomalies of the Anterior Segment
- •Anomalies of Size and Shape of the Cornea
- •Abnormalities of Corneal Structure and/or Clarity
- •Secondary Abnormalities Affecting the Fetal Cornea
- •Intrauterine Keratitis: Bacterial and Syphilitic
- •Congenital Corneal Keloid
- •Congenital Corneal Anesthesia
- •Congenital Glaucoma
- •Birth Trauma
- •Arcus Juvenilis
- •10 Corneal Dystrophies and Ectasias
- •Corneal Dystrophies
- •Epithelial and Subepithelial Dystrophies
- •Bowman Layer Corneal Dystrophies
- •Stromal Corneal Dystrophies: TGFBI Dystrophies
- •Stromal Dystrophies: Non-TGFBI Dystrophies
- •Endothelial Dystrophies
- •Ectatic Disorders
- •Keratoconus
- •Pellucid Marginal Degeneration
- •Keratoglobus
- •11 Systemic Disorders With Corneal Changes
- •Disorders of Carbohydrate Metabolism
- •Mucopolysaccharidoses
- •Diabetes Mellitus
- •Disorders of Lipid Metabolism and Storage
- •Hyperlipoproteinemias
- •Hypolipoproteinemias
- •Sphingolipidoses
- •Mucolipidoses
- •Disorders of Amino Acid Metabolism
- •Cystinosis
- •Tyrosinemia
- •Alkaptonuria
- •Disorders of Protein Metabolism
- •Amyloidosis
- •Disorders of Immunoglobulin Synthesis
- •Noninflammatory Disorders of Connective Tissue
- •Ehlers-Danlos Syndrome
- •Marfan Syndrome
- •Disorders of Nucleotide Metabolism
- •Gout
- •Porphyria
- •Disorders of Mineral Metabolism
- •Wilson Disease
- •Hypercalcemia
- •Hemochromatosis
- •Corneal and External Disease Signs of Systemic Neoplasia
- •Enlarged Corneal Nerves
- •Appendix
- •12 Clinical Approach to Depositions and Degenerations of the Conjunctiva, Cornea, and Sclera
- •Degenerative Changes of the Conjunctiva
- •Age-Related (Involutional) Changes
- •Pinguecula
- •Pterygium
- •Conjunctival Concretions
- •Conjunctival Inclusion Cysts
- •Conjunctivochalasis
- •Conjunctival Vascular Tortuosity and Hyperemia
- •Degenerative Changes in the Cornea
- •Age-Related (Involutional) Changes
- •Epithelial and Subepithelial Degenerations
- •Stromal Degenerations
- •Endothelial Degenerations
- •Scleral Degenerations
- •Drug-Induced Deposition and Pigmentation
- •Corneal Epithelial Deposits
- •Stromal and Descemet Membrane Pigmentation
- •Endothelial Manifestations
- •13 Clinical Aspects of Toxic and Traumatic Injuries of the Anterior Segment
- •Injuries Caused by Temperature and Radiation
- •Thermal Burns
- •Ultraviolet Radiation
- •Ionizing Radiation
- •Chemical Injuries
- •Alkali Burns
- •Acid Burns
- •Management of Chemical Injuries
- •Toxic Keratoconjunctivitis From Medications
- •Pathogenesis
- •Clinical Presentation
- •Management
- •Animal and Plant Substances
- •Insect Injuries
- •Vegetation Injuries
- •Concussive Trauma
- •Subconjunctival Hemorrhage
- •Corneal Changes
- •Traumatic Mydriasis and Miosis
- •Traumatic Iritis
- •Iridodialysis and Cyclodialysis
- •Traumatic Hyphema
- •Nonperforating Mechanical Trauma
- •Conjunctival Laceration
- •Conjunctival Foreign Body
- •Corneal Foreign Body
- •Corneal Abrasion
- •Perforating Trauma
- •Evaluation
- •Management
- •Surgical Trauma
- •Corneal Epithelial Changes From Intraocular Surgery
- •Descemet Membrane Changes During Intraocular Surgery
- •Corneal Endothelial Changes From Intraocular Surgery
- •Conjunctival and Corneal Changes From Extraocular Surgery
- •14 Treatment of Ocular Surface Disorders
- •Surgical Procedures of the Ocular Surface
- •Limbal Transplantation
- •Autologous Conjunctival Transplantation
- •Pterygium Excision
- •Mucous Membrane Grafting
- •Conjunctival Flap
- •Conjunctival Biopsy
- •Conjunctivochalasis Excision
- •Therapeutic Interventions for Corneal Disease
- •Superficial Keratectomy and Corneal Biopsy
