Ocular Differential Diagnosis Frederick Hampton Roy
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Section 1
1Regional Signs and Symptoms2

OrbitChapter 1

Chapter Outline
  • ▪ Pseudoproptosis (Appearance of Exophthalmos) 3
  • ▪ Exophthalmos 4
  • ▪ Syndromes and Diseases Associated with Exophthalmos 6
  • ▪ Specific Exophthalmos 8
  • ▪ Enophthalmos 11
  • ▪ Intraorbital Calcifications On X-Ray 12
  • ▪ Orbital Bruit (Noise Heard Over Orbit with Stethoscope) 12
  • ▪ Orbital Emphysema (Air Found in Orbital Tissues and Adnexa Usually Demonstrable by Palpation) 13
  • ▪ Orbital Pain 13
  • ▪ Shallow Orbits or Diminished Orbital Volume (Illusion of Proptosis or Glaucoma) 14
  • ▪ Pseudohypertelorism (Illusion of Increased Distance Between Bony Orbits and Increased Interpupillary Distance) 14
  • ▪ Hypertelorism (Increased Distance Between Bony Orbits and Increased Interpupillary Distance) 14
  • ▪ Hypotelorism (Decreased Distance Between Bony Orbits and Decreased Interpupillary Distance) 16
  • ▪ Deep-Set Eyes 17
  • ▪ Prominent Supraorbital Ridges 17
  • ▪ Osteolysis of Bony Orbit on X-Ray 17
  • ▪ Fossa Formation of Orbit (Local Expansion of Bony Orbital Wall Caused by Persistent Pressure; Bony Cortex is Intact) 18
  • ▪ Subperiosteal Orbital Hemorrhage 18
  • ▪ Orbital Hemorrhage 18
  • ▪ Expansion of Orbital Margins (Usually Associated with Benign Tumors of the Orbit) 18
  • ▪ Hypertrophy of Orbital Bones (Hyperostosis or Sclerosis or Both) 19
  • ▪ Expansion of Optic Canal 19
  • ▪ Small Optic Canals 19
  • ▪ Erosion of Optic Canal 20
  • ▪ Enlargement of Superior Orbital Fissure 20
  • ▪ Narrowing of Superior Orbital Fissure 20
  • ▪ Small Orbit 20
  • ▪ Large Orbit 21
  • ▪ Hematic Orbital Cysts (Blood Cyst of Orbit) 22
 
PSEUDOPROPTOSIS (APPEARANCE OF EXOPHTHALMOS)
ICD9: 376.30
  1. *Asymmetry of bony orbits
  2. Congenital cystic eyeball
  3. *Contralateral enophthalmos
  4. Facial asymmetry as progressive facial hemiatrophy (Parry-Romberg syndrome)
  5. Harlequin orbit (shallow orbit with arched superior and lateral wall) as with hypophosphatasia
  6. Hypoplastic supraorbital ridges as in Trisomy 18 (Edward syndrome)
  7. Retraction of upper lid as with thyroid disease
  8. Slight blepharoptosis as with Horner syndrome of contralateral eye
  9. Shallow orbit as in Crouzon disease (Craniofacial dysostosis)
*Indicates most frequent
4
  1. Unilateral congenital glaucoma
  2. Unilateral high-axial myopia
  3. Unilateral secondary glaucoma resulting from ocular trauma during childhood
Bibliography
  1. Newell FW. Ophthalmology: Principles and Concepts. 7th Edition. CV Mosby;  St Louis:  1992.
  1. Rootman J. Diseases of the orbit. JB Lippincott;  Philadelphia:  1988.
 
EXOPHTHALMOS
ICD9: 376.30
  1. Drugs, including the following:
    • Adrenal cortex injection
    • Aldosterone
    • Beclomethasone
    • Betamethasone
    • Carbimazole
    • Cocaine
    • Cortisone
    • Desoxycorticosterone
    • Dexamethasone
    • Dextrothyroxine
    • Fludrocortisone
    • Fluorometholone
    • Fluprednisolone
    • Hydrocortisone
    • Iodide and iodine solution and compounds
    • Levothyroxine
    • Liothyronine
    • Liotrix
    • Lithium carbonate
    • Medrysone succinylcholine
    • Meprednisone
    • Methimazole
    • Methylprednisolone
    • Methylthiouracil
    • Oral contraceptives
    • Paramethasone
    • Poliovirus vaccine
    • Prednisolone
    • Prednisone
    • Propranolol
    • Propylthiouracil
    • Radioactive iodides
    • Thyroglobulin
    • Thyroid
    • Triamcinolone
    • Vitamin A
  2. Inflammation
    • Acute orbital cellulitis
    • Acute suppurative-mucormycosis (Diabetic or debility)
    • Allergic fungal sinusitis
    • Benign lymphoepithelial lesion (Mikulicz disease)
    • Chronic (Nongranulomatous-pseudotumor)
    • Chronic [Granulomatous-tuberculosis, Sarcoid (Schaumann syndrome), syphilis (lues), parasites, aspergillosis]
    • Relapsing polychondritis
  3. Injuries
    • Foreign body
    • Orbital hemorrhage
    • Orbital roof fracture
    • Secondary carotid cavernous sinus fistula
    • Thermal bums
  4. Systemic disease
    • Acute intracranial hypertension
    • Amyloidosis (Lubarsch-Pick syndrome)
    • Chloroma
    • Cretinism (Hypothyroidism)
    • Hydrocephalus and ventriculoperitoneal syndrome
    • Hypervitaminosis A
    • Hypophosphatasia (Phosphoethanolaminuria)
    • Thyroid disorder
    • Myasthenia gravis (Erb-Goldflam syndrome)
    • Obesity
  5. Tumors
    • Cartilaginous tumors
      • Cartilaginous hamartoma
      • Chondroma
      • Chondrosarcoma
      • Mesenchymal chondrosarcoma
    • Cystic lesions
      • Colobomatous cyst
      • Dermoid cyst
      • Hematocele
      • Hydatid cyst5
      • Meningocele and meningoencephalocele
      • Mucocele
      • Optic nerve sheath cyst
      • Simple epithelial cyst
      • Teratoma
    • Fibrocytic tumors
      • Fibroma
      • Fibrosarcoma
      • Fibrous histiocytoma
      • Juvenile fibromatosis
      • Nodular fasciitis
    • Histiocytic lesions
      • Others
        • Juvenile xanthogranuloma (JXG, nevoxanthoendothelioma)
        • Sinus histiocytosis with massive lymphadenopathy
      • Systemic histiocytoses (Histiocytosis X) (Hand-Schüller-Christian disease)
    • Inflammatory pseudotumor of orbit
      • Ectopic cerebellar tissue in orbit
      • Local, such as fungus or foreign body
      • Systemic such as sarcoidosis syndrome (Schaumann syndrome) or collagen disease
      • Unknown cause
    • Lacrimal gland (fossa) lesions
      • Epithelial tumors
        • Adenoid cystic carcinoma
        • Mucoepidermoid carcinoma
        • Pleomorphic adenocarcinoma (Malignant mixed tumor)
        • Pleomorphic adenoma (Benign mixed tumor)
      • Non-epithelial lesions
        • Infectious
        • Inflammatory
        • Lymphoid and leukemia
        • Systemic (Sarcoid)
    • Lipocytic and myxoid tumors
      • Lipoma
      • Liposarcoma
      • Myxoid liposarcoma
      • Myxoma
    • Lymphoid tumors and leukemias (excluding lacrimal gland lesions)
      • Benign reactive lymphoid hyperplasia
      • Burkitt lymphoma
      • Lymphoblastic leukemia
      • Myelogenous leukemia (Granulocytic sarcoma)
      • Non-Hodgkin lymphoma
    • Metastatic tumors of the orbit
      • Malignant melanoma of skin
      • Neuroblastoma (Child)
      • Other sites such as Ewing sarcoma
      • Primary in breast (Adult female)
      • Primary in lung (Adult male)
      • Primary in prostate (Adult male)
    • Nonepithelial lesions
      • Benign reactive lymphoid hyperplasia
      • Inflammatory pseudotumors (Dacryoadenitis)
      • Lymphoma
      • Plasmacytoma
    • Optic nerve and meningeal tumors
      • Juvenile pilocytic astrocytoma (Optic nerve glioma)
      • Meningioma
        • Primary optic nerve sheath
        • Secondary
      • Malignant optic nerve glioma
    • Osseous and fibro-osseous tumors
      • Aneurysmal bone cyst
      • Benign osteoblastoma
      • Brown tumor of hyperparathyroidism
      • Fibrous dysplasia (Albright syndrome)
      • Giant cell granuloma
      • Giant cell tumor (Osteoclastoma)
      • Infantile cortical hyperostosis
      • Ossifying fibroma
      • Osteoma
      • Osteosarcoma
    • Peripheral nerve tumors
      • Alveolar soft-part sarcoma
      • Amputation neuroma
      • Granular cell myoblastoma
      • Neurilemoma
        • Benign
        • Malignant
      • Neurofibroma6
        • Plexiform
        • Solitary
      • Paraganglioma (Chemodectoma)
    • Primary melanocytic tumors
      • Blue nevus
      • Melanocytic hamartoma
      • Melanotic progonoma (Retinal tumor)
      • Primary orbital melanoma
    • Rhabdomyoma and rhabdomyosarcoma
      • Rhabdomyoma
      • Rhabdomyosaroma
    • Secondary orbital tumors from adjacent structures
      • Conjunctival origin
        • Melanoma
        • Mucoepidermoid
        • Squamous cell carcinoma
      • Eyelid origin
        • Basal cell carcinoma
        • Melanoma
        • Sebaceous carcinoma
        • Squamous cell carcinoma
      • Intracranial origin
        • Astrocytoma
        • Meningioma
      • Intraocular origin
        • Medulloepithelioma
        • Neurilemoma
        • Retinoblastoma
        • Uveal melanoma
      • Nasopharyngeal origin
        • Angiofibroma
        • Carcinoma
        • Melanoma
      • Paranasal sinus origin
        • Ethmoid sinus carcinoma
        • Inverting papilloma
        • Maxillary sinus carcinoma
        • Rhabdomyosarcoma
    • Vasculogenic lesions
      • Capillary hemangioma
      • Cavernous hemangioma
      • Hemangiopericytoma
      • Hemangiosarcoma
      • Kaposi sarcoma
      • Lymphangioma
      • Varices
      • Vascular leiomyoma
      • Vascular leiomyosarcoma
  6. Vascular disorders
    • Allergic vasculitis
    • Angioedema (Quincke disease)
    • Arteriovenous aneurysm or varices
    • Arteriovenous fistula (Varicose aneurysm)
    • Collagen disease—lupus erythematosus (Kaposi-Libman Sacks syndrome), periarteritis nodosa (Kussmaul disease) or dermatomucomyositis (Wagner-Unverricht syndrome)
    • Cranial arteritis
    • Thrombophlebitis
    • Scurvy causing bilateral orbital hemorrhage
 
