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Symptoms ⬇

Eyelid swelling, bulging eye(s), and double vision are common. Pain, decreased visual acuity, decreased color vision, and changes in facial sensation can occur.

Signs ⬆ ⬇

Critical

Globe dystopia (e.g., proptosis/exophthalmos, hypoglobus, and hyperglobus) and restriction of ocular motility, which can be confirmed by forced duction testing (see Appendix 6, FORCED DUCTION TEST AND FORCE GENERATION TEST). Resistance to retropulsion of the globe is common.

Differential Diagnosis ⬆ ⬇

Differential Diagnosis of Proptosis

Etiology ⬆ ⬇

Specific signs of orbital disease are rarely diagnostic. Orbital disease can be grouped into six broad categories to help tailor the necessary workup:

  1. Inflammatory: Thyroid eye disease (TED), idiopathic orbital inflammatory syndrome (IOIS), sarcoidosis, granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), reactive inflammation from paranasal sinusitis, IgG4-related disease, etc.
  2. Infectious: Orbital cellulitis, subperiosteal abscess (SPA), mucormycosis, etc.
  3. Neoplastic (discrete, infiltrative, or hematologic): Typically categorized as primary (e.g., solitary fibrous tumor), secondary (e.g., extension of sinus mucocele or intracranial meningioma, etc.), or metastatic. May be benign or malignant.
  4. Trauma: Orbital fracture, retrobulbar hemorrhage, orbital foreign body with or without secondary infection, carotid–cavernous fistula, etc.
  5. Malformation: Skeletal abnormalities, congenital/genetic syndromes, etc.
  6. Vascular: Usually either congenital or acquired and categorized as primary arterial (e.g., carotid–cavernous fistula) or venous (e.g., varix). Lymphangioma may also cause proptosis from intralesional hemorrhage.
NOTE:

As a general rule, any patient with a history of cancer and a new orbital process should be assumed to have metastatic disease unless proven otherwise.

Reference(s) ⬆ ⬇

Srinivasan A, Kleinberg T, Murchison AP, Bilyk JR. Serologic investigations in inflammatory orbital disease: part I. Ophthalmic Plast Reconstr Surg. 2016;32:321-328.Srinivasan A, Kleinberg T, Murchison AP, Bilyk JR. Serologic investigations in inflammatory orbital disease: part II. Ophthalmic Plast Reconstr Surg. 2017;33:1-8.

Work Up ⬆

Workup
  1. History: Rapid or slow onset? Pain? Ocular bruit and/or pulsation? Fever, chills, systemic symptoms, skin rash, weight change? History of malignancy, diabetes, pulmonary disease, or renal disease? Trauma? History of sinonasal congestion, epistaxis? Smoking? Up to date with health maintenance and age-appropriate screenings (e.g., mammography, prostate examination/screening)? Presence of systemic symptoms—weight loss/gain, fever, chills, night sweats, change in bowel habits, heat/cold intolerance?
  2. Examination:
    • Review vital signs, particularly temperature.
    • Check visual acuity, size and reactivity of pupils, visual fields, color vision, and intraocular pressure. Check for pulsatility of semicircles on Goldmann tonometry.
    • Check extraocular movements. Measure any ocular misalignment ([prisms or Maddox rod], see Appendix 3, COVER/UNCOVER AND ALTERNATE COVER TESTS and 10.7, ISOLATED FOURTH CRANIAL NERVE PALSY). Consider forced duction and force generation testing in select cases (see Appendix 6, FORCED DUCTION TEST AND FORCE GENERATION TEST).
    • Check for globe dystopia. Tilt the patient’s head back and look from below (“ant’s-eye view”). Measure with a Hertel exophthalmometer. Position the exophthalmometer against the lateral orbital rims, not the lateral canthi. The average value is 17 mm with the upper limit of normal about 22 to 24 mm. A difference between the two eyes of more than 2 mm is considered abnormal. Can be used in conjunction with a Valsalva maneuver if a venous malformation is suspected. In addition to classic axial exophthalmos, also look for nonaxial displacement of the globe (e.g., hypoglobus and hyperglobus).
    • Test resistance to retropulsion by gently pushing each globe into the orbit with your thumbs. Feel along the orbital rim for a mass. Check the conjunctival cul-de-sacs carefully and evert the upper eyelid.
    • Check trigeminal and facial nerve function. Check for preauricular and cervical adenopathy.
    • Perform a dilated examination to evaluate the optic nerves (pallor and swelling), posterior pole (especially for chorioretinal folds), and peripheral retina.
  3. Consider automated perimetry if compressive optic neuropathy is suspected.
  4. Imaging studies: Orbital computed tomography (CT, axial, coronal, and parasagittal views) or magnetic resonance imaging (MRI) with gadolinium and fat suppression, depending on suspected etiology. Orbital B-scan US with or without color Doppler imaging is useful if the diagnosis is uncertain or when a cystic or vascular lesion is suspected. Consider optical coherence tomography (OCT) to assess optic nerve contour. See Chapter 14, IMAGING MODALITIES IN OPHTHALMOLOGY.
  5. Laboratory tests when appropriate: Triiodothyronine (T3), thyroxine (T4), thyroid-stimulating hormone (TSH), antithyroid autoantibodies (thyroid-stimulating immunoglobulin [TSI] and antithyroid peroxidase antibody [TPO]), angiotensin-converting enzyme (ACE), cytoplasmic staining, and perinuclear staining antineutrophil cytoplasmic antibody (cANCA and pANCA), lactate dehydrogenase (LDH), IgG/IgG4 levels, antinuclear antibody (ANA), serum protein electrophoresis (SPEP), complete blood count (CBC) with differential, blood urea nitrogen (BUN)/creatinine (especially if CT contrast or gadolinium is indicated), fasting blood sugar/hemoglobin A1c, blood cultures, etc.
  6. Consider further systemic workup and additional imaging, depending on clinical suspicion and radiologic findings (e.g., metastasis, lymphoma, etc.).
  7. Consider an excisional or incisional biopsy, as dictated by the working diagnosis. Fine-needle aspiration biopsy has a limited role in orbital diagnosis.

Additional workup, treatment, and follow-up vary according to the suspected diagnosis. See individual sections.

This section provides a framework to evaluate a variety of orbital disorders.