- •Management of Descemetocele, Corneal Perforation, and Corneal Edema
- •Corneal Tattoo
- •Tarsorrhaphy
- •15 Clinical Approach to Corneal Transplantation
- •Corneal Transplantation
- •Eye Banking and Donor Selection
- •Criteria Contraindicating Donor Cornea Use
- •Surgical Approach to Corneal Disease
- •Preoperative Evaluation and Preparation
- •Penetrating Keratoplasty
- •Surgical Technique for Penetrating Keratoplasty
- •Combined Procedures
- •Intraoperative Complications
- •Postoperative Care and Complications
- •Control of Postoperative Corneal Astigmatism and Refractive Error
- •Diagnosis and Management of Graft Rejection
- •Pediatric Corneal Transplantation
- •Corneal Autograft Procedures
- •Keratoprosthesis
- •Lamellar Keratoplasty
- •Anterior Lamellar Transplantation
- •Surgical Technique
- •Postoperative Care and Complications
- •Endothelial Keratoplasty
- •DSEK Surgical Technique and Complications
- •Descemet Membrane Endothelial Keratoplasty
- •Basic Texts
- •Related Academy Materials
- •Requesting Continuing Medical Education Credit
CHAPTER 12
Clinical Approach to Depositions and Degenerations of the Conjunctiva, Cornea, and Sclera
Degeneration of a tissue refers to decomposition and deterioration of tissue elements and functions. Degenerations of the ocular surface may result from physiologic changes associated with aging, be related to a specific disease, or follow chronic environmental insults to the eye, such as exposure to ultraviolet (UV) light. They may be unilateral or bilateral; if bilateral, they may be asymmetric. They uncommonly exhibit an inheritance pattern. It is important to differentiate corneal degenerations from corneal dystrophies (Table 12-1).
Table 12-1
Degenerative Changes of the Conjunctiva
Age-Related (Involutional) Changes
As a result of aging, the conjunctiva loses transparency and becomes thinner. The substantia propria (stroma) becomes less elastic, causing conjunctival laxity. In older individuals, the conjunctival vessels can become more prominent. Saccular telangiectasias, fusiform dilatory changes, or tortuosities may appear in the vessels. These changes are not necessarily uniform; they tend to be more pronounced in the area of the interpalpebral fissure, corresponding to the area most commonly exposed to the environment.
Pinguecula
A pinguecula is a common conjunctival condition that occurs typically on the nasal side of the bulbar conjunctiva, adjacent to the limbus in the interpalpebral zone. It usually is bilateral, appears as a
yellow-white elevated mass (Fig 12-1), and occurs as a result of the effects of aging, UV-light exposure, and other environmental traumas such as dust and wind. It may enlarge gradually over long periods of time. Recurrent inflammation and ocular irritation may be observed. Pingueculae represent an elastotic degeneration (the material stains for elastin but is not broken down by elastase) of subepithelial collagen with hyalinized connective tissue. Lubricant therapy to alleviate ocular irritation is the mainstay of treatment. Excision is indicated only when pingueculae are cosmetically unacceptable or when they become chronically inflamed or interfere with successful contact lens wear. Judicious use of topical corticosteroids may be considered in patients with inflammation, but their use as a long-term therapy for pingueculae is strongly discouraged due to their adverse effects.
Figure 12-1 A pinguecula, seen, as is typical, on the nasal side of the bulbar conjunctiva. (Courtesy of Cornea Service, Paulista
School of Medicine, Federal University of São Paulo.)
