1.1.1 A Few Words of Introduction
1.1.2 Orienting, Transillumination, Cutting and Embedding
1.1.3 The Ordinary Sequence in Which to Describe the Eye in Histopathology
1.2 Embryology, Anatomy and Development
1.4 Congenital Abnormalities
1.4.2 Congenital Cystic Eye
1.4.6 Synophthalmia and Cyclopia
1.4.7 Coloboma of Iris, Choroid, Retina and Optic Nerve
1.4.7.2 Retino-Uveal Coloboma
1.4.7.3 Optic Disc Coloboma
1.4.8 Microphthalmia with Cyst
1.4.9 Anterior Cleavage Syndrome
1.4.11 Chromosomal Abnormities Involving the Eyeball
1.5.1 Panophthalmia and Endophthalmia
1.5.2 Bacterial Pyogenic Endophthalmitis
1.5.5 Granulomatous Inflammation
1.6.1 Irreparable Eye: Large Penetrating/Perforating Wounds of the Eyeball Walls
1.6.2 Battered Child Syndrome
1.6.4 Sympathetic Ophthalmia
1.6.5 Phacoanaphylactic Endophthalmitis
1.9 Artificial Eyes, Orbital Implants and Dry Socket
2.2 Embryology, Anatomy, and Development
2.3 Congenital Abnormalities
2.7.1 Squamous Epithelial Lesions
2.7.1.1 Conjunctival Papillomas
2.7.1.2 Ocular Surface Squamous Neoplasia (OSSN)
2.7.5.1 Conjunctival Melanoma
3.2 Embryology, Anatomy, and Development
3.2.1 Embryology and Development
3.2.2.2 Superficial Cell Layer
3.2.2.5 Basement Membrane
3.2.2.10 Blood Supply and Lymphatic Drainage
3.2.2.8 Descemet’s Membrane
3.2.2.12 Limbal Stem Cells
3.3 Congenital Abnormalities
3.3.1 Abnormalities of Corneal Size
3.3.2 Abnormalities of Corneal Shape
3.3.3 Abnormalities of the Anterior Segment of the Eye with Corneal Involvement
3.3.3.1 Axenfeld-Rieger Syndrome
3.3.3.3 Posterior Keratoconus
3.3.3.4 Congenital Anterior Staphyloma
3.3.3.5 Congenital Keratectasia
3.4.1 General Considerations
3.4.1.1 Corneal Ulceration and Sequelae
Scar Formation (Macula and Leucoma)
3.4.1.2 Corneal Neovascularisation
3.4.1.3 Corneal Lymphangiogenesis
3.4.2 Ocular Surface Disease
3.4.3 Immunologic Disease
3.4.3.1 Atopic/Vernal Keratoconjunctivitis
3.4.3.2 Peripheral Ulcerative Keratitis
Peripheral Ulcerative Keratitis Associated with Collagen Vascular Disease
Staphylococcus-Associated Blepharokeratoconjunctivitis
3.4.3.3 Interstitial Keratitis
Syphilitic Stromal Keratitis
3.4.4.1 Bacterial Keratitis
Adenoviral Keratoconjunctivitis Including Epidemic Keratoconjunctivitis
Herpes Simplex Viral Keratitis
Varicella Zoster Virus Keratitis
3.4.4.4 Parasitic Keratitis
3.5.1 Chemical and Thermal Injury
Corneal Abrasion Including Recurrent Erosion Syndrome
Perforating Corneal Wounds
Wound Healing After Corneal Perforating Injury
3.5.3 Postsurgical Pathology
3.5.3.1 Corneal Collagen Cross-Linking
3.5.3.2 Intrastromal Ring Implants
3.5.3.3 Radial Keratotomy (RK)
3.5.3.5 Photoablative Refractive Surgery with Excimer Laser