Additional Resources
  1. http://www.emedicine.com/oph/TOPIC616.HTM
  2. Cellulitis, Orbital http://www.emedicine.com/oph/TOPIC205.HTM
  3. Cellulitis, Preseptal http://www.emedicine.com/oph/TOPIC206.HTM
  4. Dermoid, orbital http://www.emedicine.com/oph/TOPIC620.HTM
  5. Fistula, Carotid Cavernous http://www.emedicine.com/oph/TOPIC204.HTM
  6. Hemangioma, Capillary http://www.emedicine.com/oph/TOPIC691.HTM
  7. Hemangioma, Cavernous http://www.emedicine.com/oph/TOPIC216.HTM
  8. Thyroid, Ophthalmopathy http://www.emedicine.com/oph/TOPIC237.HTM
  9. Tumors, Orbital http://www.emedicine.com/oph/TOPIC758.HTM
 
SYNDROMES AND DISEASES ASSOCIATED WITH EXOPHTHALMOS
ICD9: 376.30
  1. Actinomycosis
  2. Albright syndrome (Fibrous dysplasia)
  3. Amyloidosis (Lubarsch-Pick syndrome)
  4. Apert syndrome (Sphenoacrocraniosyndactyly)
  5. Arteriovenous fistula (Varicose aneurysm)
  6. Aspergillosis
  7. Bacillus cereus7
  8. Bloch-Sulzberger disease (Incontinentia pigment I)
  9. Bonnet-Dechaume-Blanc syndrome (Neuroretinoangiomatosis syndrome)
  10. Bourneville syndrome (Tuberous sclerosis)
  11. Caffey syndrome (Infantile cortical hyperostosis)
  12. Carotid artery-cavernous sinus fistula
  13. Clostridium perfringens
  14. Coenurosis
  15. Craniostenosis
  16. Cretinism (Hypothyroidism)
  17. Crouzon disease (Craniofacial dysostosis)
  18. Cryptococcosis
  19. Cushing syndrome (Adrenocortical syndrome)
  20. Dejean sign (Orbital floor fracture)
  21. de Lange syndrome (Congenital muscular hypertrophy-cerebral syndrome)
  22. Dermatomucomyositis (Polymyositis dermatomyositis)
  23. Dermoid
  24. Diencephalic epilepsy syndrome (Autonomic epilepsy syndrome)
  25. Dirofilariasis
  26. Dracontiasis (Guinea worm infection)
  27. Engelmann syndrome (Diaphyseal dysplasia)
  28. Ewing sarcoma
  29. Feer disease (Infantile acrodynia)
  30. Fibrosarcoma
  31. Fibrous dysplasia (Albright syndrome)
  32. Foix syndrome (Cavernous sinus thrombosis)
  33. Gardner syndrome
  34. Grönblad-Strandberg syndrome (Pseudoxanthoma elasticum)
  35. Hallermann-Streiff-François syndrome (Oculomandibulofacial dyscephaly)
  36. Hand-Schüller-Christian disease (Histiocytosis X)
  37. Heerfordt syndrome (Uveoparotid fever)
  38. Hemangiomas
  39. Herpes zoster
  40. Hodgkin disease
  41. Hollenhorst syndrome (Chorioretinal infarction syndrome)
  42. Horner syndrome (Cervical sympathetic paralysis syndrome)
  43. Hunter syndrome [MPS (mucopolysaccharidosis) II]
  44. Hurler (MPS I-H) syndrome
  45. Hutchinson disease (Adrenal cortex neuroblastoma with orbital metastasis)
  46. Hydatid cyst
  47. Hydrocephalus chondrodystrophicus congenita (Extreme hydrocephalus syndrome)
  48. Hypertension
  49. Hyperthyroidism (Basedow syndrome)
  50. Hypervitaminosis A
  51. Hypophosphatasia (Phosphoethanolaminuria)
  52. Jansen disease (Metaphyseal dysostosis)
  53. JXG (Nevoxanthoendothelioma)
  54. Kleeblattschädel syndrome (Cloverleaf skull)
  55. Leiomyoma
  56. Leopard syndrome (Multiple lentigines syndrome)
  57. Leprechaunism
  58. Leukemia
  59. Linear nevus sebaceous of Jadassohn
  60. Lupus erythematosus (Kaposi-Libman-Sacks syndrome)
  61. Lymphoid hyperplasia
  62. Lymphangioma
  63. Lymphosarcoma
  64. Melnick-Needles syndrome (Osteodysplasty)
  65. Meningioma
  66. Mikulicz syndrome (Dacryosialoadenopathy)
  67. Möbius disease (Congenital paralysis of sixth and seventh nerves)
  68. Mucocele
  69. Mucormycosis
  70. Multiple myeloma
  71. Mumps
  72. Myasthenia gravis (Erb-Goldflam syndrome)
  73. Neurilemoma
  74. Noonan syndrome (Male Turner syndrome)
  75. Osteopetrosis (Albers-Schönberg syndrome)
  76. Paget syndrome (Osteitis deformans)
  77. Periarteritis nodosa (Kussmaul syndrome)
  78. Periocular and ocular metastatic tumors
  79. Pfeiffer syndrome
  80. Pierre-Robin syndrome (Micrognathia-glossoptosis syndrome)
  81. Progeria (Hutchinson-Gilford syndrome)
  82. Quincke disease (Angioedema)8
  83. Relapsing polychondritis
  84. Retinoblastoma
  85. Rhabdomyosarcoma
  86. Rochon-Duvigneaud syndrome (Superior orbital fissure syndrome)
  87. Rollet syndrome (Orbital apex-sphenoidal syndrome)
  88. Sarcoidosis syndrome (Schaumann syndrome)
  89. Scaphocephaly syndrome
  90. Scheie syndrome (MPS I-S)
  91. Scurvy (Avitaminosis C)
  92. Sebaceous gland carcinoma
  93. Seckel syndrome (Bird-headed dwarf syndrome)
  94. Sézary syndrome (Mycosis fungoides syndrome)
  95. Shy-Gonatas syndrome (Orthostatic hypotension syndrome)
  96. Siegrist sign (Pigmented choroidal vessels)
  97. Silverman syndrome (Battered baby syndrome)
  98. Sphenocavernous syndrome
  99. Streptococcus
  100. Sturge-Weber syndrome (Encephalofacial angiomatosis)
  101. Syphilis (Lues)
  102. Thermal bums
  103. Trichinellosis
  104. Trisomy 18 syndrome (Edward syndrome)
  105. Tuberculosis
  106. Turner syndrome (Gonadal dysgenesis)
  107. von Hippel-Lindau syndrome (Retinocerebral angiomatosis)
  108. von Recklinghausen disease (Neurofibromatosis)
  109. Wegener syndrome (Wegener granulomatosis)
 
