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ProptosisMounir Bashour, M.D., C.M.

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What is proptosis? Forward protrusion of one or both

eyeballs Unilateral asymmetric protrusion of

one eye by at least 2 mm Normal upper limits

22 mm in Caucasians 24 mm in African-Americans

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How is proptosis diagnosed? Globes from above Measured with an exophthalmometer

lateral orbital rim CT scan

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List common problems associated with proptosis 1. Exposure keratopathy

poor blink mechanism corneal abrasions and ulcers

2. Diplopia displacement of the globes extraocular muscle function

3. Optic nerve compression decreased visual acuity RAPD color vision deficit visual field defect prompt therapeutic intervention

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Most common cause of unilateral proptosis? Thyroid eye disease (Graves'

ophthalmopathy)

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Most common cause of bilateral proptosis?

Thyroid eye disease

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What are other causes of proptosis? Orbital inflammatory pseudotumor Orbital infectious cellulitis Orbital tumors (benign or malignant) Lacrimal gland tumors Trauma (retrobulbar hemorrhage) Orbital vasculitis (i.e., polyartentts nodosa,

Wegener's granulomatosis) Mucormycosis Carotid-cavernous fistula Orbital varix

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List the causes of pseudoproptosis 1. Unilateral high axial myopia

A-Scan 2. Actual enophthalmos of other

eye 3. Upper lid retraction

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Which neuroimaging test is best to evaluate the etiology of proptosis? CT scans are superior in most cases MRI may be desirable in certain cases

when optic nerve dysfunction is present

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Unilateral or bilateral painless proptosis, eyelid retraction, eyelid lag, and motility disturbances? Thyroid ophthalmopathy

multisystem. autoimmune disorder hyperthyroid, hypothyroid, euthyroid

inflammation and enlargement EOM• IR>MR>SR>LR

• fusiform enlargement sparing the tendon peribulbar tissues.

Proptosis Eyelid retraction Corneal problems Diplopia Optic nerve compression Treatment depending on the severity Systemic and laboratory evaluation is mandatory

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Unilateral proptosis, pain, conjunctival injection, and motility disturbances in an adult? Orbital inflammatory pseudotumor

nonspecific idiopathic inflammatory localized to muscle, lacrimal gland, sclera vs. diffuse eyelid erythema or edema palpable mass decreased vision uveitis hyperopic shift optic nerve edema Bilateral disease more common in children CT scan

thickening 1+ EOM (inc. tendons) lacrimal gland enlargement thickening of the posterior sclera

Treatment corticosteroids +/- radiation

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Unilateral proptosis, pain, fever, decreased ocular motility, erythema, and edema of the eyelids? Infectious orbital cellulitis

usually bacterial extended posterior to orbital septum meningitis cavernous sinus thrombosis staphylococci. streptococci. anaerobes, and

Haemophilus influenza (in children under 5 years of age)

most common source -- ethmoid sinusitis intravenous antibiotics

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Persistent proptosis or progression of infection despite adequate antibiotic Rx Orbital subperiosteal abscess CT scan

confirm diagnosis locate the abscess

surgical drainage and continued intravenous antibiotics

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Child < 6 y.o. with gradual, painless, progressive, unilateral axial proptosis with visual loss?

Optic nerve glioma (juvenile pilocytic astrocytoma) slow-growing tumor Decreased visual acuity with a RAPD CT scan or MRI

“fusiform” enlargement of the ON associated with NF1 Dx if bilateral Systemic evaluation and genetic

counselling for NF is essential

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Child with rapidly progressive unilateral proptosis, displacement of the globe inferiorly, and edema of upper eyelid?

Rhabdomyosarcoma most common primary orbital malignancy of

childhood malignant growth of striated muscle tissue rapidly progressive mass in the superior orbit with

proptosis, globe displacement, and eyelid swelling average age of presentation is 7 years Prompt diagnosis with orbitotomy and biopsy is

crucial overall mortality is 60% once the disease has

extended to orbital bones Current Rx with radiation + chemo have lowered

mortality rates to 5 to 10%

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Most common benign orbital tumor in adults that causes unilateral proptosis? Cavernous hemangioma

slow-growing vascular tumor usually diagnosed in young adulthood

to middle age CT scan intraconal well-defined orbital mass Visual acuity is often not affected. Treatment observation or surgical

excision

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Most common malignant orbital tumor in adults that causes unilateral proptosis? Orbital lymphomas

typically superior orbit slow onset and progression subconjunctival “salmon-colored" mass in the fornix CT scan

poorly defined mass conforming to the shape of the orbital bones and globe without bony erosion

orbital biopsy definitive treatment is radiation associated with systemic lymphoma: therefore

medical consult and systemic evaluation are necessary for all patients

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Tumors that are encapsulated or appear well circumscribed on neuroimaging Cavernous hemangioma Schwannoma Fibrohistiocytoma Neurofibroma Hemangiopericytoma