- •Contents
- •Foreword
- •Preface
- •List of Abbreviations
- •1. General Topics in Ophthalmology
- •Approach to the Patient: Exam and History
- •Embryology and Development
- •Emergencies
- •Epidemiology/Statistics
- •Genetics
- •Imaging: Computed Tomography (CT)
- •Imaging: Magnetic Resonance Imaging (MRI)
- •Optics
- •Pathology
- •Pregnancy
- •Radiation/Laser
- •Surgery
- •2. Orbit, Eyelids, and Ocular Adnexa
- •Anatomy and Physiology
- •Signs and Symptoms
- •Exam and Imaging
- •Congenital and Genetic Disease
- •Infectious Disease
- •Neoplastic, Eyelid: Benign Cystic Lesions
- •Neoplastic, Eyelid: Benign Growths
- •Neoplastic, Eyelid: Hair Follicle Tumors (‘‘Tricky’’ Tumors)
- •Neoplastic, Eyelid: Premalignant Lesions
- •Neoplastic, Eyelid: Malignant Lesions
- •Neoplastic, Eyelid and Orbital: Pigmented Lesions
- •Neoplastic, Orbital: Lacrimal Gland Tumors
- •Neoplastic, Orbital: Lymphoproliferative Lesions
- •Neoplastic, Orbital: Mesenchymal Tumors
- •Neoplastic, Orbital: Metastatic and Invasive Tumors
- •Neoplastic, Orbital: Neurogenic Tumors
- •Neoplastic, Orbital: Structural Lesions
- •Neoplastic, Orbital: Vascular Lesions
- •Metabolic and Degenerative Disease
- •Systemic and Vascular Disease
- •Physical Disease
- •Inflammatory and Immune Disease
- •Eyelid Surgery
- •Lacrimal Surgery
- •Orbital Surgery
- •Other Surgery
- •3. Cornea and Conjunctiva
- •Anatomy and Physiology
- •Signs and Symptoms
- •Exam and Imaging
- •Congenital and Genetic Disease
- •Infectious Disease
- •Neoplastic Disease: Benign Masses
- •Neoplastic: Malignant
- •Neoplastic: Melanocytic Lesions
- •Metabolic and Degenerative Disease
- •Physical Disease
- •Inflammatory and Immune Disease
- •Surgery
- •4. Glaucoma
- •Anatomy and Physiology
- •Signs and Symptoms
- •Exam and Imaging
- •Infantile and Pediatric Glaucoma
- •Primary Open-Angle Glaucoma
- •Secondary Open-Angle Glaucomas
- •Primary Angle-Closure Glaucoma
- •Secondary Angle-Closure Glaucoma
- •Surgery
- •Iris
- •Uveitis
- •Signs and Symptoms and Clinical Presentations of Uveitis
- •Anterior Uveitis Diagnosis and Work-Up
- •Anterior Uveitis Diseases
- •Intermediate Uveitis Diagnosis and Work-Up
- •Intermediate Uveitis Diseases
- •Posterior Uveitis Diagnosis and Work-Up
- •Posterior Uveitis Diseases
- •Panuveitis/Diffuse Uveitis
- •Surgery
- •6. Lens
- •Anatomy and Physiology
- •Signs and Symptoms
- •Congenital and Genetic Disease
- •Metabolic and Degenerative Disease
- •Systemic and Vascular Disease
- •Physical Disease
- •Cataract Surgery
- •Refractive Lens Surgery
- •7. Retina and Vitreous
- •Anatomy and Physiology
- •Signs and Symptoms
- •Exam and Imaging
- •Congenital and Genetic Disease
- •Hereditary Macular Disorders
- •Progressive Tapetoretinal Disorders
- •Stationary Tapetoretinal Disorders
- •Congenital and Genetic Vitreoretinopathies
- •Infectious Disease
- •Infectious Endophthalmitis
- •Neoplastic Disease
- •Metabolic and Degenerative Disease
- •Systemic and Vascular Disease
- •Physical Disease
- •Surgery: Laser
- •Surgery: Operative
- •8. Neurologic
- •Anatomy and Physiology
- •Signs and Symptoms
- •Exam and Imaging
- •Congenital and Genetic Disease
- •Infectious Disease
- •Neoplastic Disease
- •Metabolic and Degenerative Disease
- •Systemic and Vascular Disease
- •Physical Disease
- •Inflammatory and Immune Disease
- •Selected Cranial Nerve Abnormalities
- •Gaze Abnormalities
- •Nystagmus
- •Psychiatric Diseases
- •9. Pediatrics and Strabismus
- •Anatomy and Physiology
- •Signs and Symptoms
- •Exam and Imaging
- •Congenital and Genetic Disease
- •Congenital Disease: Phakomatoses
- •Strabismus: Esotropia
- •Strabismus: Exotropia
- •Strabismus: Vertical Deviations
- •Surgery
- •Other
- •10. Medications
- •General
- •Antibacterials: Aminoglycosides
- •Antibacterials: Cephalosporins and Penicillins