Additional Resource
  1. http://www.emedicine.com/oph/TOPIC616.HTM
*Indicates most frequent
 
SPECIFIC EXOPHTHALMOS
ICD9: 376.30
  1. Age
    • Newborn-most common
      • *Orbital sepsis
      • Orbital neoplasm including congenital malignant teratoid neoplasm
    • Neonatal-osteomyelitis of the maxilla
    • Early childhood (up to 1 year of age-most common)
      • *Dermoid
      • *Hemangioma
      • Dermolipoma
      • Histiocytosis X including Hand-Schüller-Christian disease
      • *Orbital extension of retinoblastoma
    • 1–5 years-most common
      • *Dermoid
      • Metastatic neuroblastoma
      • Rhabdomyosarcoma
      • Epithelial cyst such as sebaceous cyst and epithelial inclusion cyst
      • Glioma of optic nerve
      • Sphenoid wing meningioma
      • *Orbital extension of retinoblastoma
      • Fibrous dysplasia (Albright syndrome)
      • Metastatic embryonal sarcoma
      • *Hemangioma
    • 5–10 years-most common
      • Pseudotumor
      • Orbital extension of retinoblastoma
      • Malignant lymphomas and leukemias
      • *Dermoid
      • *Hemangioma
      • Meningioma
      • Fibrous dysplasia (Albright syndrome)
      • Rhabdomyosarcoma
      • Orbital hematoma
      • Glioma of optic nerve
    • 10–30 years-most common
      • *Pseudotumor
      • Mucocele
      • Meningioma
      • *Endocrine ophthalmopathy (Thyroid-related ophthalmopathy)
      • Lacrimal gland tumor
      • Malignant lymphomas and leukemias
      • Dermoid
        9
      • Hemangioma
      • Peripheral nerve tumors
      • Undifferentiated sarcomas
      • Osteoma
      • Fibrous dysplasia (Albright syndrome)
      • Rhabdomyosarcoma
      • Glioma of optic nerve
    • 30–50 years-most common
      • *Pseudotumor
      • Mucocele
      • Malignant lymphomas and leukemias
      • *Hemangioma
      • *Endocrine ophthalmopathy (Thyroid-related ophthalmopathy)
      • Lacrimal gland tumors
      • Rhinogenic carcinoma
      • Malignant melanoma
      • Osteosarcoma
      • Fibrosarcoma
      • Metastatic carcinoma
      • Meningioma
      • Dermoid
    • 50–70 years-most common
      • *Pseudotumor
      • *Mucocele
      • *Malignant lymphomas and leukemias
      • Dermoid
      • Carcinoma of palpebral or epibulbar origin
      • *Meningioma
      • *Endocrine ophthalmopathy (Thyroid-related ophthalmopathy)
      • Lacrimal gland tumor
      • Osteosarcoma
      • Fibrosarcoma
      • Undifferentiated sarcoma
      • Metastatic carcinoma
      • Osteoma
      • Fibrous dysplasia (Albright syndrome)
      • Neurofibroma
      • Hemangioma
    • More than 70 years-most common
      • Melanoma
      • Pseudotumor
      • *Lymphoma
      • *Metastatic tumor
      • Basal cell carcinoma
      • Mucocele
*Indicates most frequent
  1. Unilateral exophthalmos-most common
    • Anatomical conditions
      • Unilateral myopia of high degree
      • Defects in the vault of the orbit: meningocele, encephalocele, hydroencephalocele
      • Exophthalmos associated with arterial hypertension
      • Recurrent exophthalmos from retrobulbar hemorrhage, lymphangioma
      • Intermittent exophthalmos associated with venous anomalies within the cranium
      • Disease of the pituitary gland; meningiomas involving sphenoid ridge
      • *Unilateral exophthalmos associated with endocrine or thyroid-related ophthalmopathy
    • Traumatic conditions
      • Contralateral floor fracture with enophthalmos
      • Fracture of the orbit with retrobulbar hemorrhage
      • Laceration and rupture of the tissues of the orbit and the extraocular muscles
      • Intracranial trauma sustained at birth; aneurysm in orbit
      • Pulsating carotid-cavernous fistula from carotid-cavernous aneurysm
      • Spontaneous retrobulbar hemorrhage as seen in whooping cough
      • Chronic subdural hematoma bulging into orbit
      • Posterior exophthalmos (Orbital apex lesion)
        • Pseudotumor
        • Malignant tumor
        • Benign tumor
        • Vascular disease
        • Infection
          10
    • Inflammatory conditions
      • Retrobulbar abscess and cellulitis
      • Thrombophlebitis of the orbital veins
      • Cavernous sinus thrombosis
      • Erysipelas (St. Anthony fire)
      • Tenonitis
      • Periostitis (Syphilitic or tuberculous)
      • Orbital mucocele, pyocele; cholesteatoma
      • Orbital exostosis
      • Paget disease with hyperostosis
      • Actinomycosis, trichinosis, myotic pseudotumor
      • Herpes (HSV-l) with acute retinal necrosis
    • Disease of blood, lymph and hematopoietic system
      • Rickets (Avitaminosis D)
      • Scurvy (Avitaminosis C)
      • Hemophilia (Factor VIII deficiency)
      • Lymphosarcoma
      • Chloroma
      • Hodgkin disease
    • Space-taking lesions
      • Vascular anomalies
        • Congenital orbital varix (Young patient with systemic abnormalities)
        • Cavernous hemangioma (Middle age)
        • Capillary hemangioma (young children) Kasabach-Merrit syndrome
        • Lymphangiomas
      • Orbital tumors: pseudotumors, orbital cysts, meningocele, lymphangioma, orbital meningioma, lacrimal gland tumor, sarcoma, metastatic carcinoma, metastatic adrenal tumors, osteomas arising in the accessory nasal sinuses, tumors of the nasopharynx (benign and malignant)
      • Intracranial tumor with orbital extension including chordoma and meningioma
    • Unilateral exophthalmos in children
      • Inflammation
      • Vascular disorders
      • Neoplasms
      • Metabolic diseases
      • Developmental anomalies
      • Others
      • *Orbital cellulitis
  2. Bilateral exophthalmos-most common
    • *Thyroid
    • Orbital myositis (Owing to causes other than thyroid dysfunction)
    • Cavernous sinus thrombosis *(Foix syndrome)*
    • Metastatic neuroblastoma
    • Hand-Schüller-Christian disease (Histiocytosis X)
    • *Crouzon disease *(Craniofacial dysostosis)
    • *Paget disease *(Osteitis deformans)
  3. Type proptosis-most common
    • Straightforward-glioma of optic nerve, intraconal cavernous hemangioma
    • Down and temporal-mucocele of frontal sinus
    • Down and nasal-lacrimal gland lesion
    • Downward-tumor of roof of orbit
    • Upward-tumor of floor of orbit
  4. Transient exophthalmos
    • *Orbital varices
    • Orbital varices with intracranial extension
    • Arteriovenous malformations
    • Cavernous hemangioma
    • Intraorbital arteriovenous malformation
  5. Pulsating exophthalmos-most common
    • *Carotid-cavernous fistula
    • *von Recklinghausen disease associated with bony defect of skull
    • Large frontal mucocele
    • Meningoencephalocele
    • Blow-in fracture of roof of orbit
    • Neurofibromatosis
    • Fistula
    • Malignancies
    • Mucoceles
    • Orbital varix
    • Dermoid cysts
    • Aneurysm
*Indicates most frequent
  1. Recurrent exophthalmos
    • Recurrent orbital inflammation (pseudo– tumor) or hemorrhage
    • Orbital cysts that rupture11
    • Lymphangioma (children)
    • Syndrome of intermittent exophthalmos-congenital venous malformations of the orbit: venous angioma and orbital varix
    • Temporal lobe tumor with orbital extension
    • Neurofibromatosis
    • Vascular neoplasm
  2. Intermittent exophthalmos
    • Orbital varices
    • Recurrent hemorrhage
    • Vascular neoplasm
    • Lymphangioma
  3. Pseudotumor associated with conjunctival chemosis, restricted movement of eyes because of pain-pseudotumor
  4. Cavernous sinus thrombosis in an acutely ill patient-cavernous sinus thrombosis
  5. Exophthalmos associated with engorged conjunctival episcleral vessels
    • Nonpulsating-cerebral arteriovenous angioma, ophthalmic vein thrombosis or cavernous sinus thrombosis
    • Pulsating exophthalmos-carotid-cavernous sinus fistula
  6. Exophthalmos associated with a palpable mass in region of the lacrimal gland
    • Primary inflammatory exophthalmos
    • Neoplasm
    • *Sarcoidosis syndrome *(Schaumann syndrome)
    • Hodgkin disease
  7. Orbital mucormycosis in patient with uncontrolled diabetes, usually with acidosis, who develops unilateral lid edema, ptosis, internal and external ophthalmoplegia, proptosis and severe vision loss-orbital mucormycosis
  8. Exophthalmos in an infant with ecchymosis of the eyelids
    • Metastatic neuroblastoma
    • Orbital leukemia infiltration
  9. Bilateral exophthalmos from bilateral orbital pseudotumor
    • Eosinophilic granuloma
    • Retroperitoneal fibrosis
    • Myasthenia gravis (Erb-Goldflam syndrome)
 
