Orbital Syndromes

ABSTRACT

The orbit is a complex anatomical structure with unique properties not observed in other regions of the body. Composed of seven bones, the orbit is filled by the eye, optic nerve, lacrimal gland, extraocular muscles, peripheral motor and sensory nerves, fat, arteries, and veins. All these structures are intimately related to one another within an intricate framework of connective tissue. A variety of traumatic, vascular, inflammatory, infectious, and neoplastic processes can affect the orbit and its structures. Aside from the many primary orbital diseases, systemic disorders and pathological processes from neighboring structures (eye, ocular adnexa, oral cavity, paranasal sinuses, and intracranial cavity) can involve the orbit. A careful history and complete physical examination, with special attention to the orbit and ocular adnexa, are required to identify subtle orbital abnormalities that otherwise could be overlooked or mistakenly contributed to a nonorbital process. This article reviews the pertinent orbital anatomy, discusses the clinical evaluation and manifestations of orbital syndromes, and highlights several important orbital syndromes germane to the neurologist, including thyroid-associated orbitopathy, nonspecific orbital inflammation (also known as inflammatory orbital pseudotumor), perineural orbital invasion of cutaneous squamous cell carcinoma, rhino-orbital-cerebral mucormycosis, and carotid-cavernous sinus fistula.

KEYWORDS

orbit - proptosis - thyroid-associated orbitopathy - Graves' disease - perineural tumor spread - cutaneous squamous cell carcinoma - mucormycosis - carotid-cavernous sinus fistula

REFERENCES

• 1 Wilson M W, Grossniklaus H E. Orbital disease in North America.  Ophthalmol Clin North Am. 1996;  9 539-547
• 2 Rootman J. Diseases of the Orbit: A Multidisciplinary Approach. Philadelphia; Lippincott Williams & Wilkins 2003
• 3 Doxanas M T, Anderson R L. Clinical Orbital Anatomy. Baltimore; Williams & Wilkins 1984
• 4 Rhoton A L, Natori Y. The Orbit and Sellar Region: Microsurgical Anatomy and Operative Approaches. New York; Thieme Medical Publishers 1996
• 5 Rootman J, Stewart B, Goldberg R A. Orbital Surgery: A Conceptual Approach. Philadelphia; Lippincott-Raven Publishers 1995
• 6 Hayek G, Mercier P, Fournier H D. Anatomy of the orbit and its surgical approach.  Adv Tech Stand Neurosurg. 2006;  31 35-71
• 7 Cheung N, McNab A A. Venous anatomy of the orbit.  Invest Ophthalmol Vis Sci. 2003;  44 988-995
• 8 Otto C S, Coppit G L, Mazzoli R A et al.. Gaze-evoked amaurosis: a report of five cases.  Ophthalmology. 2003;  110 322-326
• 9 Cline R A, Rootman J. Enophthalmos: a clinical review.  Ophthalmology. 1984;  91 229-237
• 10 Chang B Y, Cunniffe G, Hutchinson C. Enophthalmos associated with primary breast carcinoma.  Orbit. 2002;  21 307-310
• 11 Kupersmith M J. Neuro-Vascular Neuro-Ophthalmology. Berlin; Springer-Verlag 1993
• 12 Coday M P, Netland P A, Dallow R L. Thyroid-associated ophthalmolopathy (Graves' disease). In: Albert DM, Jakobiec FA Principles and Practice of Ophthalmology. Vol. 5. Philadelphia; WB Saunders Company 2000: 4742-4759
• 13 Chang E L, Rubin P A. Upper and lower eyelid retraction.  Int Ophthalmol Clin. 2002;  42 45-59
• 14 Gaymard B, Huynh C, Laffont I. Unilateral eyelid retraction.  J Neurol Neurosurg Psychiatry. 2000;  68 390-392
• 15 Yeh S, Foroozan R. Orbital apex syndrome.  Curr Opin Ophthalmol. 2004;  15 490-498
• 16 Ettl A, Salomonowitz E, Koornneef L, Zonneveld F W. High-resolution MR imaging anatomy of the orbit: correlation with comparative cryosectional anatomy.  Radiol Clin North Am. 1998;  36 1021-1045 , ix
• 17 Aviv R I, Miszkiel K. Orbital imaging: part 2. Intraorbital pathology.  Clin Radiol. 2005;  60 288-307
• 18 Herrick R C, Hayman L A, Taber K H, Diaz-Marchan P J, Kuo M D. Artifacts and pitfalls in MR imaging of the orbit: a clinical review.  Radiographics. 1997;  17 707-724