Laser In Situ Keratomileusis
Phototherapeutic and Photorefractive Keratectomy
3.5.3.7 Conjunctival Flap
3.5.3.8 Amniotic Membrane Transplantation
Anterior Lamellar Keratoplasty
Posterior Lamellar Keratoplasty
3.5.3.9 Limbal Stem Cell Transplantation
3.5.3.11 Keratoprosthesis
3.6 Degenerations and Dystrophies
3.6.1.1 Keratoconus and Keratoglobus
3.6.1.2 Pellucid Marginal Degeneration
3.6.1.3 Bullous Keratopathy
3.6.1.6 Peripheral Hypertrophic Subepithelial Degeneration
3.6.1.7 Salzmann’s Nodular Degeneration
3.6.1.10 Climatic Droplet Keratopathy
3.6.2.1 Epithelial and Subepithelial Dystrophies
Epithelial Basement Membrane Dystrophy
Epithelial Recurrent Erosion Dystrophy
Meesmann’s Corneal Dystrophy
Subepithelial Mucinous Corneal Dystrophy
Gelatinous Drop-Like Corneal Dystrophy
Lisch Epithelial Corneal Dystrophy
3.6.2.2 Dystrophies Affecting Predominantly the Region of Bowman’s Layer
Reis-Bỹcklers’ Corneal Dystrophy
Grayson-Wilbrandt Corneal Dystrophy
Thiel-Behnke Corneal Dystrophy
3.6.2.3 Dystrophies Affecting Primarily Corneal Stroma
Lattice Corneal Dystrophy, Classic and Variants
Granular Corneal Dystrophy, Type 1 and Variants
Granular Corneal Dystrophy, Type 2
Macular Corneal Dystrophy
Congenital Stromal Corneal Dystrophy
Schnyder’s Corneal Dystrophy
Central Cloudy Dystrophy of Franỗois
Posterior Amorphous Corneal Dystrophy
Pre-Descemet Corneal Dystrophy
3.6.2.4 Descemet’s Membrane and Endothelial Dystrophies
Fuchs’ Endothelial Corneal Dystrophy
Posterior Polymorphous Corneal Dystrophy
Congenital Hereditary Endothelial Dystrophy
X-Linked Endothelial Corneal Dystrophy
4.2 Embryology, Anatomy and Development
4.3 Congenital Abnormalities
4.3.1 Synophthalmia and Cyclopia
4.3.2 Microphthalmos and Nanophthalmos
4.3.3 Osteogenesis Imperfecta
4.3.5 Systemic Mucopolysaccharidosis (MPS)
4.3.6 Ocular Melanosis and Oculodermal Melanocytosis
4.6.1 Melanoma-Associated Spongiform Scleropathy (MASS)
4.6.6.1 Senile Scleral Plaques
4.6.6.2 Other Causes of Calcium Deposition
4.6.7 Age-Related Degeneration
4.7.1.1 Episcleral Osseous Choristoma
4.7.1.2 Nodular Fasciitis
4.7.1.4 Nerve Sheath Tumours
5.2 Embryology, Anatomy, and Development
5.4.1.1 Cortical Cataract
5.4.1.2 Nuclear Cataract (Nuclear Sclerosis)
5.4.1.3 Posterior Subcapsular Cataract
5.5 Tissue Processing and Artifacts
6.2 Anatomy and Embryology
6.3 Developmental Glaucoma
6.3.1 Primary Congenital Glaucoma
6.3.2 Axenfeld and Rieger Anomaly (AXRA)
6.4 Primary Open-Angle Glaucoma (POAG)
6.5 Primary Angle-Closure Glaucoma (PACG)
6.5.1 Acute Primary Angle-Closure Glaucoma
6.5.2 Chronic Angle-Closure Glaucoma
6.6 Secondary Open-Angle Glaucoma
6.6.1.1 Neovascularisation Glaucoma
