Ocular Differential Diagnosis Eighth Edition Ocular Differential Diagnosis



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Ocular Differential Diagnosis

Eighth Edition
Ocular Differential Diagnosis
Eighth Edition
Frederick Hampton Roy, M.D., F.A.C.S.

Little Rock, Arkansas

© 2007 by Frederick Hampton Roy


Published by F.E.P. International
All rights reserved. This book is protected by copyright. No part of this book may be reproduced in any form or by any means, including photocopying, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above-mentioned copyright.
Previous editions: 1972, 1977, 1982, 1987, 1992, 1997, 2002

Printed in the USA


ISBN X-XXXX-XXXX-X

Care has been taken to confirm the accuracy of the information presented and to describe generally accepted practices. However, the author and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. Application of this information in a particular situation remains the professional responsibility of the practitioner.

The author and publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accordance with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new or infrequently employed drug.

Some drugs and medical devices presented in this publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings. It is the responsibility of the health care provider to ascertain the FDA status of each drug or device planned for use in their clinical practice.

To Mary Michelle

To my children:

Nichols, Robert, Kimberly, Frederick, Jr., Charles, and Helena

To Dr. Arlington Krause,

molder and questioner in my early formative academic life

To Dr. Philip Lewis and Dr. Roger Hiatt

for guidance and direction

============= Contents =============


Preface ix
How to Use This Book xi
I. Regional Signs and Symptoms

1. Orbit 3

2. Lids 45

3. Lacrimal System 110

4. Extraocular Muscles 127

5. Conjunctiva 183

6. Globe 220

7. Sclera 231

8. Cornea 241

9. Intraocular Pressure 305

10. Anterior Chamber 329

11. Pupil 344

12. Iris 364

13. Lens 386

14. Vitreous 421

15. Retina 433

16. Choroid 526

17. Optic Nerve 559

18. Visual-Field Defects 604
II. General Signs and Symptoms

19. Visual Disturbance 619

20. Visual Complaint 644

21. Head Position 665

Subject Index 669

Preface
The first edition of Ocular Differential Diagnosis was published in 1972, and various editions have been translated in Spanish, Turkish, Chinese, Portuguese, and Italian. All previous editions have been my work. Dr. Gonzolo Murillo of La Paz, Bolivia, helped in edition four and five with the diagnostic decision tables. In edition six, section editors helped to standardize the language and made helpful suggestions. I feel this is the best edition of Ocular Differential Diagnosis thus far. This text would not have been possible without the superb efforts of Renee Tindall, Dr. Fernando Murillo, Angie Brown, and Dr. Kae Chatman.

The Ocular Differential Diagnosis book provides comprehensive lists of causes for symptoms or findings. Frequently more information is needed and hopefully Ocular Syndromes and Systemic Diseases will furnish additional information to make a better diagnosis. I hope the ophthalmologist and optometrist using this book will bring any errors in this edition to my attention.


Frederick Hampton Roy, M.D.

Little Rock, Arkansas



How to Use This Book

This book can be used easily and quickly by following the directions presented below.


1. If the sign or symptom relates to a particular region of the eye, turn to the table of contents preceding this page to find the number of the page on which listings of the signs and symptoms pertaining to the specific region begins. This latter page (or those immediately following) will refer the user to that (or those) on which the various causes of the condition are listed. For example, let us assume that the patient has pigmentation of the cornea. The table of contents on page vii shows that the cornea section begins on page 241. Turning to page 241 the user finds references to page 248 on which the causes of corneal pigmentation are listed according to type. In the subject index, this topic is listed as Cornea, pigmentation of, 248.

2. If the symptom, such as binocular diplopia or night blindness, does not relate to a particular region of the eye, look for it either in the subject index at the back of the book or under General Signs and Symptoms beginning on page 619.

Various features of a disease may be crosschecked. For instance, a "pulsating exophthalmos with orbital bruit and conjunctival edema" may be sought under orbit, page 3, where the user of the book is referred to exophthalmos, page 4, and orbital bruit, page 31, and under conjunctiva, page 183, where the user is referred to conjunctival edema, page 202. The terms "exophthalmos," "orbital bruit" (under orbit, bruit of) and "conjunctival edema" (under conjunctiva, edema of) may also be found in the subject index. Terms such as "secondary glaucoma" are indexed under the noun, e.g., glaucoma, secondary.