• 19 Belden C J, Zinreich S J. Orbital imaging techniques.  Semin Ultrasound CT MR. 1997;  18 413-422
• 20 Simon J, Szumowski J, Totterman S et al.. Fat-suppression MR imaging of the orbit.  AJNR Am J Neuroradiol. 1988;  9 961-968
• 21 Barakos J A, Dillon W P, Chew W M. Orbit, skull base, and pharynx: contrast-enhanced fat suppression MR imaging.  Radiology. 1991;  179 191-198
• 22 Lieb W E. Color Doppler imaging of the eye and orbit.  Radiol Clin North Am. 1998;  36 1059-1071
• 23 Chen C C, Chang P C, Shy C G, Chen W S, Hung H C. CT angiography and MR angiography in the evaluation of carotid cavernous sinus fistula prior to embolization: a comparison of techniques.  AJNR Am J Neuroradiol. 2005;  26 2349-2356
• 24 Bahn R S, Heufelder A E. Pathogenesis of Graves' ophthalmopathy.  N Engl J Med. 1993;  329 1468-1475
• 25 Gorman C A. Temporal relationship between onset of Graves' ophthalmopathy and diagnosis of thyrotoxicosis.  Mayo Clin Proc. 1983;  58 515-519
• 26 Bartalena L, Pinchera A, Marcocci C. Management of Graves' ophthalmopathy: reality and perspectives.  Endocr Rev. 2000;  21 168-199
• 27 Kazim M, Goldberg R A, Smith T J. Insights into the pathogenesis of thyroid-associated orbitopathy: evolving rationale for therapy.  Arch Ophthalmol. 2002;  120 380-386
• 28 Garrity J A, Bahn R S. Pathogenesis of graves ophthalmopathy: implications for prediction, prevention, and treatment.  Am J Ophthalmol. 2006;  142 147-153
• 29 Volpe N J, Sbarbaro J A, Gendron Livingston K, Galetta S L, Liu G T, Balcer L J. Occult thyroid eye disease in patients with unexplained ocular misalignment identified by standardized orbital echography.  Am J Ophthalmol. 2006;  142 75-81
• 30 Bartley G B, Fatourechi V, Kadrmas E F et al.. Clinical features of Graves' ophthalmopathy in an incidence cohort.  Am J Ophthalmol. 1996;  121 284-290
• 31 Peyster R G, Ginsberg F, Silber J H, Adler L P. Exophthalmos caused by excessive fat: CT volumetric analysis and differential diagnosis.  AJR Am J Roentgenol. 1986;  146 459-464
• 32 Dallow R L, Netland P A. Management of thyroid-associated orbitopathy (Graves' disease). In: Albert DM, Jakobiec FA Principles and Practice of Ophthalmology. Vol. 4. Philadelphia; WB Saunders Company 2000: 3082-3099
• 33 Mombaerts I, Goldschmeding R, Schlingemann R O, Koornneef L. What is orbital pseudotumor?.  Surv Ophthalmol. 1996;  41 66-78
• 34 Snebold N G. Noninfectious orbital inflammations and vasculitis. In: Albert DM, Jakobiec FA Principles and Practice of Ophthalmology. Vol. 4. Philadelphia; WB Saunders Company 2000: 3100-3121
• 35 Weber A L, Romo L V, Sabates N R. Pseudotumor of the orbit: clinical, pathologic, and radiologic evaluation.  Radiol Clin North Am. 1999;  37 151-168
• 36 Mahr M A, Salomao D R, Garrity J A. Inflammatory orbital pseudotumor with extension beyond the orbit.  Am J Ophthalmol. 2004;  138 396-400
• 37 Mombaerts I, Schlingemann R O, Goldschmeding R, Koornneef L. Are systemic corticosteroids useful in the management of orbital pseudotumors?.  Ophthalmology. 1996;  103 521-528
• 38 Kline L B, Hoyt W F. The Tolosa-Hunt syndrome.  J Neurol Neurosurg Psychiatry. 2001;  71 577-582
• 39 Yuen S J, Rubin P A. Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome.  Arch Ophthalmol. 2003;  121 491-499
• 40 Rootman J, Nugent R. The classification and management of acute orbital pseudotumors.  Ophthalmology. 1982;  89 1040-1048
• 41 Weissler M C, Miller E, Fortune M A. Sclerosing orbital pseudotumor: a unique clinicopathologic entity.  Ann Otol Rhinol Laryngol. 1989;  98 496-501
• 42 Bernardino C R, Davidson R S, Maus M, Spaeth G L. Angle-closure glaucoma in association with orbital pseudotumor.  Ophthalmology. 2001;  108 1603-1606
• 43 Purvin V, Kawasaki A, Jacobson D M. Optic perineuritis: clinical and radiographic features.  Arch Ophthalmol. 2001;  119 1299-1306