6.6.1.2 Endothelial Layer
Iridocorneal Endothelial Syndrome
Pseudoexfoliation Glaucoma
6.6.2.2 Other Trabecular Glaucoma Causes
Uveitis, e.g. Fuchs heterochromic cyclitis
6.6.2.4 Lens Particle Glaucoma
6.6.3 Alterations of the Trabecular Meshwork
6.6.3.1 Steroid-Induced Glaucoma
6.6.3.2 Trauma (Angle Recession)
6.7 Secondary Angle-Closure Glaucoma
7.2 Embryology, Anatomy, and Development
7.3 Congenital Abnormalities
7.4 Inflammation and Ischemia
7.4.1.2 Inflammation Secondary to Vascular Disease and Ischemia
7.4.1.3 Inflammation Secondary to Demyelinating and/or Autoimmune Disease
7.7.2 Optic Nerve Gliomas
7.7.3 Optic Nerve Meningiomas
8: Pathology of the Vitreous
8.4 Congenital Abnormalities
8.4.1 Persistent Hyperplastic Primary Vitreous
8.4.2 Congenital Vitreous Cyst
8.4.3 Congenital Vitreous Liquefaction
8.5.1 Infectious Endophthalmitis
8.5.1.1 Purulent Endophthalmitis
8.5.1.2 Viral Endophthalmitis
8.5.1.3 Low-Virulence Bacterial Endophthalmitis
8.5.3 Vitreous Inflammation from Parasites
8.5.3.1 Toxocara Endophthalmitis
8.5.6 Vitreous Ophthalmomyiasis
8.6 Autoimmune Vitritis and Spillover
8.6.1 Phacoantigenic Uveitis
8.8 Membranous Proliferations
8.8.1 Epiretinal Membranes
8.8.2 Proliferative Vitreoretinopathy
8.8.3 Extraretinal Neovascular Membranes
8.8.4 Retinopathy of Prematurity
8.8.5 Familial Exudative Vitreoretinopathy
8.10.2 Miscellaneous Vitreoretinal Degenerations
8.10.5 Synchysis Scintillans
8.11.1 Primary Intraocular Lymphoma
8.11.2 Vitreous Metastases
8.11.3 Primary Ocular Malignancies with Vitreous Shedding
9.2.1 Anatomy of the Retina
9.2.1.2 The Retinal Pigment Epithelium (RPE)
9.4 Retinal Inflammatory Conditions
9.6 Retinal Degenerations
9.6.1 Age-Related Macular Degeneration
9.6.4 Retinal Holes, Tears, Schisis, and Related Degenerations
9.7 Retinal Vascular Disease
9.7.1 Diabetic Retinopathy
9.7.2 Retinal Vascular Occlusions
9.7.3 Hypertensive Retinal Disease
9.7.4 Radiation Retinopathy
9.7.6 Collagen Vascular Diseases
9.7.7 Retinopathy of Prematurity
9.8 Neoplasms of the Retina
9.8.1 Astrocytoma and Astrocytic (Glial) Hamartoma
9.8.1.1 Genetics of Syndromes Associated with Retinal Astrocytomas
9.9 Retinal Vascular Tumors
9.9.1 Retinal Hemangioblastomas (Hemangiomas)
9.9.2 Retinal Cavernous Hemangioma
9.9.3 Retinal Arteriovenous (AV) Communication (Retinal Racemose Hemangioma)
9.9.4 Vasoproliferative Tumor of the Retina
9.9.5 Retinoblastoma and Retinoma/Retinocytoma
9.9.6 Retinoma (Retinocytoma)
9.9.7 Tumors of the Retinal Pigment Epithelium
9.9.7.1 Simple Hamartoma of the Retinal Pigment Epithelium
9.9.7.2 Combined Hamartoma of the Retina and Retinal Pigment Epithelium
9.9.7.3 Congenital Hypertrophy of the Retinal Pigment Epithelium (CHRPE)