- •Antibacterials: Fluoroquinolones
- •Antibacterials: Inhibitors of Cell Wall Function
- •Antibacterials: Inhibitors of Nucleic Acid Synthesis
- •Antibacterials: Inhibitors of Protein Synthesis
- •Antibacterials: Combinations
- •Antibacterial and Corticosteroid Combinations
- •Antifungal Agents
- •Anti-inflammatory: Allergy
- •Anti-inflammatory: Nonsteroidals
- •Antiseptics
- •Antiviral Agents
- •Glaucoma: Beta-Blockers
- •Glaucoma: Carbonic Anhydrase Inhibitors
- •Glaucoma: Hyperosmotics
- •Glaucoma: Miotics/Cholinergic, Direct Acting
- •Glaucoma: Miotics/Cholinergic, Indirect Irreversible
- •Glaucoma: Prostaglandin Analogues
- •Glaucoma: Sympathomimetics
- •Immunosuppressives: Antimetabolites
- •Immunosuppressives: Alkylating Agents
- •Immunosuppressives: Noncytotoxic
- •Miotics: Cholinergic, Indirect Reversible
- •Mydriatics and Cycloplegics: Anticholinergic
- •Mydriatics: Adrenergic
- •Neurologic Medications
- •Other Agents
- •Index
CHAPTER 5
Uveal Tract and
Ocular Inflammation
Iris
IRIS ANATOMY (FIG. 5–1) Dimensions: 12 mm diameter, 37.5 mm circumference (length of Schlemm’s canal); root of iris 0.18 mm thick, midstroma 0.5 mm; thickest at collarette. Radial vessels run from the major arterial circle at the root from two long posterior ciliary arteries and seven anterior ciliary arteries, with a minor circle under the collarette. Surface anatomy from outside in: peripheral crypts, ciliary iris, collarette, Fuchs’ crypts, and pupil. Anterior iris has melanocytes, fibroblasts, and capillaries (increased with rubeosis or pseudorubeosis from iritis). Stroma is composed of collagen, GAG ground substance, channels, and clump cells (macrophages). Stromal pigmentation (neural crest origin) gives iris color, and resident melanocytes are postmitotic and not reactive like the RPE (thus the patency of PI). The iris is an extension of the choroid, which is attached to the sclera only at the vortex vein ostia (the cause of the cloverleaf pattern seen with choroidal effusions) and at the scleral spur.
The iris dilator and sphincter muscles arise from the IPE. The dilator has sympathetic innervation that also stimulates pigmentation (thus, congenital Horner’s syndrome has light-colored irides). The sphincter muscle has cholinergic innervation. Fuchs’ and Michel’s spurs connect the sphincter and dilator muscles; thus, when the iris contricts, it stretches the dilator muscle and vice versa. The iris constrictor shortens by 80% (unusual for a muscle) and thus needs crypts to allow for increased movement.
Iris pigment epithelium (IPE): bilayer of anterior nonpigmented epithelium and posterior pigmented cells are in an apical-to-apical arrangement (like the ciliary body). Iris cysts form if the apical junction breaks down. Poor iris dilation is seen in patients with PXS from basement membrane material deposited into the muscle. Histopathologically, lacy vacuolization represents glycogen deposits seen in diabetic patients.
IRIS COLOBOMA Usually inferonasal.
172
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Figure 5–1 Diagram of iris anatomy and the aqueous drainage system.
173
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174 UVEAL TRACT AND OCULAR INFLAMMATION
IRIS MELANOMA Concern for melanoma if pigmented iris lesion is >2 mm. Increased risk of metastatic cancer increases with age, increased IOP, tumor in angle, extraocular extension, and prior surgery, but not the type of cells seen on pathology. There is a 5% risk of metastatic cancer at 10 years.
IRIDODIALYSIS Tear in root of iris; distinguish from coloboma (which is inferonasal and has the sphincter involved).
IRIDOSCHISIS Seen in elderly patients, with bilateral inferior iris stomal ‘‘shredding’’ and atrophy, which may obstruct the TM and cause glaucoma.
