Proptosis/Exophthalmos

Proptosis/Exophthalmos: Excerpt from In a Page: Signs and Symptoms

Proptosis, or exophthalmos, may be caused by inflammatory, infectious, neoplastic, traumatic, or vascular etiologies. Thyroid-associated orbitopathy is by far the most common cause of proptosis. Some may have a benign course, but others follow a fulminant course that threatens sight and/or life, and requires urgent intervention. Imaging studies are essential in almost all cases for diagnosis and planning treatment.

Differential Diagnosis

• TAO
  –Major cause of unilateral and bilateral proptosis
  –Usually bilateral, although often asymmetric
  –Course is variable
  –Associated with Graves’ disease; more commonly occurs in women, smokers, and in patients treated with radioactive iodine
• Orbital cellulitis
  –Most cases occur due to contiguous spread from sinusitis
• Mucormycosis
  –Occurs primarily in diabetic and immunocompromised patients
  • Orbital tumors
    –Children: Dermoid, capillary hemangioma, rhabdomyosarcoma, lymphangioma, optic nerve glioma, leukemia (chloroma or granulocytic sarcoma), metastatic neuroblastoma, plexiform neurofibroma, teratoma
    –Adults: Metastatic breast, lung, or prostate cancer; cavernous hemangioma; mucocele; lymphoid tumors; optic nerve sheath meningioma; neurofibroma; neurilemoma (schwannoma); fibrous histiocytoma; hemangiopericytoma
  • Trauma (e.g., intraorbital foreign body, retrobulbar hemorrhage)
  • Orbital vasculitis (e.g., Wegener's granulomatosis, polyarteritis nodosa)
  • Arteriovenous malformation (e.g., carotid-cavernous fistula, retina or brain)
  • Cavernous sinus thrombosis
    –Orbital cellulitis signs plus cranial neuropathies (third, fourth, fifth, and/or sixth)
    –Mental status changes
    –Usually bilateral and rapidly progressive
  • Neurofibromatosis
  • Pseudoproptosis
    –Enlarged globe (myopia, buphthalmos)
    –Enophthalmos of the fellow eye

  Workup and Diagnosis

  • History and physical examination
    –History should include age, tempo of onset, pain, fever, laterality, diplopia, thyroid disease, sinusitis, or trauma; history of diabetes, immunosuppression, or cancer; and maneuvers or conditions that worsen proptosis
    –Physical exam should include ophthalmologic, head and neck, and focal neurologic examinations
    –Measure proptosis with exophthalmometer
  • Initial laboratory evaluation may include thyroid function tests (TSH, free T₄ and T₃, TSH receptor antibodies), ESR, CRP, CBC
  • Consider ANCA, ANA, and blood cultures
  • CT and/or MRI of orbits
  • Ultrasound (with color Doppler for suspected arteriovenous malformation)
  • Consider ophthalmology, neurosurgical, and/or endocrine consultation
  • Consider biopsy of selected solid tumors

  Treatment

  • Treat the underlying cause, although treatment of Graves’ disease does not always improve ophthalmopathy, and radioactive iodine may make it worse; systemic steroids for acute flareups only
  • Prevent eye injury and discomfort with artificial tears and sunglasses; may patch eye while sleeping
  • Surgical decompression (in TAO and retrobulbar hemorrhage with acute optic neuropathy by direct compression or by increased intraocular pressure)
  • If due to infectious causes, appropriate directed systemic intravenous antibiotic therapy and/or surgical debridement
  • If due to noninfectious inflammation, administer systemic steroids or immunomodulating therapy, particularly if there is acute optic neuropathy
  • Incisional or excisional biopsy of orbital tumors

Book Source Details

• Book Title: In a Page: Signs and Symptoms
• Author(s): Scott Kahan, Ellen G. Smith
• Year of Publication: 2004
• Copyright Details: In a Page: Signs and Symptoms, Copyright © 2004 Lippincott Williams & Wilkins.

More About Graves Disease

• Back to disease: Graves Disease: Introduction
• Next Book Extract About Graves Disease: Proptosis/Exophthalmos (In A Page: Pediatric Signs and Symptoms)
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: In a Page: Signs and Symptoms Authors: Scott Kahan, Ellen G. Smith Publisher: Lippincott Williams & Wilkins Copyright: 2004 ISBN: 1-4051-0368-X

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