- •The Sclera
- •Preface
- •Contents
- •1.1 Introduction
- •1.2 Development of the Sclera
- •1.2.1.1 First Week
- •1.2.1.2 Second Week
- •1.2.1.3 Third Week
- •1.2.1.4 Fourth Week
- •1.2.1.5 Fifth Week
- •1.2.1.6 Sixth Week
- •1.2.1.7 Seventh Week
- •1.2.1.8 Ninth Week
- •1.2.1.9 Tenth Week
- •1.2.1.10 Thirteenth Week
- •1.2.1.11 Sixteenth Week
- •1.2.1.12 Twenty-Fourth Week
- •1.2.2.1 Collagens
- •1.2.2.2 Proteoglycans
- •1.2.2.3 Glycoproteins
- •1.3 Anatomy
- •1.3.1 Gross and Microscopic Anatomy
- •1.3.1.1 Scleral Foramina
- •Anterior Scleral Foramen
- •Posterior Scleral Foramen
- •1.3.1.2 Layers of the Sclera
- •Episclera
- •Scleral Stroma
- •Lamina Fusca
- •1.3.1.3 Blood Supply and Emissary Canals
- •Vascular Distribution
- •Circulatory Dynamics
- •1.3.1.4 Nerve Supply
- •1.3.2 Ultramicroscopic Anatomy
- •1.3.2.1 Sclera
- •1.3.2.2 Vessels
- •1.4 Biochemistry
- •1.5 Immunohistochemistry
- •1.6 Biomechanics
- •1.7 Molecular Structure
- •1.7.1 Collagen
- •1.7.2 Elastin
- •1.7.3 Proteoglycans
- •1.7.4 Glycoproteins
- •1.7.6 Fibroblast Growth Regulation
- •1.8 Summary
- •References
- •2.1 General Immune Response Considerations
- •2.1.1 Components of the Adaptive Immune Response
- •2.1.1.1 Lymphocytes
- •T Lymphocytes
- •B Lymphocytes
- •Third-Population Lymphocytes or Null Lymphocytes
- •2.1.1.2 Monocytes/Macrophages
- •Phagocytosis
- •Antigen-Presenting Cells
- •2.1.1.3 Polymorphonuclear Granulocytes
- •Neutrophils
- •Eosinophils
- •Basophils/Mast Cells
- •2.1.1.4 Platelets
- •2.1.2 Immunoregulation
- •2.1.2.1 Major Histocompatibility Complex
- •2.1.2.2 Humoral Mechanisms: Antibodies
- •2.1.2.3 Cellular Mechanisms
- •2.1.2.4 Summary
- •2.1.3 Abnormalities of the Immune Response
- •2.1.3.1 Hypersensitivity Reactions
- •Type III Hypersensitivity Reactions
- •Systemic Immune Complex Disease
- •Local Immune Complex Disease (Arthus Reaction)
- •Type IV Hypersensitivity Reactions
- •2.1.3.2 Autoimmunity
- •Mechanisms of Autoimmunity
- •2.2 Connective Tissue and the Immune Response
- •2.2.1 Fibroblast Functions and the Immune Response
- •2.3 The Sclera and the Immune Response: Scleritis
- •2.3.1 Immune Characteristics of the Sclera
- •2.3.2 The Susceptible Host: Immunogenetics
- •2.3.3 Etiology
- •2.3.3.1 Exogenous Agents
- •Viruses
- •Mycobacteria
- •2.3.3.2 Endogenous Substances
- •Glycosaminoglycans
- •Collagen
- •2.3.4 Pathogenesis
- •2.4 Summary
- •References
- •3.1 Investigation of the Illness
- •3.1.1 Major Complaint and History of Present Illness
- •3.1.2 Past History
- •3.1.3 Family History
- •3.1.4 Past and Present Therapy History
- •3.1.5 Review of Systems
- •3.1.6 Systemic Examination
- •3.1.6.1 Head
- •3.1.6.2 Extremities
- •3.1.7 Ocular Examination
- •3.1.7.1 Episcleral and Scleral Examination
- •External Examination of the Eye in Daylight
- •Slit-Lamp Examination
- •Diffuse Illumination
- •Slit-Lamp Illumination
- •Red-Free Illumination
- •3.1.7.2 General Eye Examination
- •Visual Acuity
- •Pupils and Extraocular Muscles
- •Cornea
- •Anterior Uvea
- •Lens
- •Fundus