Uveitis
DEFINITION AND CLASSIFICATION Uveitis is broadly defined as inflammation of the uveal tract (iris, ciliary body, and choroid). The definition is complicated by other types of intraocular inflammation (e.g., infections, endophthalmitis, or toxoplasmosis retinitis that spills over to the choroid). Thus, defining the uveitic diseases involves careful classification as to the primary tissue that is affected. In this chapter, the uveitic diseases are classified as anterior (involving the iris and anterior segment primarily), intermediate (involving the pars plana and anterior vitreous primarily), posterior (involving the choroid and retina primarily), and panuveitis (diffuse inflammation of ocular tissues). Uveitis can further be classified by the histopathologic characteristics of granulomatous or nongranulomatous types of inflammation. Further delineation of the inflammation can be made as to whether it is a local ocular disease process only or part of a systemic disease. Thus, a careful history and a review of systems are important first steps in elucidating the etiology of the patient’s inflammation.
GOALS OF THERAPY Suppress the inflammation and prevent secondary complications. Ten percent of cases of blindness in the United States are caused by uveitis complications.
Complete elimination of all active inflammation and cells: most important therapeutic goal, because allowing chronic, even low grade (1 þ cells), uveitis eventually produces permanent structural damage, such as chronic macular edema with cyst or macular hole formation, cyclitic membrane formation with hypotony, and ON damage.
Limitation of total steroid used: long-term use of corticosteroids has a 100% incidence of significant undesirable side effects.
Tables 5–1 and 5–2 present diagnostic uveitis laboratory tests, and associations between uveitis and human leukocyte antigens (HLA).
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UVEITIS 175
TABLE 5–1
Selected Diagnostic Uveitis Laboratory Tests
Laboratory Test |
Indication and Comments |
|
|
|
|
|
|
ACE level |
Elevated in two thirds of patients with active sarcoidosis |
|
|
ANA |
Family of antinuclear antibodies that are elevated in |
|
|
|
collagen-vascular diseases, particularly SLE and RA |
|
|
C-ANCA |
Cytoplasmic staining pattern of IgG autoantibodies against |
|
|
|
PMN antigen; 90% sensitive and specific for Wegener’s |
|
|
|
granulomatosis |
|
|
P-ANCA |
Perinuclear staining pattern of IgG autoantibodies |
|
|
|
against PMN antigen; positive with microscopic PAN and |
|
|
|
Churg-Strauss syndrome, and also positive occasionally in |
|
|
|
Wegener’s granulomatosis and crescentic |
|
|
|
glomerulonephritis |
|
|
CBC |
Elevated white cell count with infection or leukemia; |
|
|
|
eosinophilia with systemic toxocariasis, atopy, or Churg- |
|
|
|
Strauss syndrome; lymphopenia with SLE |
|
|
|
and HIV |
|
|
ESR/CRP/C-25 |
Nonspecific inflammation and immune system activation |
|
|
Hepatitis B surface |
Positive in 40% of PAN |
|
|
antigen |
|
|
|
Herpes virus antibodies |
Because the prevalence of seropositivity is so high in the |
|
|
|
general population, a positive titer is virtually meaningless; |
|
|
|
however, a negative titer all but eliminates herpes |
|
|
|
from consideration; in contrast, antigen sampling with |
|
|
|
PCR amplification from an intraocular specimen is very |
|
|
|
specific |
|
|
HIV tests |
Antibodies against HIV are detected by ELISA and |
|
|
|
confirmed by Western blot test; in addition, T-lymphocyte |
|
|
|
levels are decreased in HIV infection, notably with a |
|
|
|
decreased ratio of CD4+ (T-helper) relative to CD8+ |
|
|
|
(T-suppressor) cells |
|
|
Lyme serologies |
ELISA immunoassay followed by confirmatory Western blot |
|
|
|
test for Borrelia burgdorferi; may be false-negative if early in |
|
|
|
the infection; false-positive result may be seen with syphilis |
|
|
|
or collagen vascular disorders |
|
|
Lysozyme levels |
Tend to parallel ACE levels, but may be a more sensitive |
|
|
|
indicator of active pulmonary saroidosis |
|
|
Raj immune complexes |
Circulating IgG |
|
|
Rheumatoid factor (RF) |
Class of autoantibodies (usually IgM) against the Fc fragment |
|
|
|
of IgG; seropositivity is nonspecific and may occur in a |
|
|
|
number of collagen vascular disorders, but 80% of patients |
|
|
|
with RA are RF positive |
|
|
|
(Continued) |
|
|
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