About the Book
This manual is a complete guide to the diagnosis and management of ocular disorders and diseases. Divided into 35 sections, further divided by sub-specialty, the book presents more than 1000 common and uncommon conditions. Each disorder is systematically arranged in a consistent format, beginning with a brief description, followed by clinical observation, laboratory diagnosis, and treatment. Drug dosage and administration are discussed in depth and each disorder concludes with a bibliography for further reading. Disorders are listed alphabetically for quick reference, and can be searched either by anatomical region or by classification of the disorder. Written by internationally recognised Frederick Hampton Roy, and co-author, Renee Tindall, both from Hampton Roy Eyecare, Arkansas; this comprehensive book includes numerous clinical photographs, diagrams and tables, to enhance learning. Key points Guide to diagnosis and management of more than 1000 ocular diseases and disorders Disorders listed alphabetically and searchable anatomically or by classification Bibliography included for each disorder for further reading Authored by internationally recognised US-based specialists
Table of Contents:
1. Infectious Diseases 1 • Acinetobacter (Mima Polymorpha; Acinetobacter Iwoffi) 1 • Acquired Immunodeficiency Syndrome (AIDS; Acquired Cellular Immunodeficiency; Acquired Immunodeficiency) 1 • Acute Hemorrhagic Conjunctivitis (AHC; Epidemic Hemorrhagic Keratoconjunctivitis, Apollo 11 Disease) 2 • Aspergillosis 3 • Bacillus Species Infections 3 • Blastomycosis 4 • Brucellosis (Bang Disease; Malta Fever; Mediterranean Fever; Pig Breeder Disease; Gibraltar Fever; Undulant Fever) 4 • Candidiasis 5 • Catscratch Oculoglandular Syndrome (Parinaud Conjunctiva-Adenitis Syndrome; Parinaud Oculoglandular Syndrome; Catscratch Disease; Bartonella Henselae) 5 • Coccidioidomycosis (Valley Fever, San Joaquin Fever) 6 • Deerfly Fever (Francis Disease; Rabbit Fever; Tularemia; Deerfly Tularemia) 6 • Dengue Fever 6 • Dermatophytosis (Epidermophytosis; Epidermomycosis; Rubrophytia; Tinea; Trichophytosis) 7 • Diphtheria 7 • Epidemic Keratoconjunctivitis 8 • Erysipelas (Saint Anthony Fire) 8 • Escherichia Coli 9 • Gonorrhea 9 • Haemophilus Aegyptius (Koch-Weeks Bacillus) 10 • Haemophilus Influenzae 10 • Hansen Disease (Leprosy) 10 • Herpes Simplex 11 • Herpes Simplex Masquerade Syndrome 11 • Inclusion Conjunctivitis (Chlamydia; Paratrachoma) 12 Contents • Infectious Mononucleosis (Mononucleosis; Epstein-Barr Virus, Acute; Acute EpsteinBarr Virus, Glandular Fever) 12 • Influenza 13 • Leptospirosis (Weil Disease) 13 • Lyme Disease 14 • Marseilles Fever (Boutonneuse Fever) 14 • Microsporidial Infection 14 • Molluscum Contagiosum 15 • Moraxella Lacunata 15 • Mucormycosis (Phycomycosis) 16 • Mumps 16 • Newcastle Disease (Fowlpox) 16 • Nocardiosis 17 • Pappataci Fever (Phlebotomus Fever; Sandfly Fever) 17 • Pharyngoconjunctival Fever (Acute Follicular Conjunctivitis; Adenoviral Conjunctivitis; Syndrome of Beal) 18 • Pneumococcal Infections (Streptococcus Pneumoniae Infections) 18 • Presumed Ocular Histoplasmosis (Histoplasmosis Choroiditis; Histoplasmosis Maculopathy; Histoplasmosis Syndrome) 19 • Propionibacterium Acnes 19 • Proteus Infections 19 • Proteus Syndrome 20 • Pseudomonas Aeruginosa Infections 20 • Q Fever (Coxiella Burnetii) 21 • Rabies (Hydrophobia; Lyssa) 21 • Rhinosporidiosis 22 • Rocky Mountain Spotted Fever 22 • Rubella Syndrome (Congenital Rubella Syndrome; German Measles; Gregg Syndrome) 22 • Rubeola (Measles; Morbilli) 23 • Sporotrichosis 23 • Staphylococcus 24 • Streptococcus (Scarlet Fever) 24 • Syphilis (Acquired Lues; Acquired Syphilis; Lues Venerea; Malum Venereum) 25 x Ocular Treatment: Evidence Based • Syphilis, Congenital (Congenital Lues) 25 • Tetanus (Lockjaw) 26 • Trachoma 26 • Tuberculosis 27 • Typhoid Fever (Abdominal Typhus; Enteric Fever) 27 • Varicella (Chickenpox) 28 • Varicella Syndrome, Congenital 28 • Yersiniosis 28 2. Parasitic Diseases 29 • Acanthamoeba 29 • Baylisascaris (Unilateral Subacute Neuroretinitis; Ocular Larva Migrans) 29 • Coenurosis (Tapeworm) 30 • Demodicosis 30 • Hydatid Cyst (Echinococcosis) 31 • Nematode Ophthalmia Syndrome (Visceral