3. Following some of the differential diagnosis lists are diagnostic decision tables. These tables list the history, physical signs, and laboratory tests that differentiate each possible diagnosis. These can be identified in the subject index because they are followed by a t.



Ocular Differential Diagnosis

Eighth Edition
PART I

Regional Signs and Symptoms


1

Orbit
CONTENTS

Pseudoproptosis 4

Exophthalmos 4

Syndromes and diseases associated with exophthalmos 8

Specific exophthalmos 11

Diagnostic tables 16

Up to one year 16

One to five years 17

Five to ten years 19

Ten to thirty years 21

Thirty to fifty years 22

Fifty to seventy years 24

More than seventy years 26

Pulsating exophthalmos 28

Recurrent exophthalmos 29

Enophthalmos 15

Intraorbital calcifications 31

Orbital bruit 31

Diagnostic table 32

Orbital emphysema 33

Orbital pain 33

Shallow orbits or diminished orbital volume 34

Pseudohypertelorism 35

Hypertelorism 35

Hypotelorism 38

Deep-set eyes 38

Prominent supraorbital ridges 38

Osteolysis of bony orbit 39

Fossa formation of orbit 39

Subperiosteal orbital hemorrhage 39

Orbital hemorrhage 40

Expansion of orbital margins 40

Hypertrophy of orbital bones 40

Expansion of optic canal 41

Small optic canals 41

Erosion of optic canal 42

Enlargement of superior orbital fissure 42

Narrowing of superior orbital fissure 43

Small orbit 43

Large orbit 43

Hematic orbital cysts 43

PSEUDOPROPTOSIS (APPEARANCE OF EXOPHTHALMOS)

*1. Asymmetry of bony orbits

2. Congenital cystic eyeball

*3. Contralateral enophthalmos (see p. 15)

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 (Edward syndrome)

7. Retraction of upper lid as with thyroid disease

8. Slight blepharoptosis as with Homer syndrome of contralateral eye

9. Shallow orbit as in Crouzon disease (craniofacial dysostosis)

10. Unilateral congenital glaucoma

11. Unilateral high-axial myopia

12. Unilateral secondary glaucoma resulting from ocular trauma during childhood
Newell FW. Ophthalmology: principles and concepts, 7th ed. St. Louis: CV Mosby, 1992.
Rootman J. Diseases of the orbit. Philadelphia: JB Lippincott, 1988.
EXOPHTHALMOS

1. Drugs, including the following:



adrenal cortex injection

aldosterone

beclomethasone

betamethasone

carbimazole

cocaine


cortisone

desoxycorticosterone

dexamethasone

dextrothyroxine

fludrocortisone

fluorometholone

flu-prednisolone

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

A. Acute-orbital cellulitis

B. Acute suppurative-mucormycosis (diabetic or debility)

C. Allergic fungal sinusitis

D. Benign lymphoepithelial lesion (Mikulicz disease)

*E. Chronic (nongranulomatous-pseudotumor)

F. Chronic (granulomatous-tuberculosis, sarcoid (Schaumann syndrome), syphilis (lues), parasites, aspergillosis)

G. Relapsing polychondritis

3. Injuries

A. Foreign body

B. Orbital hemorrhage

C. Orbital roof fracture

D. Secondary carotid cavernous sinus fistula

E. Thermal bums

4. Systemic disease

A. Acute intracranial hypertension

B. Amyloidosis (Lubarsch-Pick syndrome)

C. Chloroma

D. Cretinism (hypothyroidism)

E. Hydrocephalus and ventriculoperitoneal syndrome

F. Hypervitaminosis A

G. Hypophosphatasia (phosphoethanolaminuria)

*H. Thyroid disorder

I. Myasthenia gravis (Erb-Goldflam syndrome)

J. Obesity

5. Tumors

A. Cartilaginous tumors

(1) Cartilaginous hamartoma

(2) Chondroma

(3) Chondrosarcoma

(4) Mesenchymal chondrosarcoma

B. Cystic lesions

(1) Colobomatous cyst

(2) Dermoid cyst

(3) Hematocele

(4) Hydatid cyst

(5) Meningocele and meningoencephalocele

(6) Mucocele

(7) Optic nerve sheath cyst

(8) Simple epithelial cyst

(9) Teratoma

C. Fibrocytic tumors

(1) Fibroma

(2) Fibrosarcoma

(3) Fibrous histiocytoma

(4) Juvenile fibromatosis

(5) Nodular fasciitis

D. Histiocytic lesions

(1) Others

a. Juvenile xanthogranuloma (JXG, nevoxanthoendothelioma)

b. Sinus histiocytosis with massive lymphadenopathy

(2) Systemic histiocytoses (histiocytosis X) (Hand-Schüller-Christian disease)

