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Proptosis Mounir Bashour, M.D., C.M. Jump to first page 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 Jump to first page How is proptosis diagnosed? Globes from above Measured with an exophthalmometer lateral orbital rim CT scan Jump to first page 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 Jump to first page Most common cause of unilateral proptosis? Thyroid eye disease (Graves' ophthalmopathy) Jump to first page Most common cause of bilateral proptosis? Thyroid eye disease Jump to first page 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 Jump to first page List the causes of pseudoproptosis 1. Unilateral high axial myopia A-Scan 2. Actual enophthalmos of other eye 3. Upper lid retraction Jump to first page 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 Jump to first page 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 Jump to first page 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 Jump to first page 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 Jump to first page 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 Jump to first page 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 Jump to first page 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% Jump to first page 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 Jump to first page 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 Jump to first page Tumors that are encapsulated or appear well circumscribed on neuroimaging Cavernous hemangioma Schwannoma Fibrohistiocytoma Neurofibroma Hemangiopericytoma Jump to first page