Larva Migrans Syndrome; Toxocariasis) 31 • Pediculosis and Phthiriasis 32 • Toxoplasmosis (Toxoplasmic Retinochoroiditis; Ocular Toxoplasmosis) 32 • Trichinellosis (Trichinosis) 33 3. Nutritional Disorders 34 • Crohn Disease (Granulomatous Ileocolitis) 34 • Hypovitaminosis A (Xerophthalmia) 35 • Inflammatory Bowel Disease (Ulcerative Colitis; Regional Enteritis) 35 4. Disorders of Protein Metabolism 36 • Lowe Syndrome (Oculo-Cerebro-Renal Syndrome) 36 • ML I (Mucolipidosis I; Lipomucopolysaccharidosis; Dysmorphic Sialidosis; Spranger Syndrome) 36 • Tyrosinosis (Hanhart Syndrome; Richner Syndrome; Recessive Keratosis Palmoplantaris; Pseudoherpetic Keratitis; Richner-Hanhart Syndrome; Tyrosinemia II; Pseudodendritic Keratitis) 37 5. Disorders of Carbohydrate Metabolism 38 • Diabetes Mellitus 38 • Fanconi Syndrome (Toni-Fanconi Syndrome; Amino Diabetes; Hypochloremic-Glycosuric Osteonephropathy Syndrome; De Toni-Fanconi Syndrome) 39 • Hunter Syndrome (MPS II Syndrome; Mucopolysaccharidosis II; Systemic Mucopolysaccharidosis Type II) 39 • Hurler Syndrome (Pfaundler-Hurler Syndrome; Gargoylism; Dysostosis Multiplex; MPS IH Syndrome; Systemic Mucopolysaccharidosis Type IH; Mucopolysaccharidosis IH) 40 • Juvenile Diabetes-Dwarfism-Obesity Syndrome (Mauriac Syndrome; DwarfismHepatomegaly-Obesity-Juvenile Diabetes Syndrome) 40 • Kimmelstiel-Wilson Syndrome (Diabetes Mellitus-Hypertension-Nephrosis Syndrome; Diabetes-Nephrosis Syndrome; Diabetic Glomerulosclerosis; Intercapillary Glomerulosclerosis;Renal Glomerulohyalinosis-Diabetic Syndrome) 41 • Marquardt-Loriaux Syndrome (Wolfram Syndrome; Diabetes InsipidusDiabetes Mellitus-OpticAtrophy-Deafness Syndrome; DIDMOAD Syndrome) 41 • Mucopolysaccharidosis VII (Beta-Glucuronidase Deficiency; MPS VII) 42 • Sanfilippo-Good Syndrome (Heparitinuria; Mucopolysaccharidosis III; MPS III) 42 • Scheie Syndrome (Mucopolysaccharidosis IS; MPS IS; MPS V; Mucopolysaccharidosis V) 43 • Spondyloepiphyseal Dysplasia (Morquio Syndrome; Morquio-Brailsford Syndrome; Brailsford-Morquio Dystrophy; Familial Osseous Dystrophy; Keratosulfaturia; MPS IV; Mucopolysaccharidosis IV; Osteochondrodystrophia Deformans; Infantile Hereditary Chondrodysplasia; Hereditary Polytopic Enchondral Dysostosis; Hereditary Osteochondrodystrophy; EccentroOsteochondrodysplasia; Dysostosis Enchondralis Meta-Epiphysaria; Morquio-Ullrich Syndrome; Atypical Chondrodystrophy; Chondrodystrophia Tarda; Chondro-Osteodystrophy) 43 • Systemic Mucopolysaccharidosis Type VI (Mucopolysaccharidosis VI; MaroteauxLamy Syndrome; MPS VI Syndrome) 44 • Tropical Pancreatic Diabetes (TPD) (Background Retinopathy) 45 • Von Reuss Syndrome (Galactosemic Syndrome; Galactokinase Deficiency; Galactosemia), Nystagmus 45 Contents xi 6. Disorders of Lipid Metabolism 46 • Fabry Disease (Angiokeratoma Corporis Diffusum Syndrome; Diffuse Angiokeratosis; Fabry-Anderson Syndrome; Glycosphingolipid Lipidosis; Glycosphingolipidosis) 46 • Hyperlipoproteinemia 47 7. Hematologic and Cardiovascular Disorders 48 • Carotid Artery Syndrome (Cavernous Sinus Fistula Syndrome; Red-Eyed Shunt Syndrome) 48 • Carotid Artery Syndrome (Carotid Vascular Insufficiency Syndrome; Ocular Ischemic Syndrome) 49 • Foramen Lacerum Syndrome (Aneurysm of Internal Carotid Artery Syndrome) 49 • Herrick Syndrome (Dresbach Syndrome; Sickle Cell Disease; Drepanocytic Anemia) 50 • Scheie Syndrome (Mucopolysaccharidosis IS; MPS IS; MPS V; Mucopolysaccharidosis V) 50 • Thalassemia (Cooley Anemia; Thalassemia Major; Thalassemia Minor) 51 • Vaquez Disease (Polycythemia Vera; Erythema; Erythrocytosis Megalosplenica; Myelopathic Polycythemia; Vaquez-Osler Syndrome; Cryptogenic Polycythemia; Polycythemia Rubra; Splenomegalic Polycythemia) 51 8. Dermatologic Disorders 52 • Atopic Dermatitis (Atopic Eczema; Besnier Prurigo) 52 • Bullous Ichthyosiform Erythroderma (Collodion Baby; Congenital Ichthyosis; Epidermolytic Hyperkeratosis; Ichthyosis; Ichthyosis Vulgaris; Lamellar Ichthyosis; Nonbullous Ichthyosiform Erythroderma; Xeroderma; X-Linked Ichthyosis) 52 • Contact Dermatitis (Dermatitis Venenata) 53 • Dermatostomatitis [Stevens-Johnson Syndrome; Erythema Multiforme Exudativum; Syndroma Mucocutaneo-Oculare; Baader Dermatostomatitis Syndrome; MucosalRespiratory