Clinical Ophthalmic Oncology: Orbital Tumors

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