Additional Resource
  1. http://www.emedicine.com/oph/TOPIC616.HTM
*Indicates most frequent
 
ENOPHTHALMOS
ICD9: 376.50
  1. Senility (common)
  2. Wasting diseases-loss of orbital fat
  3. Injury-blowout fracture of floor of orbit (most common)
  4. Orbital varices-transient exophthalmos with fat atrophy
  5. Chronic or severe liver or gallbladder disease (usually in right eye owing to increased tone of orbicularis muscle and extraocular muscles)
  6. Iatrogenic
    • Orbital decompression
    • Sinus surgery
  7. Superior sulcus deformity
    • Traumatic bony loss
    • Atrophy of the orbital tissues
    • Levator detachment with ptosis
    • Migration of muscle cone implant
    • Herniated orbital fat secondary to an orbital fracture
  8. Associated syndromes
    • Arthrogryposis (Amyoplasia congenital)
    • Babinski-Nageotte syndrome (Medullary tegmental paralysis)
    • Cestan-Chenais syndrome (Lesion in the lateral portion of medulla oblongata)
    • Cockayne syndrome (Dwarfism with retinal atrophy and deafness)
    • Craniocervical syndrome (Whiplash injury)
    • Cretinism (Hypothyroidism)
    • Cryptophthalmia syndrome
    • Dejean syndrome (Orbital floor syndrome)
    • Dejerine-Klumpke syndrome (Thalamic hyperesthetic anesthesia)
    • Duane retraction syndrome
    • Freeman-Sheldon syndrome (Craniocarpotarsal dysplasia)
    • General fibrosis syndrome
      12
    • Greig syndrome (Ocular hypertelorism syndrome)
    • Hemifacial microsomia syndrome (François-Haustrate syndrome)
    • Horner syndrome (Cervical sympathetic paralysis syndrome)
    • Klippel-Trenaunay-Weber syndrome (Angioosteohypertrophy syndrome)
    • Krause syndrome (Encephaloophthalmic syndrome)
    • Maple syrup urine disease (Branched chain ketoaciduria)
    • Morquio syndrome (MPS IV)
    • Naffziger syndrome (Scalenus anticus syndrome)
    • Pancoast syndrome (Superior pulmonary sulcus syndrome)
    • Parry-Romberg syndrome (Progressive facial hemiatrophy)
    • Passow syndrome (Bremer status dysraphicus)
    • Raeder syndrome (Paratrigeminal paralysis)
    • Retroparotid space syndrome
    • Silent sinus syndrome
    • Vernet syndrome (Jugular foramen syndrome)
    • von Herrenschwand syndrome (Sympathetic heterochromia)
    • Wallenberg syndrome (Dorsolateral medullary syndrome)
  9. Apparent enophthalmos with horizontal conjugate gaze
  10. Metastatic adenocarcinoma of orbit (Cicatricial)
  11. Neurofibromatosis (Pulsating enophthalmos)
  12. Typhoid fever (Abdominal typhus)
 