Syndrome; Fuchs (2) Syndrome; Mucocutaneous Ocular Syndrome] 53 • Disseminated Lupus Erythematosus (Systemic Lupus Erythematosus; Lupus Erythematosus; Kaposi-Libman-Sack Syndrome, SLE) 54 • Erythema Nodosum (Dermatitis Contusiformis) 54 • Mastocytosis (Urticaria; Mast Cell Leukemia) 55 • Acne Rosacea (Acne Erythematosa; Ocular Rosacea) 55 • Psoriasis (Psoriasis Vulgaris) 56 • Quincke Disease (Giant Edema; Giant Urticaria; Hives; Nettle Rash; Angioneurotic Edema) 56 9. Connective Tissue Disorders 57 • Amyloidosis (Primary Amyloidosis; LubarschPick Syndrome; Idiopathic Amyloidosis) 57 • Amyloidosis of Gingiva and Conjunctiva, with Mental Retardation (Primary Systemic Amyloidosis) 58 • Behçet Syndrome (Dermato-StomatoOphthalmic Syndrome; Oculobuccogenital Syndrome; Gilbert Syndrome) 58 • Cogan (2) Syndrome (Oculomotor Apraxia Syndrome; Wieacker Syndrome) 59 • Felty Syndrome (Chauffard-Still Syndrome; Primary Splenic Neutropenia with Arthritis; Rheumatoid Arthritis with Hypersplenism; Still-Chauffard Syndrome; Uveitis-Rheumatoid Arthritis Syndrome) 59 • Grönblad-Strandberg Syndrome (Systemic Elastodystrophy; Pseudoxanthoma Elasticum; Elastorrhexis; Darier-GrönbladStrandberg Syndrome) 60 • Hamman-Rich Syndrome (Alveolar Capillary Block Syndrome; Diffuse Pulmonary Fibrosis Syndrome; Rheumatoid Lung Syndrome) 60 • Juvenile Rheumatoid Arthritis (JRA; Still Disease) 61 • Marfan Syndrome (Dolichostenomelia; Arachnodactyly; Hyperchondroplasia; Dystrophia; Mesodermalis Congenita) 61 • Morphea (Localized Scleroderma; Circumscribed Scleroderma) 62 • Relapsing Polychondritis (Jaksch-Wartenhost Syndrome; Meyenburg-Altherz-Vehlinger Syndrome; von Meyenberg II Syndrome) 62 • Rheumatoid Arthritis (Adult) 63 • Schaumann Syndrome (Besnier-Boeck-Schaumann Syndrome; Boeck Sarcoid; Sarcoidosis) 63 • Scleroderma (Progressive Systemic Sclerosis; Systemic Scleroderma) 64 • Systemic Lupus Erythematosus (Disseminated Lupus Erythematosus; Lupus Erythematosus; Kaposi-Libman-Sack Syndrome, SLE) 64 xii Ocular Treatment: Evidence Based • Temporal Arteritis Syndrome (Cranial Arteritis Syndrome; Giant Cell Arteritis; Hutchinson-HortonMagath-Brown Syndrome) 65 • Uveitis, Juvenile Idiopathic Arthritis 65 • von Bekhterev-Strümpell Syndrome (Marie-Strümpell Spondylitis; Ankylosing Spondylitis; Pierre-Marie Syndrome; Bekhterev Disease; Rheumatoid Spondylitis), Optic Atrophy 66 • Wegener Syndrome (Wegener Granulomatosis) 66 • Weill-Marchesani Syndrome (Marchesani Syndrome; Inverted Marfan Syndrome; Brachymorphy with Spherophakia; Dystrophia Mesodermalis Congenita Hyperplastica) 67 10. Skeletal Disorders 68 • Down Syndrome (Mongolism; Trisomy G; Trisomy 21 Syndrome; Mongoloid Idiocy) 68 11. Phakomatoses 69 • Angiomatosis Retinae (von Hippel-Lindau Syndrome; Retinocerebral Angiomatosis; Cerebelloretinal Hemangioblastomatosis; Lindau Syndrome; Retinal Capillary Hamartoma) 69 • Neurofibromatosis Type I (von Recklinghausen Syndrome; Neurinomatosis) 69 • Sturge-Weber Syndrome (Meningocutaneous Syndrome; Vascular Encephalotrigeminal Syndrome; Neuro-Oculocutaneous Angiomatosis; Encephalofacial Angiomatosis; Encephalotrigeminal Syndrome) 70 12. Neurologic Disorders 71 • Amblyopia (Functional Amblyopia, Lazy Eye) 71 • Aniridia, Cerebellar Ataxia, and Mental Deficiency (Gillespie Syndrome) 71 • Arnold-Chiari Syndrome (Platybasia Syndrome; Cerebellomedullary Malformation Syndrome; Basilar Impressions) 72 • Andersen-Warburg Syndrome (WhitnallNorman Syndrome; Oligophrenia Microphthalmos Syndrome; Norrie Disease; Atrophia Oculi Congenital Fetal Iritis Syndrome; Congenital Progressive Oculo-Acoustico-Cerebral Dysplasia) 72 • Batten-Mayou Syndrome (SpielmeyerVogt Syndrome; Mayou-Batten Disease; Stock-Spielmeyer-Vogt Syndrome; Cerebroretinal Degeneration; Pigmentary Retinal Lipoid Neuronal Heredodegeneration; Vogt-Spielmeyer Syndrome; Juvenile Ganglioside Lipidosis; Neuronal Ceroid Lipofuscinosis; Myoclonic Variant of Cerebral Lipidosis; Batten Disease; Cerebromacular Dystrophy; Juvenile Amaurotic Family Idiocy; Spielmeyer-Sjögren Syndrome) 73 • Bell’s Palsy (Idiopathic Facial Paralysis) 74 • Bielschowsky-Lutz-Cogan Syndrome (Internuclear Ophthalmoplegia) 74 • Bonnet-Dechaume-Blanc Syndrome (Cerebroretinal Arteriovenous