- •Intraocular Pressure
- •3.2 Diagnostic Tests
- •3.2.1 Blood Tests
- •3.2.1.1 Rheumatoid Factor
- •3.2.1.2 Anticyclic Citrullinated Peptide Antibodies
- •3.2.1.3 Antinuclear Antibodies
- •3.2.1.4 Antineutrophil Cytoplasmic Antibodies
- •3.2.1.5 Circulating Immune Complexes
- •Fluid-Phase Binding Assays
- •C1q-Binding Assay
- •Cell-Binding Assays
- •Raji Cell-Binding Assay
- •3.2.1.6 Complement
- •Quantitation Tests
- •Functional Tests
- •3.2.1.7 HLA Typing
- •3.2.1.8 Antibody Titers Against Infectious Organisms
- •3.2.1.9 Interferon-Gamma Release Assays (IGRAs)
- •3.2.2 Anterior Chamber Polymerase Chain Reaction Testing
- •3.2.3 Smears and Cultures
- •3.2.4 Skin Testing
- •3.2.5 Radiologic Studies
- •3.2.6.1 Anterior Segment Fluorescein Angiography Techniques
- •3.2.6.2 Normal Anterior Segment Fluorescein Angiography
- •Arterial Phase
- •Capillary Phase
- •Venous Phase
- •3.2.7 Anterior Segment Indocyanine Green Angiography
- •3.2.8 Other Imaging Studies
- •3.2.8.1 Ultrasonography
- •A-Scan Ultrasonography
- •B-Scan Ultrasonography
- •High-Frequency Ultrasound Biomicroscopy
- •3.2.8.2 Optical Coherence Tomography
- •3.2.8.3 Computer Tomography Scanning
- •3.2.8.4 Magnetic Resonance Imaging
- •3.3 Biopsy
- •3.3.1 Biopsy for Suspected Systemic Vasculitic Disease
- •3.4 Data Integration: Diagnosis
- •3.5 Therapeutic Plan
- •3.6 Summary
- •References
- •4.1 Episcleritis
- •4.1.1 Introduction
- •4.1.2 Patient Characteristics
- •4.1.3 Clinical Manifestations
- •4.1.4.1 Simple Episcleritis
- •4.1.4.2 Nodular Episcleritis
- •4.1.5 Associated Diseases
- •4.1.6 Precipitating Factors
- •4.2 Scleritis
- •4.2.1 Introduction
- •4.2.2 Patient Characteristics
- •4.2.3 Clinical Manifestations
- •4.2.4.1 Diffuse Anterior Scleritis
- •4.2.4.2 Nodular Anterior Scleritis
- •Differential Diagnosis
- •Paralimbic Scleromalacia
- •Senile Scleral Hyaline Plaques
- •4.2.4.5 Posterior Scleritis
- •Symptoms and Signs
- •Fundus Findings
- •Choroidal Folds
- •Subretinal Mass
- •Disk Edema and Macular Edema
- •Annular Ciliochoroidal Detachment and Serous Retinal Detachment
- •Associated Diseases
- •Complications
- •Ancillary Tests
- •Ultrasonography
- •Computerized Tomography (CT) Scanning
- •Fluorescein Angiography
- •Differential Diagnosis
- •Proptosis, Chemosis, Lid Swelling, and Limitation of Ocular Movements
- •Subretinal Mass
- •Choroidal Folds
- •Annular Ciliochoroidal Detachment and/or Serous Retinal Detachment
- •Disk and Macular Edema
- •4.2.5 Associated Diseases
- •4.2.6 Complications of Scleritis
- •4.2.6.1 Keratopathy
- •Peripheral Corneal Thinning
- •Stromal Keratitis
- •Peripheral Ulcerative Keratitis
- •4.2.6.2 Uveitis
- •4.2.6.3 Glaucoma
- •Angle-Closure Glaucoma
- •Open-Angle Glaucoma
- •Neovascular Glaucoma
- •4.2.6.4 Cataract
- •4.3 Summary
- •References
- •5: Pathology in Scleritis
- •5.1.3 Fibrinoid Necrosis
- •5.2.1 Pathology of Episcleritis
- •5.2.2 Pathology of Scleritis
- •5.2.2.1 Noninfectious Scleritis
- •Sclera
- •Cells
- •Extracellular Matrix
- •Vessels
- •Episclera