E. Inflammatory pseudotumor of orbit

(1) Ectopic cerebellar tissue in orbit

(2) Local, such as fungus or foreign body

(3) Systemic such as sarcoidosis syndrome (Schaumann syndrome) or collagen disease

(4) Unknown cause

F. Lacrimal gland (fossa) lesions

(1) Epithelial tumors

a. Adenoid cystic carcinoma

b. Mucoepidermoid carcinoma

c. Pleomorphic adenocarcinoma (malignant mixed tumor)

d. Pleomorphic adenoma (benign mixed tumor)

(2) Nonepithelial lesions

a. Infectious

b. Inflammatory

c. Lymphoid and leukemia

d. Systemic (sarcoid)

G. Lipocytic and myxoid tumors

(1) Lipoma

(2) Liposarcoma

(3) Myxoid liposarcoma

(4) Myxoma

H. Lymphoid tumors and leukemias (excluding lacrimal gland lesions)

(1) Benign reactive lymphoid hyperplasia

(2) Burkitt lymphoma

(3) Lymphoblastic leukemia

(4) Myelogenous leukemia (granulocytic sarcoma)

(5) Non-Hodgkin lymphoma

I. Metastatic tumors of the orbit

(1) Malignant melanoma of skin

(2) Neuroblastoma (child)

(3) Other sites such as Ewing sarcoma

(4) Primary in breast (adult female)

(5) Primary in lung (adult male)

(6) Primary in prostate (adult male)

J. Nonepithelial lesions

(1) Benign reactive lymphoid hyperplasia

(2) Inflammatory pseudotumors (dacryoadenitis)

(3) Lymphoma

(4) Plasmacytoma

K. Optic nerve and meningeal tumors

(1) Juvenile pilocytic astrocytoma (optic nerve glioma)

(2) Meningioma

a. Primary optic nerve sheath

b. Secondary

(3) Malignant optic nerve glioma

L. Osseous and fibroosseous tumors

(1) Aneurysmal bone cyst

(2) Benign osteoblastoma

(3) Brown tumor of hyperparathyroidism

(4) Fibrous dysplasia (Albright syndrome)

(5) Giant cell granuloma

(6) Giant cell tumor (osteoclastoma)

(7) Infantile cortical hyperostosis

(8) Ossifying fibroma

(9) Osteoma

(10) Osteosarcoma

M. Peripheral nerve tumors

(1) Alveolar soft-part sarcoma

(2) Amputation neuroma

(3) Granular cell myoblastoma

(4) Neurilemoma

a. Benign

b. Malignant

(5) Neurofibroma

a. Plexiform

b. Solitary

(6) Paraganglioma (chemodectoma)

N. Primary melanocytic tumors

(1) Blue nevus

(2) Melanocytic hamartoma

(3) Melanotic progonoma (retinal tumor)