Aneurysm Syndrome; Neuroretinoangiomatosis Syndrome; Wyburn-Mason Syndrome) 75 • Cerebellar Ataxia, Infantile, with Progressive External Ophthalmoplegia 75 • Cerebellar Ataxia, Cataract, Deafness and Dementia or Psychosis (Heredopathia Ophthalmo-Oto-Encephalica) 76 • Cerebellar Degeneration with Slow Movements 76 • Cerebral Autosomal Dominant Arteriopathy 76 • Cerebral Cholesterolosis (Cerebrotendinous Xanthomatosis; CTX) 77 • Cerebral Palsy 77 • Chronic Progressive External Ophthalmoplegia (CPEO; Ophthalmoplegia Plus) 78 • Claude Syndrome (Inferior Nucleus Ruber Syndrome; Rubrospinal-CerebellarPeduncle Syndrome) 78 • Congenital Dyslexia Syndrome (Developmental Dyslexia of Critchley; Congenital Word Blindness of Hermann; Primary Dyslexia; Dyslexia Syndrome; Minimal Brain Dysfunction Syndrome; Attention Deficit Disorder; Congenital Word Blindness) 79 • Creutzfeldt-Jakob Syndrome (Spastic Pseudosclerosis; Corticostriatospinal Degeneration; Disseminated Encephalopathy; Heidenhain Syndrome; Presenile Dementia with Spastic Paralysis; Presenile Dementia-Cortical Degeneration Syndrome) 79 Contents xiii • Cushing (2) Syndrome (Angle Tumor Syndrome; Cerebellopontine Angle Syndrome; Pontocerebellar Angle Tumor Syndrome; Acoustic Neuroma Syndrome) 80 • De Lange Syndrome (I) (Congenital Muscular Hypertrophy Cerebral Syndrome; Brachmann-De Lange Syndrome) 80 • Disseminated Sclerosis (Multiple Sclerosis) 81 • Friedreich Ataxia (Spinocerebellar Ataxia) 81 • Hartnup Syndrome (Pellagra-Cerebellar Ataxia-Renal Aminoaciduria Syndrome; H Disease; Niacin Deficiency) 82 • Hysteria (Malingering; Ophthalmic Flake Syndrome) 82 • Joubert Syndrome (Familial Cerebellar Vermis Agenesis) 83 • Marinesco-Sjögren Syndrome (Congenital Spinocerebellar Ataxia-Congenital Cataract-Oligophrenia Syndrome) 83 • Neuroparalytic Keratitis (Neurotropic Keratitis, Trigeminal Neuropathic Keratopathy) 84 • Nothnagel Syndrome (OphthalmoplegiaCerebellar Ataxia Syndrome) 84 • Obesity-Cerebral-Ocular-Skeletal Anomalies Syndrome 84 • Oculocerebellar Tegmental Syndrome 85 • Oculocerebral Syndrome with Hypopigmentation (Amish Oculocerebral Syndrome; Cross Syndrome) 85 • Oculopalatocerebral Dwarfism (OPC Dwarfism) 86 • Oculorenocerebellar Syndrome (ORC Syndrome) 86 • Olivopontocerebellar Atrophy III (OPCA III; OPCA with Retinal Degeneration) 87 • Optic Atrophy with Demyelinating Disease of CNS 87 • Parkinson Syndrome (Paralysis Agitans; Shaking Palsy) 87 • Raeder Syndrome (Paratrigeminal Paralysis; Horton Headache; Histamine Cephalalgia; Ciliary Neuralgia; Cluster Headache; Periodic Migrainous Neuralgia) 88 • Sotos Syndrome (Cerebral Gigantism) 88 • Spinocerebellar Atrophy with Pupillary Paralysis 89 • Stannus Cerebellar Syndrome 89 • Symonds Syndrome (Otitic Hydrocephalus Syndrome; Serous Meningitis Syndrome; Benign Intracranial Hypertension; Pseudotumor Cerebri) 90 • Thyrocerebroretinal Syndrome (Familial Thyrocerebral-Retinal Syndrome) 90 • Tolosa-Hunt Syndrome (Painful Ophthalmoplegia) 91 • Traumatic Encephalopathy Syndrome (Posttraumatic General Cerebral Syndrome; Postconcussion Syndrome; Punch-Drunk Syndrome), Nystagmus 91 • Weber Syndrome (Weber-Dubler Syndrome; Cerebellar Peduncle Syndrome; Alternating Oculomotor Paralysis; Ventral Medial Midbrain Syndrome), Ptosis 92 13. Neoplasms 93 • Actinic and Seborrheic Keratosis 93 • Basal Cell Carcinoma 93 • Basal Cell Nevus Syndrome (Nevoid Basal Cell Carcinoma Syndrome; Nevoid Basalioma Syndrome; Gorlin Syndrome; Gorlin-Goltz Syndrome; Multiple Basal Cell Nevi Syndrome) 94 • Squamous Cell Carcinoma, Conjunctival 94 • Squamous Cell Carcinoma of Eyelid 95 • Corneal Dermoids 95 – Bloch-Sulzberger Syndrome (Incontinentia Pigmenti; Siemens-Bloch-Sulzberger Syndrome) 95 – Cri-du-Chat Syndrome [Cat-Cry (5p-) Syndrome; Crying Cat Syndrome; BI Deletion Syndrome; Lejeune Syndrome] 96 – Duane Syndrome 96 – Coats Disease (Leber Miliary Aneurysm; Retinal Telangiectasia) 97 – Neurocutaneous Syndrome 97 – Linear Nevus Sebaceus of Jadassohn (Nevus Sebaceus of Jadassohn; Jadassohn-Type Anetoderma; Organoid Nevus Syndrome; Sebaceus Nevus Syndrome) 98 – Goldenhar Syndrome (Oculo-AuriculoVertebral Dysplasia; Goldenhar-Gorlin Syndrome) 98 – Ring Dermoid Syndrome (Amblyopia) 99 xiv Ocular Treatment: Evidence Based • Craniopharyngioma 99 • Dermoid (Dermoid Choristoma; Dermoid Cyst; Dermolipoma; Lipodermoid) 99 • Dermoid, Orbital 100 • Ring Dermoid Syndrome (Amblyopia) 100 • Ewing Sarcoma (Ewing Syndrome) 101 • Fibrosarcoma 101 • Hemangioma, Capillary 101 • Hodgkin Disease 102 • Hutchinson Syndrome (Adrenal Cortex Neuroblastoma with Orbital Metastasis; Pepper Syndrome) 102 • Juvenile Xanthogranuloma (JXG; Nevoxanthoendothelioma) 103 • Kaposi Disease (Kaposi Sarcoma; Kaposi Hemorrhagic Sarcoma; Multiple Idiopathic Hemorrhagic Sarcoma; Kaposi Varicelliform Eruption) 103 • Kasabach-Merritt Syndrome (CapillaryAngioma-Thrombocytopenia; Hemangioma-Thrombocytopenia; Thrombocytopenia Purpura-Hemangioma) 104 • Keratoacanthoma 104 • Leiomyoma 105 • Liposarcoma 105 • Lymphangioma 105 • Lymphoid Hyperplasia (Reactive Lymphoid Hyperplasia; Lymphoid Tumors; Malignant Lymphoma; Pseudolymphoma; Pseudotumor; Burkitt Lymphoma; Neoplastic Angioendotheliomatosis) 106 • Medulloepithelioma (Diktyoma) 106 • Meningioma 107 • Meningioma, Optic Nerve Sheath 107 • Meningioma, Sphenoid Wing 108 • Mucocele (Pyocele) 108 • Neurilemmoma (Schwannoma; Neurinoma, PTEN Hamartoma Tumor Syndrome) 108 • Neuroblastoma 109 • Optic Gliomas 109 • Orbital Lymphoma 110 • Orbital Rhabdomyosarcoma 110 • Papilloma (Wart; Verruca) 110 • Periocular Merkel Cell Carcinoma 111 • Periocular Metastatic Tumors (Ocular Metastatic Tumors) 111 • Reticulum Cell Sarcoma (Non-Hodgkin Lymphoma) 112 • Retinoblastoma 112 • Rhabdomyosarcoma (Corneal Edema) 113 • Sebaceous Gland Carcinoma 113 14. Mechanical and Non-Mechanical Injuries 114 • Acid Burns 114 • Alkaline Injury of the Eye 114 • Ciliary Body Concussions and Lacerations 115 • Conjunctival Lacerations 115 • Corneal Foreign Body 115 • Electrical Injury 116 • Extraocular Muscle Lacerations 116 • Eyelid Contusions, Lacerations and Avulsions 117 • Hypothermal Injury (Cryoinjury; Frostbite) 117 • Intraocular Foreign Body: Copper 117 • Intraocular Foreign Body: Nonmagnetic Chemically Inert 118 • Intraocular Foreign Body: Steel or Iron 118 • Lacrimal System Contusions and Lacerations 118 • Orbital Cellulitis and Abscess 119 • Orbital Compartment Syndrome, Acute 119 • Orbital Fracture, Apex 120 • Orbital Hemorrhages 120 • Orbital Implant Extrusion 120 • Orbital Infarction Syndrome 121 • Scleral Ruptures and Lacerations 121 • Shaken Baby Syndrome (Battered-Baby Syndrome; Battered-Child Syndrome; Child Abuse Syndrome; Silverman Syndrome) 122 • Thermal Burns 122 15. Unclassified Disease or Conditions 123 • Kimmelstiel-Wilson Syndrome (Diabetes Mellitus-Hypertension-Nephrosis Syndrome; Diabetes-Nephrosis Syndrome; Diabetic Glomerulosclerosis; Intercapillary Glomerulosclerosis; Renal Glomerulohyalinosis-Diabetic Syndrome) 123 • Papillorenal Syndrome (Renal-Coloboma Syndrome) 123 • Vogt-Koyanagi-Harada Disease (Harada Disease; Uveitis-VitiligoAlopecia-Poliosis Syndrome) 124 Contents xv 16. Anterior Chamber 125 • Anterior Chamber Cleavage Syndrome (Reese-Ellsworth Syndrome; Peters-Plus Syndrome) 125 • Anterior Segment Ischemia Syndrome 126 • Cholesterolosis of the Anterior Chamber 126 – Chronic Uveitis, Anterior Granulomatous (Iritis) 126 – Eales Disease (Periphlebitis) 127 – Subluxation, Dislocation of the Lens 127 – Microcephaly, Microphthalmia, Cataracts, and Joint Contractures 128 – Phthisis Bulbi 128 – Retinal Detachment/Giant Retinal Tears 128 – Traumatic Cataract 129 – Vitreous Hemorrhage 129 • Epithelial Ingrowth (Epithelial Downgrowth) 129 • Frenkel Syndrome (Ocular Contusion Syndrome; Anterior Segment Traumatic Syndrome) 130 • Gas Bubbles in the Anterior Chamber 130 – Clostridium Perfringens 130 – Escherichia Coli 131 • Glaucoma, Hyphema 131 • Hyphema (Bleeding into the Anterior Chamber) 132 – Fuchs (1) Syndrome (Heterochromic Cyclitis Syndrome) 132 – Gonorrhea 133 – Herpes Zoster 133 – Juvenile Rheumatoid Arthritis (JRA; Still Disease) 134 – Von Bekhterev-Strumpell Syndrome (Marie-Strumpell Spondylitis; Ankylosing Spondylitis; Pierre-Marie Syndrome; Bekhterev Disease; Rheumatoid Spondylitis) Optic atrophy 134 – Werlhof Disease (Hemophilia and Thrombocytopenic Purpura) 135 – Leukemia 135 – Henoch-Schönlein Purpura (Purpura; Anaphylactoid Purpura) 135 – Herrick Syndrome (Dresbach