9.9.7.4 Reactive Hyperplasia of the Retinal Pigment Epithelium
9.9.10 Metastatic Tumors to the Retina
9.9.9 Adenocarcinoma of the Retinal Pigment Epithelium
10.2 Embryology, Anatomy and Development
10.3 Congenital Abnormalities
10.6 Degenerative Conditions of the Uvea
10.7.3 Bilateral Diffuse Uveal Proliferation (BDUMP)
10.7.5 Choroidal Haemangioma
10.7.10 Uveal Extranodal Marginal Zone B-Cell Lymphoma
10.7.11 Secondary Uveal Lymphoma
10.7.13 Metastatic Uveal Tumours
10.7.14 Other Intraocular Tumours
11.3 Congenital and Developmental Abnormalities
11.3.5 Phakomatous Choristoma
11.3.6 Xeroderma Pigmentosum
11.4.1 Atrophy and Solar Elastosis
11.5.5 Pyogenic Granuloma
11.5.6.1 Molluscum Contagiosum
11.5.6.2 Verruca Vulgaris
11.5.6.3 Herpesvirus Infection
11.5.7 Bacterial Infections
11.5.10 Parasitic Infections
11.5.8 Fungal and Parasitic Infections
11.5.11 Filarial Infections
11.6 Eyelid Lesions with Association to Systemic Diseases
11.6.5 Juvenile Xanthogranuloma
11.6.6 Necrobiotic Xanthogranuloma
11.6.7 Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)
11.7.1 Epidermal Inclusion Cyst
11.7.5 Seborrheic Keratosis
11.7.6 Inverted Follicular Keratosis
11.8 Premalignant Conditions
11.8.2 Bowen Disease (Squamous Cell Carcinoma In Situ)
11.9.1 Epithelial Neoplasms
11.9.1.1 Squamous Papilloma
11.9.1.2 Basal Cell Carcinoma and Variants
11.9.1.3 Nevoid Basal Cell Carcinoma Syndrome
11.9.1.4 Squamous Cell Carcinoma and Variants
11.9.2 Melanocytic Lesions and Neoplasms
11.9.2.4 Junctional Nevus
11.9.2.6 Dermal Nevus (Intradermal Nevus)
11.9.2.10 Ocular Melanocytosis
11.9.2.11 Dysplastic Nevus
11.9.2.12 Melanoma In Situ
11.9.2.13 Lentigo Maligna
11.9.2.14 Invasive Malignant Melanoma
11.9.3 Sebaceous Lesions and Neoplasms
11.9.3.1 Sebaceous Hyperplasia
11.9.3.2 Sebaceous Adenoma
11.9.3.3 Sebaceous Carcinoma
11.9.4 Adnexal Tumors with Sweat Gland Differentiation
11.9.4.2 Pleomorphic Adenoma
11.9.4.3 Low-Grade Mucinous Carcinoma
11.9.5 Adnexal Tumors with Follicular Differentiation
11.9.5.1 Trichoepithelioma
11.9.5.3 Trichofolliculoma
11.9.6 Merkel Cell Carcinoma
11.9.7 Mesenchymal (Soft Tissue) Neoplasms
11.9.7.1 Fibrous and Fibrohistiocytic Neoplasms
11.9.7.3 Vascular Neoplasms
11.10 Hematopoietic/Lymphoid Neoplasms
11.10.1 Lymphoid Hyperplasia
11.10.2 Primary Cutaneous Lymphomas
11.10.3 Mycosis Fungoides
11.10.4 Primary Cutaneous Anaplastic Large Cell Lymphoma
11.10.5 Cutaneous Follicular Lymphoma
11.10.6 Cutaneous Diffuse Large B-Cell Lymphoma
11.11 Eyelid Involvement by Other Hematopoietic Disorders
12: The Orbit, Including the Lacrimal Gland and Lacrimal Drainage System
12.1.1 Classification and Frequency
