- •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
Lamellar Keratoplasty
With advances in instrumentation and techniques, the selective removal of corneal tissue (lamellar keratoplasty [LK]) has become more popular. The general ophthalmologist should be familiar with the special indications for and limitations of the major techniques of LK, including SALK, DALK, DSEK, and DMEK (see Table 15-3). DSEK and DMEK are discussed separately later in this chapter.
Anterior Lamellar Transplantation
Lamellar corneal grafting may be indicated in patients who present with opacities or loss of tissue that, for the most part, does not involve the full thickness of the cornea. These conditions include
superficial stromal dystrophies and degenerations (eg, Reis-Bücklers dystrophy, Salzmann nodular degeneration, band keratopathy)
superficial corneal scars
multiple recurrent pterygium with visually significant corneal scarring
corneal thinning (eg, Terrien marginal degeneration, descemetocele formation, pellucid marginal degeneration) (Fig 15-13)
superficial corneal tumors congenital lesions (eg, dermoid)
corneal perforations that are not amenable to resuturing or that occur in patients with ocular surface disease (eg, keratoconjunctivitis sicca)
keratoconus
selective infections, including Acanthamoeba keratitis
Figure 15-13 A, Descemetocele in a patient with rheumatoid arthritis. B, Same patient after lamellar keratoplasty.
Advantages
LK has the following advantages over PK:
minimal requirements for donor material (as preservation of endothelium is not mandatory) reduced risk of entry into the anterior chamber (avoids risks of glaucoma, cataract, retinal detachment, cystoid macular edema, expulsive hemorrhage, and endophthalmitis)
shorter wound healing time and convalescence
reduced incidence of allograft rejection due to no risk of endothelial rejection and, consequently, decreased need for topical corticosteroids
reduced risk of traumatic wound rupture
improved long-term endothelial cell viability and increased graft longevity
better prognosis for patients who have poor adherence to medical instructions or difficulty obtaining frequent follow-up
Disadvantages
Anterior LK does not replace damaged endothelium. The procedure is more technically demanding and time-consuming than PK. It may be associated with opacification and vascularization of the interface. Descemet detachment may produce a double anterior chamber, and placement of an air bubble to tamponade the Descemet membrane against the stroma can lead to the development of an anterior subcapsular cataract.
Surgical Technique
Superficial anterior lamellar keratoplasty
SALK may be performed when the opacity is superficial. If there is a peripheral opacity or perforation, manual lamellar dissection is followed by placement of appropriately sized thin donor tissue.
To treat a central corneal opacity, SALK can be performed in 1 or 2 stages. In the 2-stage procedure, the initial step is to use a microkeratome to create a lamellar flap that encompasses the corneal pathology. This is useful only if the corneal surface is regular, because if there is irregularity, the microkeratome will produce an irregular corneal bed. The flap is lifted to ensure that the underlying stroma is clear and then replaced. After a 4- to 6-week interval that allows the flap to stabilize and partially adhere, the second stage is performed. A trephine is centered over the pupil and used to incise the previously made flap to a depth slightly below the initial lamellar dissection, ideally leaving a 1-mm flap rim. A blunt spatula is gently introduced at the plane of the lamellar flap, and the abnormal tissue is separated and lifted off. The donor tissue is then prepared using an artificial anterior chamber and a microkeratome with the same thickness head as in the initial procedure. A trephine of the same or a slightly oversized (0.25 mm) diameter is used to excise the tissue. The donor disk is then transferred to the host bed and, if there is a good match, the donor tissue may adhere spontaneously without sutures, although 10-0 nylon sutures can be used to anchor the graft and ensure proper alignment. A bandage lens can also be placed to facilitate adherence. In the 2-stage approach, the diameter of the host bed can be matched precisely with the diameter of the donor tissue with nice vertical incisions from trephination. Recently, the femtosecond laser has been used to perform the lamellar dissections of both the host and donor tissue in a single session.
Deep anterior lamellar keratoplasty
To obtain the best visual outcome, the interface must be smooth and clear, and almost all host stromal tissue must be removed. The Anwar big-bubble technique is the most widely used method of isolating Descemet membrane. If a big bubble cannot be produced, manual dissection is possible, but it risks loss of best-corrected visual acuity due to incomplete removal of the host stromal tissue. In an OCT study of patients who underwent DALK, 20 μm of residual stromal bed was not visually significant; however, 80 μm of tissue caused a reduction in vision.
The surgical technique for DALK begins with deep (at least 300 μm) trephination. Depending on surgeon preference, the anterior lamellae may be dissected and removed, leaving a thin layer of stroma, or left untouched. A tunnel is then fashioned from the deepest point of the trephination incision, extending 2–3 mm to the paracentral cornea and running parallel to the surface of the cornea to avoid perforation of Descemet membrane. Air is forcefully injected into the stroma and, hopefully, enters the pre-Descemet plane. If this step is successful, an air bubble outlined by a circular white band will suddenly appear. Specialized instruments—such as those developed by Dr Rajesh Fogla (Bausch + Lomb Instruments, St Louis, MO) and by Dr Donald Tan and Dr Vincenzo Sarnicola (both instruments available from ASICO, Westmont, IL)—aid in the performance of these maneuvers.
A limbal paracentesis is made to drain aqueous humor from the eye in order to reduce the pressure and help prevent perforation of the cornea while exposing Descemet membrane. The anterior wall of the bubble is then entered with a sharp 15° blade to create a 1-mm opening. Once incised, the bubble collapses; some surgeons then inject viscoelastic material into the collapsed bubble to help with the meticulous dissection. A spatula can be used to enter the pre-Descemet plane and advanced to the 6-o’clock position. When the spatula is lifted anteriorly, the stroma on the top of the spatula is incised using a sharp blade. As the stroma is severed, Descemet membrane is exposed. A similar maneuver is performed in the opposite direction. Blunt-pointed Vannas scissors and 0.12 forceps are used to make 2 long, perpendicular incisions, creating 4 quadrants of residual stroma; then each quadrant is excised, baring Descemet membrane. (Videos of this technique are available on the Internet.)
Only after recipient bed preparation is completed is the donor tissue prepared, because inadvertent entry into the anterior chamber requires conversion to a full-thickness PK. Inadvertent entry is not infrequent early in the learning curve, and it happens occasionally among experienced surgeons.
The donor tissue is prepared by punching an appropriate-sized button with a trephine. Trypan blue can be used to stain the endothelium to improve visualization in order to facilitate the removal of Descemet membrane and endothelium from the donor tissue. The donor tissue is then sutured into position using 10-0 nylon sutures in a continuous or interrupted fashion, as in a full-thickness PK.
Postoperative Care and Complications
Double anterior chamber or Descemet detachment
Descemet detachment or pseudo–double anterior chambers can occur because of fluid in the interface from a microperforation or retained viscoelastic material. Injection of air into the anterior chamber can help with resolution of the detachment; however, it may also reduce endothelial cell count and lead to the development of an anterior subcapsular cataract.
Opacification and vascularization of the interface
Meticulous irrigation and cleaning of the lamellar bed at the time of surgery reduces the likelihood of opacification. Retained interface debris, secondary vascularization, microbial infections, or wrinkles