Syndrome; Sickle Cell Disease; Drepanocytic Anemia) 136 – Avitaminosis C (Scurvy; Vitamin C Deficiency) 136 – Iris Neovascularization with Pseudoexfoliation Syndrome 137 – Juvenile Xanthogranuloma (JXG; Nevoxanthoendothelioma) 137 – Retinoblastoma 138 – Pupillary Membrane, Persistent 138 • Hyphema (Traumatic Hyphema) 139 • UGH Syndrome (Uveitis-GlaucomaHyphema Syndrome) Glaucoma 139 • Hypopyon (Pus in Anterior Chamber) 139 – Acanthamoeba 141 – Acquired Immunodeficiency Syndrome (AIDS; Acquired Cellular Immunodeficiency; Acquired Immunodeficiency) 141 – Aspergillosis 142 – Candidiasis 142 – Alkaline Injury of the Eye 143 – Escherichia Coli 143 – Fusobacterium 144 – Herpes Simplex 144 – Herpes Zoster 144 – Measles (Morbilli; Rubeola) 145 – Moraxella Lacunata 145 – Gonorrhea 146 – Proteus Infections 146 – Pseudomonas Aeruginosa 147 – Smallpox (Variola) 147 – Staphylococcus 147 – Streptococcus (Scarlet Fever) 148 – Behçet Syndrome (Dermato-StomatoOphthalmic Syndrome; Oculobuccogenital Syndrome; Gilbert Syndrome) 148 – Toxic Lens Syndrome (Toxic Anterior Segment Syndrome; TASS) 149 – Acanthamoeba 149 – Actinomycosis 150 – Amebiasis (Amebic Dysentery, Entamoeba Histolytica) 150 – Aspergillosis 151 – Bacillus Cereus 151 – Behçet Syndrome (Dermato-StomatoOphthalmic Syndrome; Oculobuccogenital Syndrome; Gilbert Syndrome) 151 xvi Ocular Treatment: Evidence Based – Candidiasis 152 – Coccidioidomycosis (Valley Fever, San Joaquin Fever) 152 – Coenurosis (Tapeworm) 153 – Cysticercosis 153 – Fusobacterium 154 – Hydatid Cyst (Echinococcosis) 154 – Influenza 155 – Listerellosis (Listeriosis) 155 – Lockjaw (Tetanus) 156 – Metastatic Bacterial Endophthalmitis 156 – Moraxella Lacunata 156 – Mucormycosis (Phycomycosis) 157 – Pseudomonas Aeruginosa Infections 157 – Relapsing Fever (Recurrent Fever) 158 – Staphylococcus 158 – Streptococcus (Scarlet Fever) 159 – Behçet Syndrome (Dermato-StomatoOphthalmic Syndrome; Oculobuccogenital Syndrome; Gilbert Syndrome) 159 – Histiocytosis X (Hand-Schuller-Christian Syndrome; Lipoid Granuloma; Xanthomatous Granuloma Syndrome; Schuller-Christian-Hand Syndrome; Letterer-Siwe Syndrome; Acute Histiocytosis X; Eosinophilic Granuloma; Reticuloendotheliosis Syndrome) 160 – Juvenile Rheumatoid Arthritis (JRA; Still Disease) 160 – Leukemia 161 – Von Bekhterev-Strumpell Syndrome (Marie-Strumpell Spondylitis; Ankylosing Spondylitis; Pierre-Marie Syndrome; Bekhterev Disease; Rheumatoid Spondylitis) Optic atrophy 161 – Stevens-Johnson Syndrome [Dermatostomatitis; Erythema Multiforme Exudativum; Syndroma Mucocutaneo-Oculare; Baader Dermatostomatitis Syndrome; MucosalRespiratory Syndrome; Fuchs (2) Syndrome; Mucocutaneous Ocular Syndrome] 162 – Neovascularization, Corneal Contact Lens Related 162 – Tuberculosis 163 – Weil Disease (Leptospirosis) 163 – Yersiniosis 164 – Ghost Cell Glaucoma 164 – Primary Angle-Closure Glaucoma (Primary Closed Angle Glaucoma) 164 – Reticulum Cell Sarcoma (Non-Hodgkin Lymphoma) 165 • Intraocular Epithelial Cysts 165 • Mesodermal Dysgenesis (Anterior Chamber Dysgenesis; Dysembryogenesis; Anterior Segment Ocular Dysgenesis Syndrome) 166 • Neovascularization of Anterior Chamber Angle (Newly Formed Vessels Extend into the Trabecular Meshwork) 166 – Hemangioma 167 – Iris Melanoma 167 – Retinal Venous Obstruction 168 – Fungal Enophthalmitis 168 – Retinal Detachment, Rhegmatogenous 168 – Uveitis, Anterior Nongranulomatous (Iritis, Chronic) 169 – Takayasu Syndrome (Martorell Syndrome; Aortic Arch Syndrome; Pulseless Disease; Reversed Coarctation Syndrome) 169 – Carotid Artery Syndrome (Cavernous Sinus Fistula Syndrome; Red-Eyed Shunt Syndrome) 170 – Temporal Arteritis Syndrome (Cranial Arteritis Syndrome; Giant Cell Arteritis; HutchinsonHorton-Magath-Brown Syndrome) 170 – Coats Disease (Leber Miliary Aneurysm; Retinal Telangiectasia) 171 – Eales Disease (Periphlebitis) 171 – Malignant Melanoma of the Posterior Uvea (Choroidal Melanoma, Ciliary Body Melanoma, Uveal Melanoma, Intraocular Melanoma) 171 – Andersen-Warburg Syndrome (Whitnall-Norman Syndrome; Oligophrenia Microphthalmos Syndrome; Norrie Disease; Atrophia Oculi Congenital Fetal Iritis Syndrome; Congenital Progressive OculoAcoustico-Cerebral Dysplasia) 172 – Retinal Detachment, Rhegmatogenous 