12.2 Embryology, Anatomy, and Development
12.2.2 Anatomy and Histology
12.3 Congenital Abnormalities
12.3.1.1 Epithelial Cystic Choristoma
12.3.1.3 Ectopic Lacrimal Gland (Cyst)
12.3.1.4 Osseous Choristoma
12.3.1.5 Ectopic Lymph Node
12.3.1.6 Ectopic Pituitary Adenoma
12.3.4 Congenital Malformations
12.3.4.1 Microphthalmos with Cyst
12.3.4.2 Meningoencephalocele and Ectopic Brain Tissue
12.4 Inflammatory Diseases
12.4.2 Reactive Inflammation
12.4.2.1 Cholesterol Granuloma
12.4.2.2 Paraffinoma/Sclerosing Lipogranuloma
12.4.3 Immune Reactive Diseases
12.4.3.1 Thyroid Eye Disease
12.4.3.2 Inflammatory Pseudotumors
Idiopathic Orbital Inflammatory (IOI) Disease
IG4-Related Orbital Disease (IGRD)
Xanthogranulomatous Inflammatory Diseases
Adult-Onset Asthma with Periocular Xanthogranuloma (AAPOX)
Adult-Onset Xanthogranuloma (AOX)
Erdheim–Chester Disease (ECD)
Necrobiotic Xanthogranuloma
Juvenile Orbital Xanthogranuloma (JOX)
Wegener’s Granulomatosis (WG)
12.6.1 Fat/Lacrimal Gland Prolapse
12.7.1 Fibroblastic/Myofibroblastic/Fibrohistiocytic Tumors
12.7.1.1 Nodular Fasciitis
12.7.1.2 Myofibroma/Myofibromatosis
12.7.1.3 Juvenile Hyaline Fibromatosis
12.7.1.4 Infantile Hemangiopericytoma
12.7.1.5 Inflammatory Myofibroblastic Tumor (IMT)
12.7.1.6 Solitary Fibrous Tumor/Hemangiopericytoma/Giant Cell Angiofibroma
12.7.1.7 Fibrous Histiocytoma
12.7.1.8 Myxoma and Angiomyxoma
12.7.1.9 Myxofibrosarcoma
12.7.1.10 Infantile Fibrosarcoma (IF)
12.7.3.1 Smooth Muscle Tumors
12.7.4 Rhabdomyomatous Tumors
12.7.4.2 Rhabdomyosarcoma (RMS)
12.7.5 Pericytic (Perivascular) Tumors
12.7.6.1 Juvenile Hemangioma
12.7.6.2 Cavernous Hemangioma
12.7.6.3 Arteriovenous Hemangioma/Malformation (AVH/ AVM)
12.7.6.5 Epithelioid Hemangioma (Angiolymphoid Hyperplasia with Eosinophilia)
12.7.6.6 Angiosarcoma/Epithelioid Hemangioendothelioma
12.7.7.2 Yolk Sac Tumor/Endodermal Sinus Tumor
12.7.7.3 Primary Germinoma of the Orbit
12.7.8.3 Granular Cell Tumor
12.7.8.4 Malignant Peripheral Nerve Sheath Tumor (MPNST)
12.7.8.5 Small Blue Round Cell Tumors (SBRCT)
Neuroblastoma/Ganglioneuroma
Rhabdomyosarcoma (See Sect. 12.7.4.2)
12.7.8.6 Ewing Sarcoma/Primary Peripheral Primitive Neuroectodermal Tumor (ES/pPNET)
Small Cell Lymphoma (See Also Lymphomas, Sect. 12.7.10.2)
Wilms Tumor (Nephroblastoma)
12.7.8.8 Optic Nerve Glioma
12.7.9 Chondro-osseous Tumors
12.7.9.2 Giant Cell Tumor of Bone
12.7.9.3 Fibrous Dysplasia
12.7.9.4 Osteofibrous Dysplasia
12.7.9.10 Extraskeletal Mesenchymal Chondrosarcoma
12.7.9.9 Extraskeletal Osteosarcoma
12.7.10.1 Meningioma WHO Grade I (e.g., Meningothelial, Fibrous, Psammomatous)
12.7.10.2 Atypical Meningioma WHO Grade II (Includes Chordoid and Clear Cell Meningioma)