• 44 McKinnon S G, Gentry L R. Systemic diseases involving the orbit.  Semin Ultrasound CT MR. 1998;  19 292-308
• 45 Weber A L, Jakobiec F A, Sabates N R. Lymphoproliferative disease of the orbit.  Neuroimaging Clin N Am. 1996;  6 93-111
• 46 Gordon L K. Diagnostic dilemmas in orbital inflammatory disease.  Ocul Immunol Inflamm. 2003;  11 3-15
• 47 Jabs D A, Rosenbaum J T, Foster C S et al.. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel.  Am J Ophthalmol. 2000;  130 492-513
• 48 Smith J R, Rosenbaum J T. A role for methotrexate in the management of non-infectious orbital inflammatory disease.  Br J Ophthalmol. 2001;  85 1220-1224
• 49 Garrity J A, Coleman A W, Matteson E L, Eggenberger E R, Waitzman D M. Treatment of recalcitrant idiopathic orbital inflammation (chronic orbital myositis) with infliximab.  Am J Ophthalmol. 2004;  138 925-930
• 50 Johnson T M, Rowe D E, Nelson B R, Swanson N A. Squamous cell carcinoma of the skin (excluding lip and oral mucosa).  J Am Acad Dermatol. 1992;  26 467-484
• 51 Mohs F E, Lathrop T G. Modes of spread of cancer of skin.  AMA Arch Derm Syphilol. 1952;  66 427-439
• 52 Cruveilhier J. Maladies des Nerfs: Anatomie Pahologique du Corps Humain. Vol. 2. Paris; Bailliere 1842: 1835-1842
• 53 Dodd G D, Dolan P A, Ballantyne A J, Ibanez M L, Chau P. The dissemination of tumors of the head and neck via the cranial nerves.  Radiol Clin North Am. 1970;  8 445-461
• 54 Veness M J, Biankin S. Perineural spread leading to orbital invasion from skin cancer.  Australas Radiol. 2000;  44 296-302
• 55 Smith J B, Bishop V L, Francis I C, Kos S, Kneale K A. Ophthalmic manifestations of perineural spread of facial skin malignancy.  Aust NZ J Ophthalmol. 1990;  18 197-205
• 56 Clouston P D, Sharpe D M, Corbett A J, Kos S, Kennedy P J. Perineural spread of cutaneous head and neck cancer: its orbital and central neurologic complications.  Arch Neurol. 1990;  47 73-77
• 57 Caldemeyer K S, Mathews V P, Righi P D, Smith R R. Imaging features and clinical significance of perineural spread or extension of head and neck tumors.  Radiographics. 1998;  18 97-110
• 58 Rowe D E, Carroll R J, Day Jr C L. Prognostic factors for local recurrence, metastasis, and survival rates in squamous cell carcinoma of the skin, ear, and lip: implications for treatment modality selection.  J Am Acad Dermatol. 1992;  26 976-990
• 59 Alam M, Ratner D. Cutaneous squamous-cell carcinoma.  N Engl J Med. 2001;  344 975-983
• 60 Barksdale S K, O'Connor N, Barnhill R. Prognostic factors for cutaneous squamous cell and basal cell carcinoma: determinants of risk of recurrence, metastasis, and development of subsequent skin cancers.  Surg Oncol Clin N Am. 1997;  6 625-638
• 61 McNab A A, Francis I C, Benger R, Crompton J L. Perineural spread of cutaneous squamous cell carcinoma via the orbit: clinical features and outcome in 21 cases.  Ophthalmology. 1997;  104 1457-1462
• 62 Bowyer J D, Sullivan T J, Whitehead K J, Kelly L E, Allison R W. The management of perineural spread of squamous cell carcinoma to the ocular adnexae.  Ophthal Plast Reconstr Surg. 2003;  19 275-281
• 63 Lawrence N, Cottel W I. Squamous cell carcinoma of skin with perineural invasion.  J Am Acad Dermatol. 1994;  31 30-33
• 64 Carter R L, Foster C S, Dinsdale E A et al.. Perineural spread by squamous cell carcinomas of the head and neck: a morphological study using antiaxonal and antimyelin monoclonal antibodies.  J Clin Pathol. 1983;  36 269-275
• 65 Ginsberg L E. Imaging of perineural tumor spread in head and neck cancer.  Semin Ultrasound CT MR. 1999;  20 175-186
• 66 Moore C E, Hoyt W F, North J B. Painful ophthalmoplegia following treated squamous carcinoma of the forehead: orbital apex involvement from centripetal spread via the supraorbital nerve.  Med J Aust. 1976;  1 657-659