(4) Primary orbital melanoma

O. Rhabdomyoma and rhabdomyosarcoma

(1) Rhabdomyoma

(2) Rhabdomyosarcoma

P. Secondary orbital tumors from adjacent structures

(1) Conjunctival origin

a. Melanoma

Mucoepidermoid

c. Squamous cell carcinoma

(2) Eyelid origin

a. Basal cell carcinoma

b. Melanoma

c. Sebaceous carcinoma

d. Squamous cell carcinoma

(3) Intracranial origin

a. Astrocytoma

b. Meningioma

(4) Intraocular origin

a. Medulloepithelioma

b. Neurilemoma

c. Retinoblastoma

d. Uveal melanoma

(5) Nasopharyngeal origin

a. Angiofibroma

b. Carcinoma

c. Melanoma

(6) Paranasal sinus origin

a. Ethmoid sinus carcinoma

b. Inverting papilloma

c. Maxillary sinus carcinoma

d. Rhabdomyosarcoma

Q. Vasculogenic lesions

(1) Capillary hemangioma

(2) Cavernous hemangioma

(3) Hemangiopericytoma

(4) Hemangiosarcoma

(5) Kaposi sarcoma

(6) Lymphangioma

(7) Varices

(8) Vascular leiomyoma

(9) Vascular leiomyosarcoma

6. Vascular disorders

A. Allergic vasculitis

B. Angioedema (Quincke disease)

C. Arteriovenous aneurysm or varices

D. Arteriovenous fistula (varicose aneurysm)

E. Collagen disease-lupus erythematosus (Kaposi-Libman-Sacks syndrome), periarteritis nodosa (Kussmaul disease), or dermatomucomyositis (Wagner-Unver-richt syndrome)

F. Cranial arteritis

G. Thrombophlebitis

H. Scurvy causing bilateral orbital hemorrhage
Archer KF, et al. Orbital nonchromaffin paraganglioma. Ophthalmology 1989; 96:1659-1666.
Carter K, et al. Ophthalmic manifestations of allergic fungal sinusitis. Am J OphthalmoI 1999; 127:189-195.
Fraunfelder FT, Fraunfelder FW. Drug-induced ocular side effects. Woburn, MA: Butterworth-Heinemann, 2001.
Luxenberg MN. Chloroma. Arch Ophthalmol 1991; 109:734-736.
Lyon DB, et al. Epithelioid hemangioendothelioma of the orbital bones. Ophthalmology 1993; 99:1773-1778.
Newman NJ, et al. Ectopic brain in the orbit. Ophthalmology 1986; 93:268-272.
Shields JA, et al. Classification and incidence of space-occupying lesions of the orbit. Arch Ophthalmol 1984; 102:- 1606-1611.
Sloan B, et al. Scurvy causing bilateral orbital hemorrhage. Arch OphthalmoI 1999; 117:842.
SYNDROMES AND DISEASES ASSOCIATED WITH EXOPHTHALMOS
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 cereus

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. Homer 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. Pyknodysostosis

83. Quincke disease (angioedema)

84. Relapsing polychondritis

85. Retinoblastoma

86. Rhabdomyosarcoma

87. Rochon-Duvigneaud syndrome (superior orbital fissure syndrome)

88. Rollet syndrome (orbital apex-sphenoidal syndrome)

89. Sarcoidosis syndrome (Schaumann syndrome)

90. Scaphocephaly syndrome

91. Scheie syndrome (MPS I-S)

92. Scurvy (avitaminosis C)

93. Sebaceous gland carcinoma

94. Seckel syndrome (bird-headed dwarf syndrome)

95. Sézary syndrome (mycosis fungoides syndrome)

96. Shy-Gonatas syndrome (orthostatic hypotension syndrome)

97. Siegrist sign (pigmented choroidal vessels)

98. Silverman syndrome (battered baby syndrome)

99. Sphenocavernous syndrome

100. Streptococcus

101. Sturge-Weber syndrome (encephalofacial angiomatosis)

102. Syphilis (lues)

103. Thermal bums

104. Trichinellosis

105. Trisomy syndrome (Edward syndrome)

106. Tuberculosis

107. Turner syndrome (gonadal dysgenesis)

108. von Hippel-Lindau syndrome (retinocerebral angiomatosis)

109. von Recklinghausen disease (neurofibromatosis)

110. Wegener syndrome (Wegener granulomatosis)


Goodman RM, Gorlin RJ. The face in genetic disorders. 51. Louis: CV Mosby, 1970.
Miller NR, et al. Lytic Paget disease as a cause of orbital cholesterol granuloma. Arch Ophthalmol I999; 117: 1084-1085.
Roy PH. Ocular syndromes and systemic diseases, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 2002.
SPECIFIC EXOPHTHALMOS

1. Age


A. Newborn-most common

*(1) Orbital sepsis

(2) Orbital neoplasm including congenital malignant teratoid neoplasm

B. Neonatal-osteomyelitis of the maxilla

C. Early childhood (up to 1 year of age-most common)

*(1) Dermoid

*(2) Hemangioma

(3) Dermolipoma

(4) Histiocytosis X including Hand-Schüller-Christian disease

*(5) Orbital extension of retinoblastoma

D. One to five years-most common

*(1) Dermoid

(2) Metastatic neuroblastoma

(3) Rhabdomyosarcoma

(4) Epithelial cyst, such as sebaceous cyst and epithelial inclusion cyst

(5) Glioma of optic nerve

(6) Sphenoid wing meningioma

*(7) Orbital extension of retinoblastoma

(8) Fibrous dysplasia (Albright syndrome)