- •Conjunctiva
- •Iris, Ciliary Body, and Choroid
- •Cornea
- •Other Ocular Structures
- •Polyarteritis Nodosa
- •Allergic Granulomatous Angiitis (Churg–Strauss Syndrome)
- •Granulomatosis with Polyangiitis (Wegener)
- •Connective Tissue Diseases
- •Clinicopathological Correlates in Infectious Scleritis
- •Systemic Infections
- •Local Infections
- •5.3 Biopsy
- •5.3.1 Noninfectious Necrotizing Scleritis
- •5.3.2 Noninfectious Recurrent Diffuse or Nodular (Nonnecrotizing) Scleritis
- •5.3.3 Infectious Scleritis (Diffuse, Nodular, or Necrotizing Scleritis)
- •5.3.4 Biopsy Technique
- •5.4 Summary
- •References
- •6: Noninfectious Scleritis
- •6.1.1 Adult Rheumatoid Arthritis
- •6.1.1.1 Epidemiology
- •Signs and Symptoms of Joint Involvement
- •Extraarticular Systemic Manifestations
- •6.1.1.2 Systemic Manifestations
- •Onset
- •Tegument
- •Vessels
- •Lung
- •Heart
- •Nervous System
- •Lymph Nodes
- •Larynx
- •Felty’s Syndrome
- •Amyloidosis
- •Miscellaneous
- •6.1.1.3 Ocular Manifestations
- •Keratoconjunctivitis Sicca
- •Scleritis
- •Keratitis
- •Anterior Uveitis
- •Glaucoma
- •Cataract
- •Retinal, Choroidal, and Optic Nerve Changes
- •Motility Disturbances
- •Episcleritis
- •6.1.1.4 Laboratory Findings
- •Rheumatoid Factor
- •Antibodies to Cyclic Citrullinated Polypeptides
- •Complete Blood Count
- •Acute-Phase Reactants
- •Synovial Fluid Analysis
- •Circulating Immune Complexes
- •Antinuclear Antibodies
- •Complement
- •Cryoglobulins
- •Radiographic Evaluation
- •Diagnosis
- •6.1.2 Systemic Lupus Erythematosus
- •6.1.2.1 Epidemiology
- •6.1.2.2 Systemic Manifestations
- •Musculoskeletal
- •Tegument
- •Vessels
- •Kidney
- •Hearth
- •Nervous System
- •Lung
- •Miscellaneous
- •6.1.2.3 Ocular Involvement
- •Scleritis
- •Episcleritis
- •Other Ocular Findings
- •6.1.2.4 Laboratory Findings
- •6.1.2.5 Diagnosis
- •6.1.3 Ankylosing Spondylitis
- •6.1.3.1 Epidemiology
- •6.1.3.2 Systemic Manifestations
- •Articular Involvement
- •Extraarticular Systemic Manifestations
- •6.1.3.3 Ocular Manifestations
- •Anterior Uveitis
- •Scleritis
- •Episcleritis
- •6.1.3.5 Diagnosis
- •6.1.4 Reactive Arthritis (Reiter)
- •6.1.4.1 Epidemiology
- •6.1.4.2 Systemic Manifestations
- •Articular Involvement
- •Extraarticular Systemic Manifestations
- •6.1.4.3 Ocular Manifestations
- •Conjunctivitis
- •Anterior Uveitis
- •Scleritis
- •Episcleritis
- •Other Ocular Findings
- •6.1.4.4 Laboratory and Radiographic Findings
- •6.1.4.5 Diagnosis
- •6.1.5 Psoriatic Arthritis
- •6.1.5.1 Epidemiology
- •6.1.5.2 Systemic Manifestations
- •Skin and Articular Involvement
- •6.1.5.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.5.4 Laboratory and Radiographic Findings
- •6.1.5.5 Diagnosis
- •6.1.6.1 Epidemiology
- •6.1.6.2 Systemic Manifestations
- •Gastrointestinal and Articular Manifestations
- •6.1.6.3 Ocular Manifestations
- •Anterior Uveitis
- •Scleritis
- •Episcleritis
- •Keratitis
- •6.1.6.4 Laboratory and Joint Radiologic Findings
- •6.1.6.5 Diagnosis
- •6.1.7 Relapsing Polychondritis
- •6.1.7.1 Epidemiology