Additional Resource
  1. http://www.emedicine.com/oph/TOPIC617.HTM
 
INTRAORBITAL CALCIFICATIONS ON X-RAY
ICD9: 376.9
  1. Calcification of more irregular configuration and texture
    • Cysticercosis
    • Orbital hematoma
    • Plexiform neurofibroma
    • Toxoplasmosis
    • Tuberculosis
  2. Calcification of orbital vessels
    • Atheromatous plaque
    • Monkeberg sclerosis
    • Secondary to metabolic-endocrine disturbances such as hyperparathyroidism or hypervitaminosis
    • Band-shaped keratopathy
  3. Chronic inflammatory and parasitic disease of the orbit
  4. Hemangiopericytoma
  5. Intraocular calcifications following
    • Congenital deformity
    • Malignant lacrimal gland tumor
    • Recurrent iritis and keratitis
    • Retinal detachment
    • Trauma (Perforating, nonperforating or surgical)
  6. Intraocular sarcoma
  7. Mucocele
  8. Myositis ossificans
  9. Orbital phleboliths: helical form in veins-smooth, round or oval
  10. Organized hematomas of the orbit
  11. Retinoblastoma
  12. Retrolental fibroplasia
  13. Sites of intraocular calcification
    • Cyclitic membrane
    • Lens
    • Peripapillary choroid
    • Posterior pole to ora serrata in region of choroid and pigment epithelium
    • Retina
    • Vitreous
 
Additional Resource
  1. http://www.emedicine.com/oph/TOPIC671.HTM
 
ORBITAL BRUIT (NOISE HEARD OVER ORBIT WITH STETHOSCOPE)
ICD9: 376.89
  1. Bilateral
    • Hyperthyroidism
    • Severe anemias
    13
  2. Unilateral
    • *Abnormal communication in the cavernous sinus (i.e. bilateral carotid-cavernous sinus)
    • Aneurysmal angioma of orbit or fundus such as in Wyburn-Mason syndrome (Bonnet-Dechaume-Blanc syndrome)
    • Arteriovenous aneurysm (arteriovenous fistula)
    • Intermittent or pulsating exophthalmos
    • Stenosis of carotid artery including thrombosis, sclerosis or external pressure such as that due to an outer-ridge sphenoid meningioma
Bibliography
  1. Kushner FH. Carotid-cavernous fistula as a complication of carotid endarterectomy. Ann Ophthalmol. 1981;13:979.
  1. Malzone WF, Gonyea EF. Exophthalmos with intracranial arteriovenous malformations. Neurology. 1973;23:534–8.
 
ORBITAL EMPHYSEMA (AIR FOUND IN ORBITAL TISSUES AND ADNEXA USUALLY DEMONSTrable BY PALPATION)
ICD9: 376.89
  1. *Due to fracture of ethmoid sinuses or orbital floor
  2. Following forceful blowing of nose
  3. Injury from compressed air
  4. Orbital cellulitis and abscess with gas formation by infecting organism
  5. Osteomyelitis and infected sinus with fistulous communication with gas formation by infecting organism
  6. Resulting from use of high-speed dental drill and air-water spray during oral operation
  7. Subconjunctival emphysema seen with mechanical ventilation
Bibliography
  1. Buckley MJ, et al. Orbital emphysema causing vision loss after a dental extraction. J Am Dent Assoc. 1990;120:421–2.
  1. Hunts JH, et al Orbital emphysema: staging and acute management. Ophthalmology. 1994;101:960–6.
 
ORBITAL PAIN
ICD9: 379.91
  1. Acute dacryoadenitis
  2. Amputation neuroma of the orbit
  3. Associated syndromes
    • Cavernous sinus thrombosis syndrome
    • Charlin syndrome (Nasal nerves syndrome)
    • Erysipelas
    • Ophthalmoplegic migraine syndrome
    • Raeder syndrome (Paratrigeminal paralysis)
    • Tolosa-Hunt syndrome (Painful ophthalmoplegia).
  4. Break-bone fever (Dengue fever)
  5. Clostridium perfringens
  6. Eye strain from uncorrected errors of refraction
  7. Myositis
    • Collagen diseases
    • Infectious myositis
    • Trichinosis
  8. Orbital cellulitis or abscess
  9. Orbital periostitis because of injury, tuberculosis, syphilis, extension of sinus disease or other conditions
  10. *Pseudotumor or tumor of the orbit-pain infrequently present
  11. Retrobulbar neuritis
  12. Trauma
  13. Tumors of cerebellopontine angle, frequent lesion of seventh nerve
*Indicates most frequent
Bibliography
  1. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. WB Saunders;  Philadelphia:  1994. pp:1881-2095.
  1. Bullen CL, Younge BR. Chronic orbital myositis. Ophthalmology. 1982;89:1749.
  1. Lanzino G, et al. Orbital pain and unruptured carotid-posterior communicating artery aneurysms: the role of sensory fibers of the third cranial nerve. Acta Neurochir (Wien). 1993;120:7–11.
  1. 14 Roy FR. Ocular Syndromes and Systemic Diseases. 4th Edition. Lippincott Williams & Wilkins;  Philadelphia:  2007.
 
SHALLOW ORBITS OR DIMINISHED ORBITAL VOLUME (ILLUSION OF PROPTOSIS OR GLAUCOMA)
  1. Aminopterin-induced sign
  2. Apert syndrome
  3. Carpenter syndrome
  4. Cerebrohepatorenal syndrome (Smith-Lemli-Opitz syndrome)
  5. Craniostenosis
  6. Crouzon disease (Craniofacial dysostosis)
  7. Diseases of nasal passages and sinuses
    • Dentigerous cysts
    • Fibrous dysplasia (Albright syndrome)
    • Hypoplasia of maxilla associated with chronic maxillary sinusitis
    • Rhinoscleroma
  8. Dubowitz syndrome
  9. Early enucleation of eye
  10. Familial hypoplasia of orbital margin
  11. Frontometaphyseal dysplasia (FMD)
  12. Hyperostosis (Hypertrophy of orbital bones)
  13. Hypophosphatasia-Harlequin orbit (Shallow orbit with arched superior and lateral wall)
  14. Kleeblattschädel syndrome
  15. Lateral displacement of medial orbital wall by hypertrophic polypoid nasal sinus disease
  16. Marshall-Smith syndrome
  17. Oculoauriculovertebral dysplasia (Goldenhar syndrome)
  18. Osteogenesis imperfecta (van der Hoeve syndrome)
  19. Radiation injury of bone
  20. Robert syndrome (Pseudothalidomide syndrome)
  21. Saethre-Chotzen syndrome
  22. Secondary to fracture
  23. Stanesco dysostosis syndrome
  24. Trisomy 13-(trisomy D) (Patau syndrome)
  25. Trisomy 18 (Edward syndrome)
  26. Zellweger syndrome
  27. 6q-D syndrome
  28. 9p– syndrome
Bibliography
  1. Cursiefen C, et al. Adenoma of the nonpigmented ciliary epithelium mimicking a malignant melanoma of the iris. Arch Ophthalmol. 1999;117:113–8.
  1. Roy FR. Ocular Syndromes and Systemic Diseases. 4th Edition. Lippincott Williams & Wilkins,  Philadelphia:  2007.
  1. Smith DW. Recognizable Patterns of Human Malformation. 4th Edition. WB Saunders,  Philadelphia:  1988.
 
PSEUDOHYPERTELORISM (ILLUSION OF INCREASED DISTANCE BETWEEN BONY ORBITS AND INCREASED INTERPUPILLARY DISTANCE)
  1. Blepharophimosis
  2. *Epicanthal skin folds
  3. Exotropia
  4. *Flat nasal bridge of nose
  5. Increased distance between the inner canthi (Telecanthus)
  6. Widely spaced eyebrows
*Indicates most frequent
Bibliography
  1. DeMyer W. The median cleft face syndrome. Neurology. 1967;17:961.
 
