Clinical ophthalmic oncology. Orbital tumors
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Preface Acknowledgments Contents Contributors Editors 1: Examination Techniques Introduction History Examination External Examination Pupils Extraocular Motility Eyelid Position and Function Globe Position Proptosis Hyperglobus or Hypoglobus Palpation Resistance to Globe Retropulsion Slit Lamp Examination Fundus Examination Cranial Nerves V and VII Lacrimal System Nasal Endoscopy Special Issues in Examination of Children Complete Eye Examination Orbital Examination Globe Displacement Summary 2: Classification of Orbital Tumors Introduction Differential Diagnosis of Orbital Tumors Clinicopathological Classification of Orbital Tumors Cystic Lesions Vascular Lesions Myogenic Tumors Lipomatous and Myxomatous Tumors Primary Melanocytic Tumors Tumors of the Lacrimal Gland Tumors of the Lacrimal Sac Lymphoproliferative Tumors Peripheral Nerve Tumors Optic Nerve, Meningeal, and Other Neural Tumors Fibrous Connective Tissue (Fibrohistiocytic Lesions) Histiocytic Tumors Primary Bone Tumors Benign Fibro-osseous Lesions Benign Cartilaginous Tumors Reactive Bone Lesions Bone Neoplasms Bone Vascular Tumors Miscellaneous Bone Tumors Metastatic Tumors to the Orbit Adult Metastatic Disease Metastatic Lesions in Children Secondary Orbital Tumors Imaging Classification of Orbital Tumors Summary References 3: Differential Diagnosis in Children Introduction History Presenting Symptoms and Complaints Rate of Onset Past Medical History Examination Pulsation and Bruits Periorbital Changes Head and Neck Examination Laboratory Evaluation Diagnostic Imaging Biopsy Summary References 4: Orbital Tumors: Differential Diagnosis in Adults Introduction History Rate of Onset Examination Nature of Proptosis and Dystopia The Presence of Pain Changes in Vision Gaze Restriction Diagnostic Imaging Imaging Location: Optic Nerve and Nerve Sheath Imaging Location: Intraconal Space Imaging Location: Extraocular Muscles Imaging Location: Extraconal Space Imaging Location: Lacrimal Gland and Lacrimal Gland Fossa Imaging Location: Lacrimal Sac Imaging Location: Orbital Apex Imaging Location: Orbital Bones Imaging Location: Eyelid, Globe, Sinus, and Brain Imaging Location: Diffuse Lesions Conclusion References 5: Imaging Techniques Introduction Techniques of Orbital Imaging Ultrasonography Color Doppler Imaging (CDI) Computed Tomography Conventional Computed Tomography Helical Computed Tomography Three-Dimensional (3D) Computed Tomography Magnetic Resonance Imaging Interpretation of Imaging Studies Location Appearance Content Contrast Enhancement Bone Characteristics Bone Molding Bone Erosion Bone Lysis Bone Infiltration Hyperostosis Radiological Differential Diagnosis Well-Circumscribed Solid Orbital Lesions Ill-Defined Solid Orbital Lesions Well-Circumscribed Cystic Lesions Enlarged Optic Nerve Enlarged Lacrimal Gland Enlarged Extraocular Muscles Anomalies of Bony Orbit Summary References 6: Specific Orbital Inflammatory Diseases Introduction Granulomatous Orbital Diseases Sarcoidosis Xanthogranulomatous Disease Sjögren’s Syndrome Vasculitic Diseases Granulomatosis with Polyangiitis Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) IgG4-Related Disease References 7: Idiopathic Orbital Inflammation Introduction Historical Perspective and Terminology Pathogenesis Epidemiology Clinical Features Clinical Subtypes Idiopathic Dacryoadenitis Idiopathic Orbital Myositis Orbital Fat IOI Diagnostic Evaluation Imaging Laboratory Testing Known History of Orbital-Related Systemic Disease Biopsy Histopathology The Corticosteroid Trial