172 – Hemangioma 173 Contents xvii – Retinal Artery Occlusion 173 – Retinoblastoma 174 – Retrolental Fibroplasia (RLF; Retinopathy of Prematurity) 174 – Herrick Syndrome (Dresbach Syndrome; Sickle Cell Disease; Drepanocytic Anemia) 175 • Synechiae, Peripheral Anterior 175 • Peters Anomaly 176 • Postoperative Flat Anterior Chamber 176 • Recurrent Hyphema 177 • Shaken Baby Syndrome (Battered-Baby Syndrome; Battered-Child Syndrome; Child Abuse Syndrome; Silverman Syndrome) 177 • Spontaneous Hyphema 178 – Werlhof Disease (Hemophilia and Thrombocytopenic Purpura) 178 – Leukemia 179 – Lymphoid Hyperplasia (Reactive Lymphoid Hyperplasia; Lymphoid Tumors; Malignant Lymphoma; Pseudolymphoma; Pseudotumor; Burkitt Lymphoma; Neoplastic Angioendotheliomatosis) 179 – Henoch-Schönlein Purpura (Purpura; Anaphylactoid Purpura) 180 – Avitaminosis C (Scurvy; Vitamin C Deficiency) 180 – Retinoschisis (RS) 180 – Retrolental Fibroplasia (RLF; Retinopathy of Prematurity) 181 – Hypertension 181 – Juvenile Xanthogranuloma (JXG; Nevoxanthoendothelioma) 182 – Exophthalmos (Proptosis) 182 – Moraxella Lacunata 182 – Rubeosis Iridis 183 – Behçet Syndrome (DermatoStomato-Ophthalmic Syndrome; Oculobuccogenital Syndrome; Gilbert Syndrome) 184 – Diabetes Mellitus 184 – Gonorrhea 185 – Herpes Zoster 185 – Herpes Simplex 186 • Spontaneous Hyphema in Infants 186 – Juvenile Rheumatoid Arthritis (JRA; Still Disease) 186 – Anemia 187 – Leukemia 187 – Andersen-Warburg Syndrome (Whitnall-Norman Syndrome; Oligophrenia Microphthalmos Syndrome; Norrie Disease; Atrophia Oculi Congenital Fetal Iritis Syndrome; Congenital Progressive OculoAcoustico-Cerebral Dysplasia) 188 – Juvenile Xanthogranuloma (JXG; Nevoxanthoendothelioma) 188 – Persistent Hyperplastic Primary Vitreous (PHPV, Persistent Fetal Vasculature) 189 – Retinoblastoma 189 – Retinoschisis (RS) 190 – Retrolental Fibroplasia (RLF; Retinopathy of Prematurity) 190 • Toxic Lens Syndrome (Toxic Anterior Segment Syndrome; TASS) 191 • White Mass in Anterior Chamber 191 – Bacterial Endophthalmitis 191 – Aspergillosis 192 17. Choroid 193 • Angioid Streaks 193 – Bassen-Kornzweig Syndrome (Abetalipoproteinemia; Acanthocytosis; Familial Hypolipoproteinemia) 194 – Cooley Anemia (Thalassemia; Thalassemia Major; Thalassemia Minor) 194 – Paget Disease (Osteitis Deformans; Congenital Hyperphosphatemia; Hyperostosis Corticalis Deformans; Pozzi Syndrome; Chronic Congenital Idiopathic Hyperphosphatemia; Osteochalasis Desmalis Familiaris; Familial Osteoectasia) 195 – Ehlers-Danlos Syndrome (Fibrodysplasia Elastica Generalisata; Cutis Hyperelastica; Meekeren-EhlersDanlos Syndrome; Indian Rubber Man Syndrome; Cutis Laxa) 195 – Hallermann-Streiff Syndrome [Dyscephalic-Mandibulo-Oculo-Facial Syndrome; Oculo-MandibuloDyscephaly; Ullrich-Fremery-Dohna Syndrome; Francois Dyscephalic Syndrome; Mandibulo-Oculo-Facial Dyscephaly Syndrome; FrancoisHallermann-Streiff Syndrome; Hallermann-Streiff-Francois Syndrome; xviii Ocular Treatment: Evidence Based Audry I Syndrome; Dohna Syndrome; Francois Syndrome (1); DyscephalyTeeth Abnormality-Dwarfism; Dyscephalia OculomandibularisHypotrichosis; Mandibulo-Ocular Dyscephalia Hypotrichosis; Fremery-Dohna Syndrome; Oculo-Mandibulo-Facial Dyscephaly] 196 – Hemochromatosis 197 – Congenital Spherocytic Anemia (Congenital Hemolytic Jaundice; Hereditary Spherocytosis) 197 – Kasabach-Merritt Syndrome (Capillary Angioma-Thrombocytopenia; Hemangioma-Thrombocytopenia; Thrombocytopenia PurpuraHemangioma) 197 – Lead Poisoning 198 – Von Recklinghausen Syndrome (Neurofibromatosis Type I; Neurinomatosis) 198 – Conjunctival Melanotic Lesions (Conjunctival Nevus, Conjunctival Juncional Nevus, Subepithelial Nevus, Compound Nevus, Blue Nevus, Melanosis Oculi, Conjunctival Melanosis, Epithelial Nelanosis, Racial Melanosis, Sub-Epithelial Melanocytosis, Congenital Melanocytosis, Primary Acquired Melanosis, Malignant Melanoma, Conjunctival Melanoma, Conjunctival Malignant Melanoma) 199 – Pseudopapilledema (Optic Nerve Head Drusen) 199 – Paget Disease (Osteitis Deformans; Congenital Hyperphosphatemia; Hyperostosis Corticalis Deformans; Pozzi Syndrome; Chronic Congenital Idiopathic Hyperphosphatemia; Osteochalasis Desmalis Familiaris; Familial Osteoectasia) 200 – Choroidal Detachment (Ciliochoroidal Detachment) 200 – Grönblad-Strandberg Syndrome (Systemic Elastodystrophy; Pseudoxanthoma Elasticum; Elastorrhexis; Darier-GrönbladStrandberg Syndrome) 201 – Herrick Syndrome (Dresbach Syndrome; Sickle Cell Disease; Drepanocytic Anemia) 201 – Sturge-Weber Syndrome (Meningocutaneous Syndrome; Vascular Encephalotrigeminal Syndrome; Neuro-Oculocutaneous Angiomatosis; Encephalofacial Angiomatosis; Encephalotrigeminal Syndrome) 202 – Bourneville Syndrome (Bourneville-Pringle Syndrome; Tuberous Sclerosis; Epiloia) 202 • Central Serous Chorioretinopathy 203 • Choroid Coloboma 203 – Aicardi Syndrome 204 – Basal Cell Nevus Syndrome (Nevoid Basal Cell Carcinoma Syndrome; Nevoid Basalioma Syndrome; Gorlin Syndrome; Gorlin-Goltz Syndrome; Multiple Basal Cell Nevi Syndrome) 204 – Cat’s-Eye Syndrome (Schachenmann Syndrome; Schmid-Fraccaro Syndrome; Partial Trisomy G Syndrome) 205 – Charge Association (Multiple Congenital Anomalies Syndrome; Coloboma, Heart Disease, Atresia, Retarded Growth, Genital Hypoplasia, Ear Malformation Association) 206 – Crouzon Syndrome (Dysostosis Craniofacialis; Oxycephaly; Craniofacial Dysostosis; Parrot-Head Syndrome; Möbius-Crouzon Syndrome; Hereditary Craniofacial Dysostosis) 206 – Zollinger-Ellison Syndrome (Polyglandular Adenomatosis Syndrome; Multiple Endocrine Adenomatosis Partial Syndrome) Optic atrophy 207 – Hallermann-Streiff Syndrome (Dyscephalic-Mandibulo-Oculo-Facial Syndrome; Oculo-MandibuloDyscephaly; Ullrich-Fremery-Dohna Syndrome; Francois Dyscephalic Syndrome; Mandibulo-Oculo-Facial Dyscephaly Syndrome; FrancoisHallermann-Streiff Syndrome; Hallermann-Streiff-Francois Syndrome; Audry I Syndrome; Dohna Syndrome; Francois Syndrome (1); DyscephalyTeeth Abnormality-Dwarfism; Dyscephalia OculomandibularisHypotrichosis; Mandibulo-Ocular Dyscephalia Hypotrichosis; FremeryDohna Syndrome; Oculo-MandibuloFacial Dyscephaly) 207 Contents xix – Bloch-Sulzberger Syndrome (Incontinentia Pigmenti; SiemensBloch-Sulzberger Syndrome) 208 – Kartagener Syndrome (SinusitisBronchiectasis-Situs Inversus Syndrome; Bronchiectasis-Dextrocardia-Sinusitis; Kartagener Triad) 208 – Laurence-Moon-Bardet-Biedl Syndrome (Bardet-Biedl Syndrome; Retinitis Pigmentosa-Polydactyly-Adiposogenital Syndrome) 209 – Pierre-Robin Syndrome (Robin Syndrome; MicrognathiaGlossoptosis Syndrome) 209 – Stickler Syndrome (Hereditary Progressive Arthroophthalmopathy) 210 – Bourneville Syndrome (Bourneville-Pringle Syndrome; Tuberous Sclerosis; Epiloia) 210 – Goldenhar Syndrome (Oculo-Auriculo-Vertebral Dysplasia; Goldenhar-Gorlin Syndrome) 211 – Goltz Syndrome (Focal Dermal Hypoplasia Syndrome) 211 – Joubert Syndrome (Familial Cerebellar Vermis Agenesis) 212 – Klinefelter Syndrome (GynecomastiaAspermatogenesis Syndrome; XXY Syndrome; XXXY Syndrome; XXYY Syndrome; Reifenstein-Albright Syndrome) 212 – Lenz Microphthalmia Syndrome 213 – Linear Nevus Sebaceus of Jadassohn (Nevus Sebaceus of Jadassohn; Jadassohn-Type Anetoderma; Organoid Nevus syndrome; Sebaceus nevus syndrome) 213 – Frontonasal Dysplasia Syndrome (Median Cleft Face Syndrome) 214 – Meckel Syndrome (Dysencephalia Splanchnocystic Syndrome; Gruber Syndrome) 214 – Retinoblastoma 214 – Rubinstein-Taybi Syndrome (Broad-thumbs Syndrome) 215 – Triploidy Syndrome (Iris Coloboma) 215 – Trisomy 18 Syndrome (E Syndrome; Edwards Syndrome), Congenital Glaucoma 216 – Trisomy 13 Syndrome (Trisomy D1 Syndrome, Patau Syndrome, Reese Syndrome) Iris Coloboma 216 – Turner Syndrome (Turner-Albright Syndrome; Gonadal Dysgenesis; Genital Dwarfism Syndrome; Ullrich-Turner Syndrome; Bonnevie-Ullrich Syndrome; Pterygolymphangiectasia Syndrome; Ullrich-Bonnevie Syndrome) Cataract, Posterior 217 – Walker-Warburg Syndrome (Cerebro-ocular Dysplasia-Muscular Dystrophy; Warburg Syndrome; COD-MD Syndrome; Fukuyama Congenital Muscular Dystrophy; Hard + or – E Syndrome) Cataract 217 • Choroidal Detachment (Ciliochoroidal Detachment) 218 • Choroidal Folds 218 – Malignant Melanoma of the Posterior Uvea (Choroidal Melanoma, Ciliary Body Melanoma, Uveal Melanoma, Intraocular Melanoma) 219 – Junius-Kuhnt Syndrome [Kuhnt-Junius Disease; Macular Senile Disciform Degeneration (I); Macula Lutea Juvenile Degeneration (2)] 219 – Exophthalmos (P