(9) Metastatic embryonal sarcoma

*(10) Hemangioma

E. Five to ten years-most common

(1) Pseudotumor

(2) Orbital extension of retinoblastoma

(3) Malignant lymphomas and leukemias

*(4) Dermoid

*(5) Hemangioma

(6) Meningioma

(7) Fibrous dysplasia (Albright syndrome)

(8) Rhabdomyosarcoma

(9) Orbital hematoma

(10) Glioma of optic nerve

F. Ten to thirty years-most common

*(1) Pseudotumor

(2) Mucocele

(3) Meningioma

*(4) Endocrine ophthalmopathy (thyroid-related ophthalmopathy)

(5) Lacrimal gland tumor

(6) Malignant lymphomas and leukemias

(7) Dermoid

(8) Hemangioma

(9) Peripheral nerve tumors

(10) Undifferentiated sarcomas

(11) Osteoma

(12) Fibrous dysplasia (Albright syndrome)

(13) Rhabdomyosarcoma

(14) Glioma of optic nerve

G. Thirty to fifty years-most common

*(1) Pseudotumor -

(2) Mucocele

(3) Malignant lymphomas and leukemias

*(4) Hemangioma

*(5) Endocrine ophthalmopathy (thyroid-related ophthalmopathy)

(6) Lacrimal gland tumors

(7) Rhinogenic carcinoma

(8) Malignant melanoma

(9) Osteosarcoma

(10) Fibrosarcoma

(11) Metastatic carcinoma

(12) Meningioma

(13) Dermoid

H. Fifty to seventy years-most common

*(1) Pseudotumor

*(2) Mucocele

*(3) Malignant lymphomas and leukemias

(4) Dermoid

(5) Carcinoma of palpebral or epibulbar origin

*(6) Meningioma

*(7) Endocrine ophthalmopathy (thyroid-related ophthalmopathy)

(8) Lacrimal gland tumor

(9) Osteosarcoma

(10) Fibrosarcoma

(11) Undifferentiated sarcoma

(12) Metastatic carcinoma

(13) Osteoma

(14) Fibrous dysplasia (Albright syndrome)

(15) Neurofibroma

(16) Hemangioma

I. More than seventy years-most common

(1) Melanoma

(2) Pseudotumor

*(3) Lymphoma

*(4) Metastatic tumor

(5) Basal cell carcinoma

(6) Mucocele

2. Unilateral exophthalmos-most common

A. Anatomical conditions

(1) Unilateral myopia of high degree

(2) Defects in the vault of the orbit: meningocele, encephalocele, hydroencephalocele

(3) Exophthalmos associated with arterial hypertension

(4) Recurrent exophthalmos from retrobulbar hemorrhage, lymphangioma

(5) Intermittent exophthalmos associated with venous anomalies within the cranium

(6) Disease of the pituitary gland; meningiomas involving sphenoid ridge

*(7) Unilateral exophthalmos associated with endocrine or thyroid-related ophthalmopathy

B. Traumatic conditions

(1) Contralateral floor fracture with enophthalmos

(2) Fracture of the orbit with retrobulbar hemorrhage

(3) Laceration and rupture of the tissues of the orbit and the extraocular muscles

(4) Intracranial trauma sustained at birth; aneurysm in orbit

(5) Pulsating exophthalmos from carotid-cavernous aneurysm

(6) Spontaneous retrobulbar hemorrhage as seen in whooping cough

(7) Chronic subdural hematoma bulging into orbit

(8) Posterior exophthalmos (orbital apex lesion)

a. Pseudotumor

b. Malignant tumor

c. Benign tumor

d. Vascular disease

e. Infection

C. Inflammatory conditions

(1) Retrobulbar abscess and cellulitis

(2) Thrombophlebitis of the orbital veins

(3) Cavernous sinus thrombosis

(4) Erysipelas (St. Anthony fire)

(5) Tenonitis

(6) Periostitis (syphilitic or tuberculous)