- •6.1.7.2 Systemic Manifestations
- •6.1.7.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.7.4 Laboratory Findings
- •6.1.7.5 Diagnosis
- •6.1.8 Polyarteritis Nodosa
- •6.1.8.1 Epidemiology
- •6.1.8.2 Systemic Manifestations
- •6.1.8.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.8.4 Laboratory and Angiographic Findings
- •6.1.8.5 Diagnosis
- •6.1.9.1 Epidemiology
- •6.1.9.2 Systemic Manifestations
- •6.1.9.3 Ocular Manifestations
- •6.1.9.4 Laboratory Findings
- •6.1.9.5 Diagnosis
- •6.1.10 Granulomatosis with Polyangiitis (Wegener)
- •6.1.10.1 Epidemiology
- •6.1.10.2 Clinical Manifestations
- •6.1.10.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.10.4 Laboratory Findings
- •6.1.10.5 Diagnosis
- •6.1.11 Adamantiades–Behçet’s Disease
- •6.1.11.1 Epidemiology
- •6.1.11.2 Systemic Manifestations
- •6.1.11.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.11.4 Laboratory Findings
- •6.1.11.5 Diagnosis
- •6.1.12 Giant-Cell Arteritis
- •6.1.12.1 Epidemiology
- •6.1.12.2 Systemic Manifestations
- •6.1.12.3 Ocular Manifestations
- •Scleritis
- •6.1.12.4 Laboratory Findings
- •6.1.12.5 Diagnosis
- •6.1.13 Cogan’s Syndrome
- •6.1.13.1 Clinical Manifestations
- •Scleritis
- •Episcleritis
- •6.1.13.2 Laboratory Findings
- •6.2.1 Rosacea
- •6.3.1 Gout
- •6.5 Chemical Injury-Associated Scleritis
- •6.6 Summary
- •References
- •7: Infectious Scleritis
- •7.1 Bacterial Scleritis
- •7.1.1.1 Pathogenesis
- •7.1.1.2 Organisms
- •7.1.1.3 Management
- •7.1.1.4 Therapy
- •7.1.1.5 Prognosis
- •7.1.1.6 Our Experience
- •7.1.2 Mycobacterial Scleritis
- •7.1.2.1 Atypical Mycobacterial Disease
- •7.1.2.2 Tuberculosis
- •7.1.2.3 Leprosy
- •7.1.3 Spirochetal Scleritis
- •7.1.3.1 Syphilis
- •Epidemiology
- •Pathogenesis and Clinical Features
- •Scleritis and Episcleritis
- •Diagnosis
- •Therapy
- •7.1.3.2 Lyme Disease
- •Epidemiology
- •Pathogenesis and Clinical Features
- •Scleritis and Episcleritis
- •Diagnosis
- •7.1.3.3 Treatment
- •7.1.4 Chlamydial Scleritis
- •7.1.5 Actinomycetic Scleritis
- •7.1.5.1 Nocardiosis
- •7.2 Fungal Scleritis
- •7.2.1 Filamentous and Dimorphic Fungal Scleritis
- •7.2.1.1 Pathogenesis
- •7.2.1.2 Organisms
- •7.2.1.3 Management
- •7.2.1.4 Therapy
- •7.2.1.5 Our Experience
- •7.3 Viral Scleritis
- •7.3.1 Herpes Scleritis
- •7.3.1.1 Herpes Zoster Scleritis
- •Epidemiology
- •Pathogenesis
- •Clinical Features
- •Scleritis
- •Episcleritis
- •Diagnosis
- •Treatment
- •7.3.1.2 Herpes Simplex Scleritis
- •Epidemiology
- •Pathogenesis
- •Clinical Features
- •Scleritis
- •Episcleritis
- •Diagnosis
- •Therapy
- •Our Experience
- •7.3.2 Mumps Scleritis
- •7.4 Parasitic Scleritis
- •7.4.1 Protozoal Scleritis
- •7.4.1.1 Acanthamoeba
- •7.4.1.2 Toxoplasmosis
- •7.4.2 Helminthic Scleritis
- •7.4.2.1 Toxocariasis
- •7.5 Summary
- •References
- •8.1 Scleral Deposits
- •8.1.1 Scleral Protein Deposition
- •8.1.1.1 Porphyria
- •8.1.1.2 Cystinosis
- •8.1.1.3 Alkaptonuria
- •8.1.1.4 Amyloidosis
- •8.1.2 Scleral Lipid Deposition