• 67 Esmaeli B, Ginsberg L, Goepfert H, Deavers M. Squamous cell carcinoma with perineural invasion presenting as a Tolosa-Hunt-like syndrome: a potential pitfall in diagnosis.  Ophthal Plast Reconstr Surg. 2000;  16 450-452
• 68 Trobe J D, Hood C I, Parsons J T, Quisling R G. Intracranial spread of squamous carcinoma along the trigeminal nerve.  Arch Ophthalmol. 1982;  100 608-611
• 69 Williams L S, Mancuso A A, Mendenhall W M. Perineural spread of cutaneous squamous and basal cell carcinoma: CT and MR detection and its impact on patient management and prognosis.  Int J Radiat Oncol Biol Phys. 2001;  49 1061-1069
• 70 Williams L S. Advanced concepts in the imaging of perineural spread of tumor to the trigeminal nerve.  Top Magn Reson Imaging. 1999;  10 376-383
• 71 Esmaeli B, Ahmadi M A, Gillenwater A M, Faustina M M, Amato M. The role of supraorbital nerve biopsy in cutaneous malignancies of the periocular region.  Ophthal Plast Reconstr Surg. 2003;  19 282-286
• 72 Spellberg B, Edwards Jr J, Ibrahim A. Novel perspectives on mucormycosis: pathophysiology, presentation, and management.  Clin Microbiol Rev. 2005;  18 556-569
• 73 Ribes J A, Vanover-Sams C L, Baker D J. Zygomycetes in human disease.  Clin Microbiol Rev. 2000;  13 236-301
• 74 Yohai R A, Bullock J D, Aziz A A, Markert R J. Survival factors in rhino-orbital-cerebral mucormycosis.  Surv Ophthalmol. 1994;  39 3-22
• 75 Harril W C, Stewart M G, Lee A G, Cernoch P. Chronic rhinocerebral mucormycosis.  Laryngoscope. 1996;  106 1292-1297
• 76 Dhiwakar M, Thakar A, Bahadur S. Improving outcomes in rhinocerebral mucormycosis: early diagnostic pointers and prognostic factors.  J Laryngol Otol. 2003;  117 861-865
• 77 Guevara N, Roy D, Dutruc-Rosset C, Santini J, Hofman P, Castillo L. Mucormycosis: early diagnosis and treatment.  Rev Laryngol Otol Rhinol (Bord). 2004;  125 127-131
• 78 Ferry A P, Abedi S. Diagnosis and management of rhino-orbitocerebral mucormycosis (phycomycosis): a report of 16 personally observed cases.  Ophthalmology. 1983;  90 1096-1104
• 79 Safar A, Marsan J, Marglani O, Al-Sebeih K, Al-Harbi J, Valvoda M. Early identification of rhinocerebral mucormycosis.  J Otolaryngol. 2005;  34 166-171
• 80 Bhansali A, Bhadada S, Sharma A et al.. Presentation and outcome of rhino-orbital-cerebral mucormycosis in patients with diabetes.  Postgrad Med J. 2004;  80 670-674
• 81 Nussbaum E S, Hall W A. Rhinocerebral mucormycosis: changing patterns of disease.  Surg Neurol. 1994;  41 152-156
• 82 Bodenstein N P, McIntosh W A, Vlantis A C, Urquhart A C. Clinical signs of orbital ischemia in rhino-orbitocerebral mucormycosis.  Laryngoscope. 1993;  103 1357-1361
• 83 Peterson K L, Wang M, Canalis R F, Abemayor E. Rhinocerebral mucormycosis: evolution of the disease and treatment options.  Laryngoscope. 1997;  107 855-862
• 84 Hargrove R N, Wesley R E, Klippenstein K A, Fleming J C, Haik B G. Indications for orbital exenteration in mucormycosis.  Ophthal Plast Reconstr Surg. 2006;  22 286-291
• 85 Barrow D L, Spector R H, Braun I F, Landman J A, Tindall S C, Tindall G T. Classification and treatment of spontaneous carotid-cavernous sinus fistulas.  J Neurosurg. 1985;  62 248-256
• 86 Miller N R. Carotid-cavernous sinus fistulas. In: Miller NR, Newman NJ, Biousse V, Kerrison JB Walsh and Hoyt's Clinical Neuro-Ophthalmology. Vol. 2. Baltimore; Williams & Wilkins 2005: 2263-2296
• 87 Bhatti M T, Peters K R. A red eye and then a really red eye.  Surv Ophthalmol. 2003;  48 224-229
• 88 Keltner J L, Satterfield D, Dublin A B, Lee B C. Dural and carotid cavernous sinus fistulas: diagnosis, management, and complications.  Ophthalmology. 1987;  94 1585-1600
• 89 Sergott R C, Grossman R I, Savino P J, Bosley T M, Schatz N J. The syndrome of paradoxical worsening of dural-cavernous sinus arteriovenous malformations.  Ophthalmology. 1987;  94 205-212
• 90 Barnwell S L, O'Neill O R. Endovascular therapy of carotid cavernous fistulas.  Neurosurg Clin N Am. 1994;  5 485-495