Treatment Surgical Debulking Corticosteroids Radiation Therapy Immunomodulatory Drugs Conclusion References 8: Orbital Vascular Anomalies Introduction Orbital Vascular Tumors Infantile Hemangioma Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Pharmacologic Treatment Laser Photocoagulation Surgery Prognosis Congenital Hemangioma (CH) Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Pyogenic Granuloma Clinical Features Diagnostic Evaluation Treatment Prognosis Angiosarcoma Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Prognosis Orbital Vascular Malformations Lymphatic Malformations Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Prognosis Venous Malformations Clinical Features Diagnostic Evaluation Treatment Surgery Sclerotherapy Other Methods Arteriovenous Malformations Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Prognosis Orbital Cavernous Venous Malformations Clinical Features Diagnostics Differential Diagnosis Treatment Prognosis Orbital Hemangiopericytoma Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Prognosis References 9: Benign Orbital Tumors Introduction Conjunctival Epithelial Cysts Introduction Clinical Features Differential Diagnosis Diagnostic Evaluation Treatment Dermoid and Epidermoid Cysts Introduction Clinical Features Differential Diagnosis Diagnostic Evaluation Treatment Intradiploic Arachnoid Cyst Introduction Clinical Features Differential Diagnosis Diagnostic Evaluation Treatment Mucocele Introduction Clinical Features Frontoethmoid Mucocele Sphenoid and Posterior Ethmoid Sinus Mucocele Maxillary Mucocele Diagnostic Evaluation Treatment Sinonasal Inverted Papilloma Introduction Clinical Features Diagnostic Evaluation Treatment Cholesterol Granuloma Introduction Clinical Features Diagnostic Evaluation Treatment Orbital Cephalocele Introduction Clinical Features Anterior Cephalocele Basal Cephalocele Posterior Cephalocele Diagnostic Evaluation Treatment Neurofibroma and Neurilemmoma (Schwannoma) Introduction Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Solitary Fibrous Tumor Introduction Clinical Features Diagnostic Evaluation Treatment Meningioma Introduction Clinical Features Diagnostic Evaluation Treatment Teratoma Introduction Clinical Features Diagnostic Evaluation Treatment Fibrous Histiocytoma Introduction Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Osteoma Introduction Clinical Features Diagnostic Evaluation Treatment Fibrous Dysplasia Introduction Monostotic Fibrous Dysplasia Polyostotic Fibrous Dysplasia McCune-Albright Syndrome Clinical Features Diagnostic Evaluation Differential Diagnosis Treatment Aneurysmal Bone Cyst Introduction Clinical Features Diagnostic Evaluation Treatment Leiomyoma Introduction Clinical Features Diagnostic Evaluation Treatment References 10: Orbital Meningioma Introduction Primary Orbital Meningioma Optic Nerve Sheath Meningioma Sphenoid Wing Meningioma with Orbital Involvement Pathophysiology Ionized Radiation Genetic Abnormalities Hormonal Factors Viruses Head Injury and Electromagnetic Fields Epidemiology Natural History Clinical Features Differential Diagnosis Diagnostic Evaluation Laboratory Studies Imaging (Figs. 10.1 and 10.2) Other Tests Management Surgery (Figs. 10.1, 10.2, and 10.3) Medical Treatment Radiotherapy Histology Mortality and Morbidity Prognosis Conclusion References 11: Tumors of the Optic Nerve Introduction Anterior Visual Pathway Glioma Association with Neurofibromatosis Type 1 Clinical Features Age Distribution Sex Distribution Location Signs and Symptoms Diagnostic Evaluation Neuroimaging Computed Tomography (CT) Magnetic Resonance Imaging (MRI) Histopathology Treatment Options Observation Surgery Radiotherapy Chemotherapy Prognosis Glioma Confined to the Optic Nerve Gliomas with Extension to the Chiasm Gliomas with Extension to the Chiasm and with Invasion of the Hypothalamus Malignant Optic Nerve Glioma Clinical Features Age Distribution Sex Distribution Location Signs and Symptoms Diagnostic Evaluation Histopathology Treatment Prognosis Optic Nerve Sheath Meningioma Association with Neurofibromatosis Type 1 Clinical Features Age Distribution Sex Distribution Laterality Sites of Origin Signs and Symptoms Diagnostic Evaluation Computed Tomography Magnetic Resonance Imaging Histopathology Treatment Observation Surgery Radiotherapy Prognosis References 12: Optic Nerve Sheath Fenestration and Optic Nerve Biopsy Introduction Definition of ONSF Mechanism of ONSF Indications for ONSF History of ONSF Development of the Superomedial Approach to the Optic Nerve Anatomy Preparation Instruments (Fig. 12.2) Patient Positioning Anesthesia Surgical Technique (Figs. 12.3a, b and 12.4) Incision Surgical Dissection Fenestration of the Optic Nerve Sheath (Fig. 12.6a, b) Effect of Unilateral ONSF Removal of Intraconal Tumors Bleeding Closure Postoperative Care Complications Comparison to Other Surgical Approaches to ONSF (Boxes 12.1, 12.2, and 12.3) Medial Transconjunctival Approach (Galbraith and Sullivan Procedure) Lateral Orbital Approach with Bone Flap (Tse et al. Procedure) (Fig. 12.9) Lateral Orbital Approach Without Bone Flap (Patel and Anderson Procedure) Advantages of the Superomedial Approach to the Optic Nerve and Intraconal Tumors Surgical Tips for Successful ONSF with the Superomedial Approach Conclusion References 13: Lacrimal Gland Tumors Introduction Epidemiology Clinical Features Symptoms Signs Diagnostic Evaluation Benign Tumors Malignant Tumors Pathology Pleomorphic Adenoma Adenoid Cystic Carcinoma Differential Diagnosis Treatment Pleomorphic Adenoma Adenoid Cystic Carcinoma Malignant Mixed Tumor Metastatic Tumors Prognosis Pleomorphic Adenoma Carcinomas Systemic and Metastatic Tumors Genomics Conclusion References 14: Lacrimal Sac Tumors Introduction Clinical Features Diagnostic Evaluation Histopathological Classification Treatment Clinical Course References 15: Orbital and Adnexal Lymphoma Introduction Epidemiological Aspects Etiology and Pathogenesis: B-cell Biology and Lymphomagenesis Classification Clinical Features Symptoms Signs Diagnostic Evaluation Local Imaging Studies Staging Procedures Differential Diagnosis Pathologic Features Rare Variants Langerhans Cell Histiocytosis Rosai-Dorfman Syndrome T-Cell Lymphoma Burkitt Lymphoma Treatment Surgery Cryotherapy Radiation Chemotherapy Immunotherapy Antimicrobial Treatment Prognosis Future Research References 16: Malignant Orbital Tumors Introduction Esthesioneuroblastoma Introduction Clinical Features Diagnostic Evaluation Treatment Malignant Peripheral Nerve Sheath Tumor Introduction Clinical Features Diagnostic Evaluation Treatment Alveolar Soft Part Sarcoma Introduction Clinical Features Diagnostic Evaluation Treatment Osteosarcoma Introduction Clinical Features Diagnostic Evaluation Treatment Malignant Fibrous Histiocytoma Leiomyosarcoma Introduction Clinical Features Diagnostic Evaluation Treatment Liposarcoma Introduction Clinical Features Diagnostic Evaluation Treatment Secondary Orbital Tumors Introduction Clinical Features Basal Cell Carcinoma Squamous Cell Carcinoma Melanoma Sebaceous Carcinoma Merkel Cell Carcinoma Diagnostic Evaluation Treatment Orbital Metastases: Adults Introduction Clinical Features Diagnostic Evaluation Treatment Orbital Metastases: Children Introduction Neuroblastoma Ewing’s Sarcoma References 17: Orbital Rhabdomyosarcoma Introduction Etiology Pathogenesis Clinical Features Symptoms Signs Diagnostic Evaluation Imaging Tissue Diagnosis: Biopsy Salient Diagnostic Findings Histology Light Microscope Immunohistochemistry Molecular Analysis Differential Diagnosis Treatment Surgery Chemotherapy Radiotherapy Follow-Up Prognosis Nonrhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) (Fig. 17.8a–d) Future Research New Proposed Classification Improving the Standard Treatment Conclusion References 18: Enucleation for Ocular Tumors Introduction Indications Preoperative Preparation Technique Results and Complications Tumor Recurrence Complications Postoperative Pain Control Alternative Technique of Enucleation Follow-Up Conclusion References 19: Orbital Exenteration Introduction Indications Eyelid Malignant Tumors Conjunctival Malignant Tumors Intraocular Malignant Tumors Orbital Malignant Tumors Paranasal Sinus Malignant Tumors Miscellaneous Diseases Preoperative Preparation Technique Total Exenteration (Including the Eyelids) Total Exenteration (Eyelid Sparing) Subtotal Exenteration Eyelid- and Conjunctiva-Sparing Exenteration Reconstruction of the Orbital Cavity Local Reconstruction Techniques Laissez-Faire Split-Thickness Skin Graft Dermis Fat Graft Locoregional Reconstruction Techniques Cheek Advancement Flap Temporalis Muscle Transfer Distal Reconstruction Technique (Free Flap) Novel Technique Complications (Table 19.1) Intraoperative Persistent Hemorrhage Management of Cerebrospinal Fluid (CSF) Leak Management of Sino-Orbital Fistula Management of Other Complications Cosmetic Rehabilitation Titanium Osseointegrated Implants Ocular Prosthesis (Prosthetic Shell) Prognosis References 20: Principles of Orbital Surgery Introduction Anatomical Limitations: The ‘Conoid of View’ in Orbital Surgery Orbital Surgery: Risks to Visual Functions Preoperative Counselling Perioperative Medical and Anaesthetic Considerations The Six Essential Orbitotomies: An Overview Anterior Orbitotomy: Upper Eyelid Skin-Crease Approach Anterior Orbitotomy: Swinging Lower Eyelid Approach The ‘High’ Variant of the Swinging Lower Eyelid Approach The ‘Low’ Variant of the Swinging Lower Eyelid Approach Anterior Orbitotomy: Lateral Canthotomy Approach Lateral Orbitotomy with Bone Mobilization Transconjunctival Retrocaruncular Approach to the Medial Orbit The Conjunctival Peritomy Approach Early Postoperative Management and Counselling Summary References 21: Orbital Implants Introduction Historical Perspective Porous Orbital Implants Hydroxyapatite Implants Synthetic Porous Polyethylene Implants Synthetic Hydroxyapatite Implants Bioceramic Implants Porous Versus Nonporous Implants Implant Selection Socket Volume Considerations Orbital Implant Wrapping Pegging Porous Orbital Implants Summary References 22: Orbital Prostheses Introduction Pre-exenteration Decision-Making Intraoperative Considerations Postoperative Considerations Prosthesis Fabrication Using an Orbital Prosthesis Historical Review and Advances in Realistic Prostheses References 23: Ocular Prostheses Introduction Historical Perspective Psychological Aspects of Loss of an Eye Surgical Aspects of Importance to the Ocularist Referral to the Ocularist Materials Used to Make Prosthetic Eyes Fabrication of an Ocular Prosthesis (Fig. 23.3) The Ideal Prosthesis Prosthetic Mobility Advances and Modifications of Prostheses Care for Ocular Prostheses, Eyelids, and Socket Long-Term Care Future Research and Advances References Index
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