- •8.1.2.1 Familial Hypercholesterolemia and Histiocytosis X
- •8.1.2.2 Age-Related Degeneration
- •8.1.3 Scleral Carbohydrate Deposition
- •8.1.3.1 Mucopolysaccharidosis
- •8.1.4 Scleral Mineral Deposition: Calcium
- •8.1.4.1 Hyperparathyroidism
- •8.1.4.2 Other Causes of Hypercalcemia
- •8.1.4.3 Age-Related Degeneration
- •Senile Scleral Hyaline Plaques
- •8.1.5 Scleral Pigment Deposition: Bilirubin
- •8.1.5.1 Jaundice
- •8.2 Scleral Thinning (Blue Sclerae)
- •8.2.1 Scleral Thinning in Inherited or Congenital Diseases
- •8.2.1.1 Marfan’s Syndrome
- •8.2.1.2 Osteogenesis Imperfecta
- •8.2.1.3 Pseudoxanthoma Elasticum
- •8.2.1.4 Ehlers–Danlos Syndrome
- •8.2.1.5 Keratoconus
- •8.2.1.6 Buphthalmos
- •8.2.1.7 Coloboma
- •8.2.1.8 Myopia
- •8.2.2 Scleral Thinning in Acquired Diseases
- •8.2.2.2 Paralimbal Scleromalacia
- •8.3 Scleral Thickening
- •8.3.1 Nanophthalmos
- •8.3.2 Scleropachynsis
- •8.3.3 Phthisis Bulbi
- •8.4 Scleral Tumors
- •8.4.1 Dermoid Choristomas
- •8.4.2 Epithelial Tumors
- •8.4.2.1 Papillomas or Intraepithelial Epitheliomas
- •8.4.2.2 Squamous Cell Carcinoma
- •8.4.3 Dense Connective Tissue Tumors
- •8.4.3.1 Nodular Fasciitis
- •8.4.3.2 Fibroma
- •8.4.3.3 Fibrous Histiocytoma
- •8.4.3.4 Sarcomas
- •8.4.4 Vascular Tumors
- •8.4.4.1 Hemangiomas
- •8.4.4.2 Lymphangiomas
- •8.4.5 Blood Cell Tumors
- •8.4.5.1 Leukemia
- •8.4.5.2 Lymphoma and Lymphosarcoma
- •8.4.6 Nervous Tumors
- •8.4.6.2 Neurilemmoma (Schwannoma)
- •8.4.7 Pigmented Tumors
- •8.4.7.1 Nevus
- •8.4.7.2 Melanocytoma
- •8.4.8 Secondary Tumors
- •8.5 Summary
- •References
- •9.1 Treatment of Episcleritis
- •9.2 Treatment of Scleritis
- •9.2.1 Medical Treatment
- •9.2.1.1 Rheumatoid Arthritis
- •9.2.1.2 Systemic Lupus Erythematosus
- •9.2.1.3 Polyarteritis Nodosa
- •9.2.1.4 Granulomatosis with Polyangiitis (Wegener)
- •9.2.1.5 Relapsing Polychondritis
- •9.2.1.7 Posterior Scleritis
- •9.2.1.8 Infectious Scleritis
- •9.2.2 Ancillary Therapy
- •9.2.3 Drug Management Responsibility
- •9.2.4 Surgical Treatment
- •9.3 Summary
- •References
- •Index
116 |
4 Clinical Considerations of Episcleritis and Scleritis |
|
|
Fig. 4.23 Posterior scleritis with annular retinochoroidal and serous retinal detachment
Fig. 4.24 Same eye as shown in Fig. 4.23. Fluorescein angiogram, conÞrming the Þndings described in Fig. 4.23
head down; despite a cloudy subretinal ßuid, a pale gray subretinal mass with a surrounding dark gray line and an overlying normal choroidal vascular pattern may sometimes be visible through the poorly mobile bullous serous retinal detachment. The bullous serous retinal detachments do not have retinal holes or Þxed retinal folds. The ciliochoroidal or retinal detachments usually resolve completely with prompt and aggressive treatment of the scleritis. Although they may disappear within hours, they are often absorbed slowly over a period of several weeks or months, leaving only a diffuse pigmentation in the affected area; however, if the macular area has been
involved, loss of vision will remain as a permanent sequela [5].