HYPERTELORISM (INCREASED DISTANCE BETWEEN BONY ORBITS AND INCREASED INTERPUPILLARY DISTANCE)
ICD9: 376.41
  1. Aarskog syndrome (Faciodigitogenital syndrome)
  2. Albers-Schönberg disease (Osteopetrosis)
  3. Aminopterin-induced syndrome
  4. Apert syndrome (Acrodysplasia)
  5. Association of hypertelorism, microtia and facial clefting
    15
  6. Baraitser-Winter syndrome
  7. BBB syndrome (Hypertelorism-hypospadias syndrome)
  8. Blatt syndrome (Cranioorbitoocular dysraphia)
  9. Carpenter syndrome (Acrocephalopolysyndactyly II)
  10. Cat's eye syndrome (Schachenmann syndrome)
  11. Cerebral gigantism (Sotos syndrome)
  12. Cerebrohepatorenal syndrome (Zellweger)
  13. Cherubism
  14. Chromosome partial long-arm deletion syndrome (de Grouchy syndrome)
  15. Chromosome partial short-arm deletion syndrome [monosomy partial (short-arm) syndrome]
  16. Chondrodystrophia calcificans congenita (Conradi syndrome)
  17. Cleft lip and palate sequence
  18. Cleidocranial dysostosis syndrome
  19. Coffin-Lowry syndrome
  20. Congenital hemihypertrophy
  21. Craniocarpotarsal syndrome (Freeman-Sheldon syndrome)
  22. Craniocleidodysostosis syndrome (Marie-Sainton syndrome)
  23. Craniosynostosis-radial aplasia (Baller-Gerold syndrome)
  24. Cretinism (Hypothyroidism)
  25. Cri-du-chat syndrome (Cry of the cat syndrome)
  26. Crouzon disease (Craniofacial dysostosis)
  27. Cryptophthalmos syndrome
  28. Curtius syndrome (Ectodermal dysplasia with ocular malformations)
  29. Diamond-Blackfan syndrome
  30. DiGeorge sequence
  31. Down syndrome (Mongolism)
  32. Dubowitz syndrome (Dwarfism-eczema-peculiar facies)
  33. Duplication 14Q syndrome
  34. Ehlers-Danlos syndrome (Fibrodysplasia elastica generalisata)
  35. Engelmann syndrome (Diaphyseal dysplasia)
  36. Faciooculoacousticorenal syndrome
  37. Familial characteristic
  38. Familial metaphyseal dysplasia (Pyle disease)
  39. Fetal alcohol syndrome
  40. Fetal aminopterin effects
  41. Fetal hydantoin effects
  42. Fish odor syndrome
  43. 4Q syndrome
  44. Frontonasal dysplasia syndrome (Median cleft face syndrome)
  45. Frontal encephaloceles
  46. Gorlin syndrome (Orodigitofacial dysostosis)
  47. Greig syndrome (Hypertelorism)
  48. Haney-Falls syndrome (Congenital keratoconus posticus circumscriptus)
  49. Hurler syndrome (MPS I-H)
  50. Hydrocephalus
  51. Hypomelanosis of Ito syndrome (Systematized achromic nevus)
  52. Ichthyosis (Collodion baby)
  53. Infantile gigantism
  54. Infantile hypercalcemia with supravalvular aortic stenosis (Williams-Beuren syndrome)
  55. Iris dysplasia-hypertelorism-psychomotor retardation syndrome
  56. Jacobs syndrome (Triple X syndrome)
  57. KBG syndrome (Initials of family studied)
  58. Kleeblattschädel syndrome (Extreme hydrocephalus syndrome)
  59. Klein syndrome
  60. Klinefelter XXY syndrome (Gynecomastia-aspermatogenesis syndrome)
  61. Klippel-Feil syndrome (Synostosis cervical vertebrae)
  62. Larsen syndrome
  63. Leprechaunism
  64. Lissencephalia (Miller-Dieker syndrome)
  65. Little syndrome (Nail-patella syndrome)
  66. Mandibulofacial dysostosis (Franceschetti syndrome)
  67. Maple syrup urine disease (Branched-chain ketoaciduria)
  68. Marfan syndrome (Arachnodactyly-dystrophia-mesodermalis congenita)
  69. Marshall-Smith syndrome
  70. Meckel-Gruber syndrome
  71. Melnick-Needles syndrome (Osteodysplasty)16
  72. Metaphyseal dysostosis (Jansen disease)
  73. McFarland syndrome
  74. Morquio-Ullrich syndrome (MPS IV)
  75. Multiple basal cell nevi (Gorlin-Goltz syndrome)
  76. Multiple lentigines syndrome (Leopard syndrome)
  77. Myelomeningocele-Chiari malformations
  78. Noonan syndrome (Male Turner syndrome)
  79. Oculodentodigital syndrome
  80. Oculomandibulofacial dyscephaly (Hallermann-Streiff syndrome)
  81. Optic nerve hypoplasia
  82. Orofaciodigital (OFD) type I and type II (Mohr syndrome)
  83. Osteogenesis imperfecta (van der Hoeves syndrome)
  84. Otopalatodigital syndrome (OPD syndrome)
  85. Pallister-Killian syndrome
  86. Pfeiffer syndrome
  87. Potter syndrome (Renofacial syndrome)
  88. Ring D chromosome
  89. Ring chromosome
  90. Rieger syndrome (Dysgenesis mesostromalis)
  91. Roberts syndrome (Pseudothalidomide syndrome)
  92. Robinow syndrome (Fetal face syndrome)
  93. Saethre-Chotzen syndrome (Acrocephalosyndactyly type III)
  94. Sjögren-Larsson syndrome (Oligophrenia-ichthyosis-spastic diplegia syndrome)
  95. Sprengel syndrome
  96. Traumatic nasoorbital fracture
  97. Triploidy syndrome
  98. Trisomy 6q syndrome
  99. Trisomy 9q syndrome
  100. Trisomy 13 (Patau syndrome)
  101. Trisomy 17p syndrome
  102. Trisomy 8 syndrome
  103. Turner syndrome (Gonadal dysgenesis)
  104. Waardenburg syndrome (Embryonic fixation syndrome)
  105. Williams-Beuren syndrome
  106. XXXXX syndrome
  107. XXXXY syndrome
  108. 4p-syndrome (Wolf syndrome)
  109. 13q syndrome
Bibliography
  1. Pallotta R. Iris coloboma ptosis, hypertelorism, and mental retardation: a new syndrome possibly localized on chromosome 2. J Med Genet. 1991;28:342–4.
  1. Roy FH. Ocular Syndromes and Systemic Diseases. 4th Edition. Lippincott Williams & Wilkins;  Philadelphia:  2007.
  1. Seaver LH, Cassidy SB. New syndrome: mother and son with hypertelorism, downslanting palpebral fissures, malar hypoplasia, and apparently low-set ears associated with joint and scrotal anomalies. Am J Med Genet. 1991;41:405–9.
 
