- •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
Figure 2-15 Topography of a normal cornea with regular astigmatism. The white circle indicates the pupil. Simulated
keratometry is 41.3, 46.2@102. (Courtesy of John E. Sutphin, MD.)
Corneal Tomography
Placido disk–based topography describes only the surface corneal curvature (power), whereas corneal tomography provides details such as the anterior and posterior corneal curvature, corneal thickness, and anterior chamber depth, as well as information on the iris and lens. The Orbscan IIz (Bausch + Lomb, Rochester, NY) combines an advanced Placido disk system with slit-scanning technology and derives its posterior elevation map mathematically. This map may overestimate the posterior corneal curvature, however, especially in patients who have undergone LASIK procedures. The Scheimpflug system creates an optical section of the cornea and lens, producing a 3-dimensional image of the anterior segment. The Pentacam (Oculus, Lynwood, WA) uses a rotating Scheimpflug camera, whereas the Galilei (Ziemer USA, Inc, Wood River, IL) combines a dual Scheimpflug camera system with Placido disk technology.
Scheimpflug camera–based systems present considerable information, including anterior curvature, corneal thickness, anterior chamber depth, anterior and posterior elevation, and pupil indices; they also provide keratoconus detection and classification. As a result, the pachymetry and
topography of the entire anterior and posterior surface of the cornea can be displayed (Fig 2-16). There is also a densitometry function that measures the amount of corneal or lens opacification, information that is useful in observing patients with Fuchs corneal dystrophy or those who have undergone endothelial keratoplasty. In addition, these systems can provide a measurement of the true corneal power for use in IOL power calculation.
Figure 2-16 Scheimpflug image of a 65-year-old patient with Fuchs endothelial dystrophy and cataract. The general display clearly depicts epithelial and endothelial opacity of the cornea with a densitometry measurement of 49.7 (normal, 22–30) and the lenticular opacity with a densitometry reading of 37.0. In addition, keratometry, axis of astigmatism, corneal thickness,
and anterior chamber depth are provided. (Reproduced with permission from Goins KM, Wagoner MD. Imaging the anterior segment. Focal Points: Clinical Modules for Ophthalmologists. San Francisco: American Academy of Ophthalmology; 2009, module 11.)
Indications
Corneal evaluation is important in the preoperative evaluation of cataract and refractive surgery patients. For most normal corneas, keratometry measurements and corneal topographic maps are accurate and can be used in contact lens fitting or IOL power calculation. They are also useful in detecting irregular astigmatism in which the reflected images cannot be superimposed or are not regular ovals. In these patients, the surface regularity index will be reduced. Patients with corneal warpage (irregular astigmatism and/or peripheral steepening, distorted keratoscopic mires) due to contact lens wear should be instructed to discontinue the lenses until the corneal map and refraction stabilize.
Corneal topography is helpful to screen for forme fruste or subclinical keratoconus, particularly
in prospective refractive surgery patients. Corneal tomography may provide more useful information in these patients, as it may reveal subtle changes in the posterior corneal curvature that may precede the development of anterior steepening. Pellucid marginal degeneration is characterized by peripheral steepening or a “crab claw” configuration on corneal topography. Measurements of the posterior corneal curvature and pachymetry provide important confirmation of thinning and steepening, ensuring an accurate diagnosis (Fig 2-17).
Figure 2-17 Topography of a patient with pellucid marginal degeneration. The “crab claw” appearance is fully developed, with central flattening and inferior steepening; forme fruste keratoconus may have a similar but less definite appearance. (Courtesy
of John E. Sutphin, MD.)
Corneal evaluation can also be used to show the effects of keratorefractive procedures. Preoperative and postoperative maps may be compared algebraically to determine whether the desired effect was achieved. Corneal mapping may help explain unexpected results, including undercorrections, aberrations, induced astigmatism, or glare and halos, by detecting decentered or inadequate surgery; in addition, it may help confirm the expected physiologic effects of refractive surgery. For example, in LASIK for myopia, the ablation profile leads to flattening of the central cornea and a relative peripheral steepening.
Corneal mapping is useful in managing congenital and postoperative astigmatism, particularly following penetrating keratoplasty. Complex peripheral patterns may result in a refractive axis of astigmatism that is not aligned with a topographic axis. Before removing sutures or performing surgery, the surgeon must identify the steep axis based on the corneal curvature and not the refraction (incisional surgery is done on the steep axis, compression sutures on the flat axis). See also Chapter 15 for discussion of the management of astigmatism after corneal transplantation.
In patients with previous radial keratotomy, photorefractive keratectomy, or LASIK, neither the keratometer nor the corneal topographer measures the true central corneal power, and other methods
are required for IOL power calculation. In these patients, corneal tomography can be very helpful. See also BCSC Section 3, Clinical Optics, and BCSC Section 13, Refractive Surgery.
Belin MW, Asota IM, Ambrosio R Jr, Khachikian SS. What’s in a name: keratoconus, pellucid marginal degeneration, and related thinning disorders. Am J Ophthalmol. 2011;152(2):157–162.
Courville CB, Klyce SD. Corneal topography. In: Foster CS, Azar DT, Dohlman CH, eds. Smolin and Thoft’s The Cornea: Scientific Foundations and Clinical Practice. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2004:175–185.
Martinez CE, Klyce SD. Keratometry and topography. In: Krachmer JH, Mannis MJ, Holland EJ, eds. Cornea. 3rd ed. Vol 1. Philadelphia: Elsevier/Mosby; 2011:161–176.
Additional Imaging Techniques of the Cornea and Anterior
Segment
Ultrasound Biomicroscopy
Anterior segment echography, or ultrasound biomicroscopy (UBM)—specifically, high-frequency ultrasonography—uses a water-bath immersion technique to image the anterior segment. With this technique, the depth of tissue penetration is approximately 5 mm and the resolution is 35 to 70 μm. UBM allows structures to be viewed through opaque media. Figure 2-18 is an example of ultrahighfrequency biomicroscopy of the normal limbus. This technology allows impressive visualization of the iris, ciliary body, and ciliary processes, thereby enabling accurate white-to-white, sulcus-to- sulcus, and angle width measurements, which are essential for selection of the appropriate lens size prior to placement of a phakic refractive implant. UBM can also be used in the diagnosis and followup of iris cysts and iris/ciliary body melanomas. In addition, UBM is particularly useful in cases of trauma, as it allows visualization of the iris and lens even in the patient with an “eight-ball” hyphema. Also, using UBM, one can search for possible angle recession or cyclodialysis in the patient with severe blunt trauma to the eye.