Associated Diseases
Disease association in posterior scleritis is less common than in anterior scleritis [2, 7]. In our series of 31 patients with posterior scleritis, 6 (19%) were found to have an associated systemic disease (Table 4.4). This is in contrast to the 36% disease association found in our patients with anterior scleritis. Diagnoses included psoriatic arthritis in two patients, HLA-B27+ (without spondyloarthropathy)-associated scleritis in one patient, arthritis and inßammatory bowel disease in one patient, systemic lupus erythematosus in one patient, and tubulointerstitial nephritis and uveitis syndrome in one patient (Table 4.5).
Complications
Posterior uveitis is universally present in posterior scleral inßammation because the choroid is always involved by the adjacent posterior scleral inßammation; [2, 6] anterior uveitis may also appear, but it is often associated with concomitant anterior scleritis. Glaucoma, particularly in combination with uveitis, is considered to be an ominous sign in the course of scleritis because its presence indicates a more diffuse and severe process [3]. Whether caused by anterior uveitis, ciliochoroidal detachment, angle neovascularization, or chronic use of steroids, increased intraocular pressure in posterior scleritis may result in the development of irreversible optic nerve damage [9, 100].
Ancillary Tests
Ultrasonography
Ultrasonography is the most useful test in the diagnosis of posterior scleritis because it shows the ßattening and thickening of the posterior coats of the eye (choroid and sclera) associated with retrobulbar edema [84, 86, 89, 101] (Fig. 4.25). Occasionally, retinal and choroidal detachments may also be detected. B-scan ultrasonography shows multiple internal echoes in the area of the scleral thickening and lack of echoes in the area of retrobulbar edema; the multiple echoes remain after sound beam attenuation, indicating high
4.2 Scleritis |
117 |
|
|
Fig. 4.25 B scan ultrasonogram with superimposed A scan proÞle. Note the thickening of the retinal choroid layer and the edema in TenonÕs space
Fig. 4.26 Ultrasonogram of a patient with posterior scleritis. Note particularly the A scan tracing showing the multiple retrobulbar echoes. The multiple echoes in the area of the sclera indicate high internal reßectivity of the sclera
Fig. 4.27 Ultrasonogram of choroidal thickening in a patient with chronic uveitis without scleritis. Note particularly the A scan tracing showing a lack of high internal reßectivity in the area of the sclera with sound beam attenuation
Fig. 4.28 Ultrasonogram ÒTÓ sign formed by the sonagraphically, empty space occupied by the optic nerve and the edematous TenonÕs space adjacent to the optic nerve
internal reßectivity of the scleral mass (Fig. 4.26). On the other hand, in choroidal thickening without scleral thickening, the echoes do not remain after sound beam attenuation (low internal reßectivity) (Fig. 4.27). When retrobulbar edema surrounds the optic nerve, the ÒTÓ sign may appear, which consists of a lack of echoes in the edematous TenonÕs space and adjacent optic nerve (Fig. 4.28). A-scan ultrasonography shows highamplitude internal spikes in the area of the scleral thickening and low-amplitude internal spikes in the area of retrobulbar edema. The combination of both A scan and B scan techniques gives the most useful results in distinguishing posterior scleritis
from orbital, choroidal, and retinal entities that clinically may mimic it (Tables 4.7Ð4.9) [86].