HYPOTELORISM (DECREASED DISTANCE BETWEEN BONY ORBITS AND DECREASED INTERPUPILLARY DISTANCE)
  1. Arrhinencephaly (Holoprosencephaly)
  2. Cebocephalia
  3. Cockayne syndrome (Dwarfism with retinal atrophy and deafness)
  4. Coffin-Lowry syndrome
  5. Ethmocephalus
  6. Familial
  7. François diencephalic syndrome (Hallerman-Streiff syndrome)
  8. Goldenhar syndrome (Oculoauriculovertebral dysplasia)
  9. Maternal phenylketonuria fetal effects
  10. Meckel-Gruber syndrome
  11. Median cleft lip (Frontonasal dysplasia syndrome)
  12. Median philtrum-premaxilla anlage
  13. Ocular-dental-digital dysplasia (Meyer-Schivickerath and Weyers syndrome)
  14. Trigonocephaly (C syndrome, Opitz trigonocephaly syndrome)
  15. Trisomy 13 (Patau syndrome, trisomy D syndrome)17
  16. Trisomy 20 syndrome
  17. Trisomy (Down syndrome, mongolism)
  18. Turner syndrome (Gonadal dysgenesis)
  19. Wolf syndrome (Monosomy partial syndrome)
  20. 18p syndrome
Bibliography
  1. Evans DG. Dominantly inherited microcephaly, hypotelorism and normal intelligence. Clin Genet. 1991;39:178–80.
  1. Judisch GF, et al. Orbital hypotelorism. Arch Ophthalmol. 1984;102:995–7.
  1. Richieri-Costa A, et al. Mental retardation, microbrachycephaly, hypotelorism, palpebral ptosis, thin/long face, cleft lip, and lumbosacral/pelvic anomalies. Am J Med Genet. 1992;43:565–8.
  1. Roy FH. Ocular Syndromes and Systemic Diseases. 4th Edition. Lippincott Williams & Wilkins;  Philadelphia:  2007.
 
DEEP-SET EYES
  1. Cockayne syndrome (Dwarfism with retinal atrophy and deafness)
  2. Craniocarpotarsal syndrome (Freeman-Sheldon syndrome)
  3. Familial
  4. Marfan syndrome (Dolichostenomelia-arach– nodactylyhyperchondroplasia-dystrophia mesodermalis congenita)
  5. Mesodermal dysmorphodystrophy (Weill– Marchesani syndrome)
  6. Oculocerebrorenal syndrome (Lowe syndrome)
  7. Pyknodysostosis
  8. Syndrome of blepharophimosis with myopathy
Bibliography
  1. Aita JA. Congenital facial anomalies with neurologic defects. Charles C Thomas;  Springfield  IL:1969.
 
PROMINENT SUPRAORBITAL RIDGES
ICD9: 376.9
  1. Apert syndrome (Acrocephalosyndactylism syndrome)
  2. Basal cell nevus syndrome (Gorlin-Goltz syndrome)
  3. Cleidocranial dysostosis (Marie-Sainton syndrome)
  4. Congenital lipodystrophy
  5. Congenital syphilis (Congenital lues)
  6. Ectodermal dysplasia (Curtius syndrome)
  7. *Frontometaphyseal dysplasia
  8. Hurler syndrome (MPS I-H)
  9. Marfan síndrome (Arachnodactyly-dystrophia mesodermalis congenita)
  10. Otopalatodigital syndrome
  11. Pyle metaphyseal dysplasia syndrome
*Indicates most frequent
Bibliography
  1. Albert DM, Jakobiec FA. Principles and practice of ophthalmology. WB Saunders,  Philadelphia:  1994. pp.1881-2095.
  1. Roy FH. Ocular Syndromes and Systemic Diseases. 4th Edition. Lippincott Williams & Wilkins;  Philadelphia:  2007.
 
OSTEOLYSIS OF BONY ORBIT ON X-RAY
ICD9: 376.9
  1. Autoimmune diseases such as Wegener granulomatosis
  2. Congenital
  3. Hyperparathyroidism
  4. Injury such as blowout fracture of orbital floor
  5. Meningocele and encephalocele of orbit
  6. Metastasis from remote primary neoplasms
  7. Primary orbital disease
    • Infectious, including tuberculosis and syphilis
    • Neoplastic, including neurofibroma and lacrimal gland tumor
    • Cystic, including dermoid and epidermoid cyst
  8. Reticuloendotheliosis as histiocytosis X (Hand-Schüller-Christian disease)
  9. Secondary extension of infectious or neoplastic disease from adjacent sinuses, brain, skin, bone, nasopharynx and esophagus
  10. Sinus disease including mucoceles
18
Bibliography
  1. Margo CE, et al. Psammomatoid ossifying fibroma. Arch Ophthalmol. 1986;104:1347–51.
  1. Oh KT, et al. Adenocarcinoma of the esophagus presenting as an orbital cellulitis. Arch Ophthol. 2000;118:986–8.
 
FOSSA FORMATION OF ORBIT (LOCAL EXPANSION OF BONY ORBITAL WALL CAUSED BY PERSISTENT PRESSURE; BONY CORTEX IS INTACT)
ICD9: 376.9
  1. Encapsulated benign lacrimal gland tumor
  2. Encapsulated malignant lacrimal gland tumor
  3. Orbital dermoid
Bibliography
  1. Albert DM, Jakobiec FA. Principles and practice of ophthalmology. WB Saunders;  Philadelphia:  1994. pp:1881-2095.
  1. Jacobs L, et al. Computerized tomography of the orbit and sella turcica. Raven Press;  New York:  1980.
  1. Zizmor J. Orbital radiology in unilateral exophthalmos. In: Turtz AI. Proceedings of the Centennial Symposium: Manhattan Eye. Ear, and Throat Hospital. Val I. CV Mosby;  St. Louis:  1969.
 
SUBPERIOSTEAL ORBITAL HEMORRHAGE
ICD9: 376.32
  1. Generalized diseases with bleeding diatheses
  2. Paranasal sinusitis
  3. Sudden elevation of cranial venous pressure
  4. Trauma
Bibliography
  1. Atalla ML, et al. Nontraumatic subperiosteal orbital hemorrhage. Ophthalmology. 2001;108:183–9.
  1. Hunt KE, Ross JJ. Orbital hemorrhage in the nonoperated eye as a complication of general endotracheal anesthesia. Arch Ophthalmol. 1998;116L:105–6.
 
ORBITAL HEMORRHAGE
ICD9: 376.32
  1. Idiopathic including myositis and pseudotumor and in the laboring mother caused by Valsalva maneuver
  2. Other
    • General endotracheal anesthesia
    • Late migration of orbital implant
  3. Pre-existing vascular tumors
    • Cholesterol granuloma
    • Cystic lymphangiomas
    • Hemorrhagic varix
  4. Surgery
    • Retrobulbar injection
    • Rhinoplasty
    • Subtentorial infusion anesthesia
    • Intraorbital, orbital and paranasal surgery
    • Following blepharoplasty
    • Following dacryocystorhinostomy
  5. Systemic
    • Heparin
    • Thrombolytic
    • Vascular anomalies, blood dyscrasias and idiopathic spontaneous hemorrhage
  6. Trauma
  7. Systemic diseases including leukemia, hemophilia, hypertension, uremia, scurvy, sickle cell disease, malaria and hepatitis
  8. Newborn including complicated delivery, hemorrhagic diathisis and oribtal tumor
Bibliography
  1. Chorich LJ, et al. Hemorrhagic ocular complications associated with the use of systemic thrombolytic agents. Ophthalmology. 1998;105:428–31.
  1. Polito E, et al. Diagnosis and treatment of orbital hemorrhagic lesions. Ann Opthalmol. 1994;26:85–93.
 
EXPANSION OF ORBITAL MARGINS (USUALLY ASSOCIATED WITH BENIGN TUMORS OF THE ORBIT)
ICD9: 376.81
  1. Dermoid
  2. Hemangioma19
  3. Lacrimal gland tumors
  4. Meningioma
  5. Neurofibroma
Bibliography
  1. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. WB Saunders;  Philadelphia:  1994; pp:1881-2095.
  1. Coleman DJ, et al. Ultrasonography of the Eye and Orbit. Lea & Febiger;  Philadelphia:  1977.
 
