Exophthalmos (Proptosis) Clinical Presentation

Updated: Sep 25, 2018
  • Author: Michael Mercandetti, MD, MBA, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

A meticulous history of the patient's ocular and systemic systems is key in establishing a diagnosis.

The ophthalmic history should address the duration and the rate of onset of the proptosis. The patient should be queried about pain, change in visual acuity or refraction, diplopia, and decreased fields of vision. Transient visual loss or blackout periods may signify optic nerve compromise and may call for rapid intervention.

Complaints of foreign body sensation or dry gritty eyes are symptoms that may indicate corneal decompensation.

In performing a thorough medical history and a review of systems, the ophthalmologist should consider orbital involvement secondary to systemic pathology.

Past trauma and family history also may aid in the diagnosis.

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Physical

Evaluation of the patient with exophthalmos begins with a thorough ophthalmic and medical history. When concomitant sinus disease or an intranasal source is suspected, a speculum or endoscopic intranasal examination is warranted. Special emphasis on the duration and rate of progression of the patient's signs and symptoms is essential. Pain, diplopia, pulsation, change in effect or size with position or Valsalva maneuver, and disturbance of visual acuity are symptoms that should be explored. In general, a difference of more than 2 mm between a person's 2 eyes is abnormal.

A complete ophthalmic examination is paramount. Periorbital changes can be noted easily on gross examination in a well-illuminated examination room. Hypertelorism, exorbitism, eyeball protrusion (proptosis), eyelid lesions or edema, chemosis, and engorged conjunctival vessels are several periorbital signs. Blepharoptosis, lagophthalmos (incomplete lid closure), and interpalpebral fissure distance are additional signs to be considered during the examination.

Palpation of the anterior orbit can assess the level of tenderness, texture, and mobility of the mass. Tenderness may denote an inflammatory process or neural invasion by a neoplasm. Attention should be paid to regional lymph nodes. Tactile inspection of the globe may reveal pulsations secondary to arteriovenous communications or physiological intracranially pulsations transmitted through a bony defect of the orbit, such as an encephalocele.

Protrusion of the eye is an important clinical manifestation of orbital disease. In addition to proptosis, one should note the displacement of the eye in planes other than the anteroposterior dimension (eg, downward, lateral). Hertel exophthalmometry is a well-accepted tool to quantitate proptosis. The base is determined by the interlateral canthal space. The transection of the central cornea by the premarked millimeter ruler records the amount of anterior displacement of the globe. Its use requires intact lateral orbital rims. If the rim is not intact, a Luedde exophthalmometer. Relative protrusion can be observed by simply standing behind a seated patient and gazing downward toward the chin from the forehead to assess the displacement of one globe as compared to the contralateral side.

Auscultation of the orbit may detect a high flow state in the orbit or intracranially. The bell is useful for this examination. If a high-flow lesion is suspected (eg, carotid cavernous fistula), arteriography should be sought to further qualify these lesions. It is important to have the contralateral eye remain fixated on a target while auscultating the orbit.

Decreased visual acuity, change of refraction, and pupillary abnormalities should be noted.

Extraocular motility dysfunction and diplopia should be carefully assessed and documented.

Forced duction testing may qualify the dysfunction as restrictive or neurogenic in nature. Intraocular pressure may be elevated, and slit lamp examination can discern chemosis and engorged or sentinel vessels.

Dilated funduscopic examination may reveal optic disc edema or pallor, retinal detachment, choroidal folds, vascular engorgement or shunt vessels, or indentation of the posterior pole.

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Causes

Proptosis can be the result of a myriad of disease processes resulting from primary orbital pathology or systemic disease processes. The list below is not comprehensive but can help in forming a differential diagnosis. The list only consists of adult causes since a fair amount of overlap exists in the differential diagnosis of exophthalmos in adults and children.

  • Proptosis in adults

    • Infectious

      • Orbital cellulitis

      • Mucormycosis

      • Concurrent sinus disease

    • Inflammatory

      • Orbital inflammatory syndrome (orbital pseudotumor, benign orbital inflammation)

      • Thyroidopathy

      • Erdheim-Chester disease

    • Endocrine

      • Cushing syndrome

    • Vasculitis

      • Wegener granulomatosis

      • Churg-Strauss syndrome

    • Neoplastic

      • Lacrimal

      • Lymphoma

      • Leukemia

      • Meningioma

      • Glioma

      • Ossifying fibroma [33]

      • Metastatic (breast in women, lung and prostate in men, gastrointestinal, kidney)

      • Dermoid cyst

      • Hand-Schüller-Christian disease

    • Orbital vascular disease

      • Orbital varix (venous malformation)

      • Orbital arteriovenous malformation (carotid-cavernous sinus fistula, arteriovenous malformation)

    • Trauma

      • Traumatic or iatrogenic orbital hemorrhage

      • Orbital fractures

      • Facial fractures

    • Other

      • Craniosynostosis

      • Pfeiffer syndrome

      • Apert syndrome

      • Crouzon syndrome

      • Carpenter syndrome

    • Pseudoproptosis (pseudoexophthalmos)

      • Buphthalmos

      • Contralateral enophthalmos

      • Ipsilateral lid retraction

      • Axial myopia

      • Contralateral blepharoptosis

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