Computerized Tomography (CT) Scanning
Computerized tomography also shows scleral thickening, the image of which can be enhanced by radiopaque medium injection [86, 102]. Retrobulbar edema may also be seen. The ability of computerized tomography to delineate extraocular muscles, lacrimal glands, optic nerves, scleral coats, orbital walls, and paranasal tissues helps to detect extraocular muscle or lacrimal gland enlargement, optic nerve or scleral inßammation, bone erosion, and sinus involvement,
Table 4.7 Differential diagnosis of posterior scleritis: proptosis, chemosis, lid swelling, and limitation of ocular movements
|
|
|
Acute diffuse idiopathic orbital |
|
Parameter |
Posterior scleritis |
Orbital tumor |
inßammation |
Thyroid ophthalmopathy |
Sex predilection |
Female |
Ð |
Ð |
Female |
|
|
|
|
|
Age predilection |
Middle aged and elderly |
Ð |
Ð |
Middle aged and elderly |
|
|
|
|
|
Laterality |
Unilateral |
Unilateral |
Unilateral |
Bilateral |
Onset |
Gradual |
Gradual |
Acute |
Gradual |
|
|
|
|
|
Pain |
Variable |
± |
Variable |
− |
|
|
|
|
|
Tenderness |
+ |
− |
+ |
− |
|
|
|
|
|
Anterior scleritis |
+ |
− |
− |
− |
Visual loss |
+ |
± |
± |
Variable |
|
|
|
|
|
Fundus mass |
Variable |
± |
± |
− |
|
|
|
|
|
Color of the mass |
Orange |
Orange |
Orange |
− |
Proptosis |
± |
+ |
+ |
+ |
Motility disturbance |
± |
+ |
+ |
+ |
|
|
|
|
|
Conjunctival chemosis |
± |
+ |
+ |
− |
|
|
|
|
|
Lid edema |
± |
+ |
+ |
− |
Pigment epithelium |
Yellowish nodules |
Normal |
Normal |
Normal |
|
|
|
|
|
Disk edema |
+ |
± |
± |
± |
|
|
|
|
|
Uveitis |
+ |
− |
± |
− |
|
|
|
|
|
Choroidal folds |
+ |
± |
± |
± |
Serous retinal detachment |
+ |
− |
± |
− |
|
|
|
|
|
Fluorescein angiography |
Multiple small leaks |
Normal |
Normal |
Normal |
(other than choroidal folds) |
|
|
|
|
|
|
|
|
|
Ultrasound |
Scleral and choroid thickening |
Orbital mass |
Orbital mass (low reßectivity) |
EOM enlargement |
|
retrobulbar edema (high reßectivity) |
|
and/or EOM enlargement |
|
|
|
|
|
|
CT scan |
Scleral and choroid thickening |
Orbital mass with sinus |
Orbital mass without sinus involvement |
EOM enlargement |
|
|
involvement/bone erosion |
or bone erosion; EOM enlargement |
|
|
|
|
|
|
Biopsy indication |
No biopsy |
Biopsy |
Biopsy |
No biopsy |
|
|
|
|
|
Response to steroids |
Good |
Absent |
Very good |
Variable |
EOM extraocular muscle |
|
|
|
|
118
Scleritis and Episcleritis of Considerations Clinical 4
Table 4.8 Differential diagnosis of posterior scleritis: subretinal mass
Parameter |
Posterior scleritis |
Choroidal melanoma |
Metastatic uveal carcinoma |
Choroidal hemangioma |
Sex predilection |
Female |
Ð |
Ð |
Ð |
|
|
|
|
|
Age predilection |
Middle aged and elderly |
Elderly |
Middle aged and elderly |
Middle aged and elderly |
|
|
|
|
|
Laterality |
Unilateral |
Unilateral |
Unilateral |
Unilateral |
Onset |
Gradual |
Gradual |
Gradual |
Gradual |
|
|
|
|
|
Pain |
Variable |
Ð |
Ð |
Ð |
|
|
|
|
|
Tenderness |
+ |
− |
− |
− |
Anterior scleritis |
+ |
− |
− |
− |
Visual loss |