(7) Orbital mucocele, pyocele; cholesteatoma

(8) Orbital exostosis

(9) Paget disease with hyperostosis

(10) Actinomycosis, trichinosis, mycotic pseudotumor

(11) Herpes (HSV-l) with acute retinal necrosis

D. Disease of blood, lymph, and hematopoietic system

(1) Rickets (avitaminosis D)

(2) Scurvy (avitaminosis C)

(3) Hemophilia (factor VIII deficiency)

(4) Lymphosarcoma

(5) Chloroma

(6) Hodgkin disease

E. Space-taking lesions

(1) Vascular anomalies

a. Congenital orbital varix (young patient with systemic abnormalities)

b. Cavernous hemangioma (middle age)

c. Capillary hemangioma (young children) Kasabach-Merrit syndrome

d. Lymphangiomas

(2) 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)

(3) Intracranial tumor with orbital extension including chordoma and meningioma

F. Unilateral exophthalmos in children

(1) Inflammation

(2) Vascular disorders

(3) Neoplasms

(4) Metabolic diseases

(5) Developmental anomalies

(6) Others

*(7) Orbital cellulitis

3. Bilateral exophthalmos-most common

*A. Thyroid or endocrine ophthalmopathy

B. Orbital myositis (owing to causes other than thyroid dysfunction)

C. Cavernous sinus thrombosis (Foix syndrome)

D. Metastatic neuroblastoma

E. Hand-Schüller-Christian disease (histiocytosis X)

F. Crouzon disease (craniofacial dysostosis)

G. Paget disease (osteitis deformans)

4. Type proptosis-most common

A. Straightforward-glioma of optic nerve, intraconal cavernous hemangioma

B. Down and temporal-mucocele of frontal sinus

C. Down and nasal-lacrimal gland lesion

D. Downward-tumor of roof of orbit

E. Upward-tumor of floor of orbit

5. Transient exophthalmos

*A. Orbital varices

B. Orbital varices with intracranial extension

C. Arteriovenous malformations

D. Cavernous hemangioma

E. Intraorbital arteriovenous malformation

6. Pulsating exophthalmos-most common

*A. Carotid-cavernous fistula

B. von Recklinghausen disease associated with bony defect of skull

C. Large frontal mucocele

D. Meningoencephalocele

E. Blow-in fracture of roof of orbit

F. Neurofibromatosis

G. Fistula

H. Malignancies

I. Mucoceles

J. Orbital varix

K. Dermoid cysts

L. Aneurysm

7. Recurrent exophthalmos

A. Recurrent orbital inflammation (pseudotumor) or hemorrhage

B. Orbital cysts that rupture

C. Lymphangioma (children)

D. Syndrome of intermittent exophthalmos-congenital venous malformations of the orbit: venous angioma and orbital varix

E. Temporal lobe tumor with orbital extension

F. Neurofibromatosis

G. Vascular neoplasm

8. Intermittent exophthalmos

A. Orbital varices

B. Recurrent hemorrhage

C. Vascular neoplasm

D. Lymphangioma

9. Exophthalmos associated with conjunctival chemosis, restricted movement of eyes because of pain-pseudotumor

10. Exophthalmos in an acutely ill patient-cavernous sinus thrombosis

11. Exophthalmos associated with engorged conjunctival episcleral vessels

A. Nonpulsating-cerebral arteriovenous angioma, ophthalmic vein thrombosis, or cavernous sinus thrombosis

B. Pulsating exophthalmos-carotid-cavernous sinus fistula

12. Exophthalmos associated with a palpable mass in region of the lacrimal gland

A. Primary inflammatory exophthalmos

B. Neoplasm

C. Sarcoidosis syndrome (Schaumann syndrome)

D. Hodgkin disease

13. Exophthalmos 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

14. Exophthalmos in an infant with ecchymosis of the eyelids

A. Metastatic neuroblastoma

B. Orbital leukemia infiltration

15. Bilateral exophthalmos from bilateral orbital pseudotumor

A. Eosinophilic granuloma

B. Retroperitoneal fibrosis

C. Myasthenia gravis (Erb-Goldflam syndrome)
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Klapper SR, et al. Orbital involvement in allergic fungal sinusitis. Ophthalmology 1997; 104:2094-2100.
Krohel GB, et al. Orbital disease: a practical approach. Stewart WB, Chavis RM, eds. New York: Grune & Stratton, 1981.
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