Pterygium
A pterygium is a wing-shaped growth of conjunctiva and fibrovascular tissue on the superficial cornea (Fig 12-2). As with a pinguecula, the pathogenesis of a pterygium is strongly correlated with UV-light exposure, although environmental traumas such as exposure to dust, wind, or other irritants that cause chronic inflammation may also be factors. The predominance of pterygia on the nasal side in the interpalpebral zone is theorized to result from light passing medially through the cornea, focusing on the nasal limbus area, while the shadow of the nose reduces the intensity of light transmitted to the temporal limbus. The prevalence of pterygia increases steadily with proximity to the equator and is more common in men than women, in persons 20–30 years of age (the most common age of onset), and in people who work outdoors. The histopathology of pterygia is similar to that of pingueculae, only it involves subepithelial fibrovascular tissue. Further discussion of the histopathology of both pingueculae and pterygia can be found in BCSC Section 4, Ophthalmic Pathology and Intraocular Tumors.
Figure 12-2 A pterygium: a wing-shaped growth of conjunctiva and fibrovascular tissue on the superficial cornea. (Courtesy of
Cornea Service, Paulista School of Medicine, Federal University of São Paulo.)
Pterygia are nearly always preceded by pingueculae, although why some patients develop pterygia whereas others have only pingueculae is not known. Regular and irregular astigmatism, as well as corneal scarring, occurs in proportion to pterygium size. A pigmented iron line (called a Stocker line) may be seen at the central anterior edge of the pterygium on the cornea. A pterygium must be differentiated from a pseudopterygium, which may occur after trauma or secondary to inflammatory corneal disease. Treatment with artificial tears can alleviate associated irritation, but as with pingueculae, long-term use of topical corticosteroids is contraindicated. Excision is indicated if the pterygium approaches the visual axis, exhibits rapid growth, causes chronic irritation, or is cosmetically unacceptable. See Chapter 14 for discussion of the surgical treatment of pterygium.
Conjunctival Concretions
Concretions are small, yellow-white dots found in the palpebral conjunctiva of older patients or patients who have had chronic conjunctivitis. Concretions appear to be epithelial inclusion cysts filled with epithelial and keratin debris, as well as mucopolysaccharide and mucin. Concretions are almost always asymptomatic but may erode the overlying epithelium, causing foreign-body sensation. If symptomatic, concretions can be easily removed under topical anesthesia.
Conjunctival Inclusion Cysts
Inclusion cysts of the conjunctival epithelium are typically asymptomatic and are often an incidental finding during routine ophthalmic examination. Conjunctival inclusion cysts can be congenital or acquired. Most acquired cysts of the conjunctiva are derived from an inclusion of conjunctival epithelium into the substantia propria. The implanted cells proliferate to form a central fluid-filled cavity that is lined by nonkeratinized conjunctival epithelium. Conjunctival cysts may also form from ductal epithelium of the accessory lacrimal glands; these cysts are lined by a double layer of epithelium. Stimuli for cyst formation include chronic inflammation, trauma, and surgery.
Conjunctival inclusion cysts typically appear clear and can occur in either the bulbar conjunctiva or the conjunctival fornix (Fig 12-3). A corneal epithelial inclusion cyst is rare, but it can occur if trauma, surgery, or chronic inflammation results in conjunctival overgrowth onto the surface of the cornea. Dilated lymphatic channels may mimic an inclusion cyst of the bulbar conjunctiva.
Figure 12-3 Large conjunctival epithelial inclusion cyst.
Epithelial inclusion cysts are most commonly asymptomatic and therefore may be simply observed. Cysts usually re-form after simple drainage because the inner epithelial cell wall remains. Complete excision is necessary to prevent recurrence.
Conjunctivochalasis
Conjunctivochalasis is poor adherence of the bulbar conjunctiva. It occurs commonly with chronic inflammation or aging and is often overlooked and asymptomatic. Occasionally, the redundant conjunctiva overhangs the lower eyelid margin to such an extent that various clinical problems appear (Fig 12-4). These range from the aggravation of dry eye in the mild stages (from exposure of the redundant conjunctiva due to uneven wetting), to secondary tearing due to occlusion of the lower punctum when the chalasis is prominent medially, to exposure-related pain and irritation in its severe stages. The etiology of conjunctivochalasis remains unknown, but increased tear inflammation seems to accompany loss of conjunctival epithelial cohesiveness and increased collagenolytic activity, which may explain the conjunctival laxity. Histologic studies have also revealed elastosis and chronic nongranulomatous inflammation, in addition to collagenolysis, in conjunctivochalasis.
Figure 12-4 A “redundant” conjunctiva seen in conjunctivochalasis. (Courtesy of Cornea Service, Paulista School of Medicine, Federal
University of São Paulo.)