+ |
Variable |
Variable |
Variable |
|
|
|
|
|
Color of the mass |
Orange |
Hyperor hypo-pigmented |
Hypopigmented |
Pinkish orange |
|
|
|
|
|
Overlying retina |
Yellow deposits |
Orange pigment |
Dark mottling |
Cystoid edema |
Proptosis |
± |
− |
− |
− |
|
|
|
|
|
Motility disturbance |
± |
− |
− |
− |
|
|
|
|
|
Conjunctival chemosis |
± |
− |
− |
− |
|
|
|
|
|
Lid edema |
± |
− |
− |
− |
Disk edema |
+ |
− |
− |
− |
|
|
|
|
|
Uveitis |
+ |
− |
− |
− |
|
|
|
|
|
Choroidal folds |
+ |
± |
± |
− |
Serous retinal detachment/ |
+/cloudy |
+/clear |
+/clear |
+/clear |
subretinal ßuid |
|
|
|
|
|
|
|
|
|
Fluorescein angiography |
Small leaks; intrinsic vasculature |
Small leaks |
Small leaks |
Small leaks; early ßuorescence |
(other than choroidal folds) |
|
|
|
prior to Þlling retinal vessels |
|
|
|
|
|
Ultrasound |
Scleral and choroid thickening |
Choroidal mass |
Choroidal mass |
Choroidal mass (high reßectivity); |
|
(high reßectivity) retrobulbar edema |
(low reßectivity); |
(moderate reßectivity); |
no retrobulbar edema |
|
|
no retrobulbar edema |
no retrobulbar edema |
|
|
|
|
|
|
Response to steroids |
Good |
Absent |
Absent |
Absent |
|
|
|
|
|
Scleritis 2.4
119
Table 4.9 Differential diagnosis of posterior scleritis: serous detachment of choroid, ciliary body, and retina
|
|
|
|
Idiopathic centralserous |
Parameter |
Posterior scleritis |
Uveal effusion syndrome |
VogtÐKoyanagiÐHarada syndrome |
chorio retinopathy |
|
|
|
|
|
Sex predilection |
Female |
Male |
Ð |
Male |
Age predilection |
Middle aged and elderly |
Middle aged |
Young and middle aged |
Middle aged |
Race predilection |
Ð |
Ð |
Oriental and pigmented |
Caucasian |
|
|
|
|
|
Laterality |
Unilateral |
Bilateral |
Bilateral |
Unilateral |
|
|
|
|
|
Pain |
Variable |
− |
− (photophobia) |
− |
Anterior scleritis |
+ |
− |
− |
− |
|
|
|
|
|
Uveitis |
+ |
− |
+ |
− |
|
|
|
|
|
Disk edema |
+ |
+ |
+ |
− |
|
|
|
|
|
Pigment epithelium |
Yellowish nodules |
ÒLeopard spotsÓ |
Depigmented or |
Serous detachments |
|
|
|
hyperpigmented lines |
pigment epithelium |
|
|
|
|
|
Serous retinal detachment |
+ |
+ |
+ |
+ |
Serous ciliochoroidal |
+ |
+ |
± |
− |
detachment |
|
|
|
|
Subretinal ßuid |
Cloudy |
Clear |
Cloudy |
Clear |
|
|
|
|
|
Fluorescein angiography |
Multiple small leaks |
Slow choroidal perfusion; |
Multiple small leaks; late-staining |
Serous detachments |
|
|
occasional leaks |
subretinal ßuid |
pigment epithelium |
Ultrasound |
Scleral and choroidal thickening (high |
Choroid thickened; serous |
Choroid thickened (low internal |
Serous retinal |
|
reßectivity); retrobulbar edema; serous |
cilio-choroid and retinal |
reßectivity) Serous retinal |
detachment |
|
ciliochoroid and retinal detachment |
detachment |
detachment |
|
|
|
|
|
|
Miscellaneous |
Collagen vascular disease association |
High protein level in CSF |
Headaches, fever dysacousis, |
Anxiety |
|
|
(50% of cases) |
vitiligo, meningism (50% of cases) |
|
|
|
|
|
|
120
Scleritis and Episcleritis of Considerations Clinical 4