12.7.10.3 Anaplastic (Malignant) Meningioma, WHO Grade III (and Includes Rhabdoid Papillary Meningioma)
12.7.11.1 Lymphoid Hyperplasia
12.7.11.3 Multiple Myeloma/Waldenstrưm Macroglobulinemia
12.7.11.4 Granulocytic Sarcoma
12.7.12 Dendritic Cell Disorders
12.7.12.1 Langerhans Cell Histiocytosis (LCH)
12.7.12.2 Erdheim–Chester Disease (See Also Sect. 12.4.3.2.3)
12.7.12.3 Rosai–Dorfman Disease
12.7.12.4 Castleman Disease
12.7.13 Tumors of Uncertain Differentiation
12.7.13.1 Primary Synovial Sarcoma
12.7.13.2 Extrarenal Rhabdoid Tumor
12.7.14 Miscellaneous Tumors
12.7.14.1 Orbital Melanoma
12.7.14.2 Melanotic Neuroectodermal Tumor of Infancy (MNTI)
12.7.14.3 Primary Orbital Carcinoid and Paraganglioma
12.7.14.4 Alveolar Soft Part Sarcoma
12.7.15 Secondary Orbital tumors
12.7.15.1 Direct Extension
12.8.1 General Description
12.8.1.1 Classification and Frequency
12.8.2 Embryology, Anatomy, and Development
12.8.2.2 Anatomy and Histology
12.10.1.1 Dacryoadenitis Secondary to Sjưgren Syndrome (SS)
12.10.2 IgG4-Related Chronic Sclerosing Dacryoadenitis
12.10.3 Dacryoadenitis Secondary to Thyroid Disease
12.10.4 Dacryoadenitis Secondary to Connective Tissue Diseases
12.9 Congenital Abnormalities
12.13.1 Benign Mesenchymal Tumors
12.13.1.1 Granular Cell Tumor
12.13.1.3 Solitary Fibrous Tumor
12.13.2 Benign Epithelial Lacrimal Gland Tumors
12.13.2.1 Pleomorphic Adenoma (PA)
12.13.2.2 Warthin’s Tumor (WT)
12.13.2.3 Basal Cell Adenoma (BCA)
12.13.2.5 Myoepithelioma (MYO)
12.13.3 Malignant Epithelial Neoplasms of the Lacrimal Gland
12.13.3.1 Adenoid Cystic Carcinoma (ACC)
12.13.3.2 Carcinoma Ex Pleomorphic Adenoma (Ca ex PA) and Pleomorphic Carcinoma (PC)
12.13.3.3 Primary Adenocarcinoma, NOS (ADC)
12.13.3.4 Mucoepidermoid Carcinoma (MEC)
12.13.3.5 Ductal Adenocarcinoma (DA)
12.13.3.6 Squamous Cell Carcinoma
12.13.3.7 Sebaceous Carcinoma (SC)
12.13.3.8 Lymphoepithelial Carcinoma (LEC)
12.13.3.9 Polymorphous Low- Grade Adenocarcinoma (PLGA)
12.13.3.10 Epithelial–Myoepithelial Carcinoma (EMC)
12.13.3.11 Cystadenocarcinoma (CAC)
12.13.3.12 Acinic Cell Carcinoma (AC)
12.13.3.13 Basal Cell Adenocarcinoma (BCCA)
12.13.3.14 Mucinous Adenocarcinoma (MA)
12.13.3.15 Oncocytic Adenocarcinoma (OCA)
12.13.3.16 Large Cell Carcinoma (LCCA)
12.13.3.17 Merkel Cell Carcinoma (MCC)
12.13.3.18 Other Unusual Malignant Lacrimal Gland Neoplasms
12.14 Lacrimal Drainage System
12.14.2 Embryology, Anatomy, and Development
12.14.2.2 Anatomy and Histology
12.15 Congenital Abnormalities
12.15.1 Congenital Nasolacrimal Duct Obstruction
12.15.2 Congenital Lacrimal Fistula
12.19 Neoplasms of the Lacrimal Drainage System
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