HYPERTROPHY OF ORBITAL BONES (HYPEROSTOSIS OR SCLEROSIS OR BOTH)
ICD9: 376.81
  1. Acromegaly
  2. Anemias of childhood (Severe: Cooley, sickle cell, spherocytosis, iron deficiency)
  3. Cerebral atrophy (Childhood)
  4. Craniostenosis
  5. Engelmann disease (Hereditary diaphyseal dysplasia)
  6. Hyperostosis frontalis interna
  7. Idiopathic
  8. Infantile cortical hyperostosis (Caffey disease)
  9. Microcephaly
  10. Myotonia atrophica (Myotonic dystrophy, Curschmann-Steinert syndrome)
  11. Osteopetrosis (Albers-Schönberg disease)
  12. Paget disease (Osteitis deformans)
  13. Tumors of orbit, including osteoma, fibrous dysplasia (Albright syndrome), meningioma, metastatic neuroblastoma, mixed tumors of lacrimal gland, transitional cell carcinomas of the nasopharynx
Bibliography
  1. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. WB Saunders;  Philadelphia:  1994. pp:1881-2095.
  1. Jacobs L, et al. Computerized tomography of the orbit and sella turcica. Raven Press;  New York:  1980.
  1. Teplick JG, Hoskin ME. Roentgenologic Diagnosis. 3rd Edition. WB Saunders;  Philadelphia:  1976.
 
EXPANSION OF OPTIC CANAL
ICD9: 377.53
  1. Increased intracranial pressure
  2. Inflammatory lesions
    • Chiasmatic arachnoiditis
    • Nonspecific granuloma
    • Sarcoid granuloma
    • Tuberculoma
  3. Tumors
    • Meningioma
    • Metastatic sarcoma to choroid
    • Neurofibromatosis (von Recklinghausen syndrome)
    • Optic nerve glioma
    • Retinoblastoma
  4. Vascular lesions
    • Arteriovenous malformation
    • Ophthalmic artery aneurysm
Bibliography
  1. Levin LA, Rubin PD. Advances in orbital imaging. Int Ophthalmol Clin. 1992;32:1–25.
  1. Potter GD, Trakel SL. Optic canal. In: Newton TH, Potts DG (Eds). Radiology of the Skull and Brain Vol 1, Book 2. CV Mosby;  St Louis:  1971.
  1. Zizmor J. Orbital radiology in unilateral exophthalmos. In: Turtz AI (Ed). Proceedings of the Centennial Symposium: Manhattan Eye, Ear, and Throat Hospital, Vol I. CV Mosby;  St. Louis:  1969.
 
SMALL OPTIC CANALS
ICD9: 377.54
  1. Developmental abnormalities
    • Anophthalmos or microphthalmos
    • Enucleation
    • Craniosynostosis (CSO)
  2. Dysostoses
    • Osteopetrosis (Albers-Schönberg syndrome)
    • Fibrous dysplasia (Albright syndrome)
    • Pyle disease (Craniometaphyseal dysplasia syndrome)
    • Paget disease (Osteitis deformans)
  3. Inflammatory lesions-osteitis
  4. Tumor-meningioma
20
Bibliography
  1. Albert DM, Jakobiec FA. Principles and practice of ophthalmology. WB Saunders;  Philadelphia:  1994. pp:1881-2095.
  1. Potter GD, Trokel SL. Optic canal. In: Newton TH, Potts DG (Eds). Radiology of the Skull and Brain, Vol 1, Book 2. CV Mosby;  St. Louis:  1971.
 
EROSION OF OPTIC CANAL
ICD9: 377.51
  1. Lateral wall
    • Pituitary tumor
    • Aneurysm of internal carotid artery
    • Craniopharyngioma
    • Tumor of orbital apex
  2. Medial wall
    • Carcinoma of sphenoid sinus
    • Mucocele of sphenoid sinus
    • Granuloma of sphenoid sinus
  3. Roof
    • Tumor of anterior cranial fossa
    • Surgical unroofing
  4. Decrease in length
    Tumor of orbital apex
  5. Complete destruction
    • Malignant tumor
    • Eosinophilic granuloma
Bibliography
  1. Roy FH. Ocular Syndromes and Systemic Diseases. 4th Edition. Lippincott Williams & Wilkins;  Philadelphia:  2007.
 
ENLARGEMENT OF SUPERIOR ORBITAL FISSURE
  1. Carotid cavernous fistula
  2. Chronic increased intracranial pressure
  3. Extension of infraorbital mass into fissure
  4. Intracavernous carotid aneurysm
  5. Intracranial chordoma
  6. Masses within middle fossa
  7. Metastatic carcinoma to sphenoid wings
  8. Nasopharyngeal carcinoma-rare
  9. Neurofibromatosis including optic nerve glioma
  10. Orbital dysplasia
  11. Orbital varix
  12. Pituitary neoplasm-changes in sella and clinoid process
  13. Posterior orbital encephalocele
  14. Sarcomas, neurilemoma or other orbital malignancies
Bibliography
  1. Ferry AP, et al. Orbital invasion by an intracranial chordoma. Am J Ophthalmol. 1981;92:7–12.
  1. Grimson BS, Perry DD. Enlargement of the optic disk in childhood optic nerve tumors. Am J Ophthalmol. 1984;97:627–31.
 
NARROWING OF SUPERIOR ORBITAL FISSURE
ICD9: 376.89
  1. Chronic hemolytic anemias of childhood
  2. Fibrous dysplasia (Albright syndrome)
  3. Meningioma
  4. Osteitis
  5. Osteoblastoma
  6. Osteoma
  7. Osteopetrosis (Albers-Schönberg syndrome)
  8. Paget's disease (Osteitis deformans)
Bibliography
  1. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. WB Saunders;  Philadelphia:  1994. pp:1881-2095.
  1. Jacobs L, et al. Computerized tomography of the orbit and sella turcica. Raven Press,  New York:  1980.
  1. Kieffer SA. Superior orbital fissure. In: Newton TH, Potts DG (Eds). Radiology of the Skull and Brain, Vol 1, Book 2. CV Mosby;  St Louis:  1971.
 
SMALL ORBIT
ICD9: 376.40
  1. Anophthalmos
  2. Enucleation
  3. Microphthalmos
  4. Mucocele
21
Bibliography
  1. Kieffer SA. Orbit. In: Newton TH, Potts DG (Eds). Radiology of the Skull and Brain, Vol 1, Book 2. CV Mosby;  St Louis:  1971.
  1. Sarnat BG. Eye and orbital size in the young and adult. Ophthalmologica. 1982;185:74–89.
 
LARGE ORBIT
ICD9: 376.40
  1. Congenital
    • Dysplasia
    • Glaucoma
    • Serous cysts
  2. Pseudotumor
  3. Tumors within the muscle cone
    • Hemangiomas
    • Neurofibroma
    • Optic glioma
    • Orbital varix
    • Retinoblastoma
Bibliography
  1. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. WB Saunders;  Philadelphia:  1994. pp:1881-2095.
  1. Kieffer SA. Orbit. In: Newton TH, Potts DG (Eds). Radiology of the Skull and Brain, Vol 1, Book 2. CV Mosby;  St Louis:  1971.
 
HEMATIC ORBITAL CYSTS (BLOOD CYST OF ORBIT)
ICD9: 376.32
  1. Blood dyscrasia
  2. Cavernous hemangioma
  3. Childbirth
  4. Lymphangioma
  5. Orbital blunt trauma
  6. Spontaneous hemorrhage
  7. Vascular disease
Bibliography
  1. Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. WB Saunders;  Philadelphia:  1994. pp:1881-2095.
  1. Jacobson DM, et al. Maternal orbital hematoma associated with labor. Am J Ophthalmol. 1988;105:547–53.
  1. Shapiro A, et al. A clinicopathologic study of hematic cysts of the orbit. Am J Ophthalmol. 1986;102:237–41.