2. Jump to first page Proptosis Mounir Bashour, M.D., C.M.

3. Jump to first page What is proptosis? n Forward protrusion of one or both eyeballs n Unilateral asymmetric protrusion of one eye by at least 2 mm n Normal upper limits u 22 mm in Caucasians u 24 mm in African-Americans

4. Jump to first page How is proptosis diagnosed? n Globes from above n Measured with an exophthalmometer u lateral orbital rim n CT scan

5. Jump to first page List common problems associated with proptosis n 1.Exposure keratopathy u poor blink mechanism u corneal abrasions and ulcers n 2.Diplopia u displacement of the globes u extraocular muscle function n 3.Optic nerve compression u decreased visual acuity u RAPD u color vision deficit u visual field defect u prompt therapeutic intervention

6. Jump to first page Most common cause of unilateral proptosis? n Thyroid eye disease (Graves' ophthalmopathy)

7. Jump to first page Most common cause of bilateral proptosis? n Thyroid eye disease

8. Jump to first page What are other causes of proptosis? n Orbital inflammatory pseudotumor n Orbital infectious cellulitis n Orbital tumors (benign or malignant) n Lacrimal gland tumors n Trauma (retrobulbar hemorrhage) n Orbital vasculitis (i.e., polyartentts nodosa, Wegener's granulomatosis) n Mucormycosis n Carotid-cavernous fistula n Orbital varix

9. Jump to first page List the causes of pseudoproptosis n 1.Unilateral high axial myopia u A-Scan n 2.Actual enophthalmos of other eye n 3.Upper lid retraction

10. Jump to first page Which neuroimaging test is best to evaluate the etiology of proptosis? n CT scans are superior in most cases n MRI may be desirable in certain cases when optic nerve dysfunction is present

11. Jump to first page Unilateral or bilateral painless proptosis, eyelid retraction, eyelid lag, and motility disturbances? n Thyroid ophthalmopathy u multisystem. autoimmune disorder u hyperthyroid, hypothyroid, euthyroid F inflammation and enlargement EOM IR>MR>SR>LR fusiform enlargement sparing the tendon F peribulbar tissues. u Proptosis u Eyelid retraction u Corneal problems u Diplopia u Optic nerve compression u Treatment depending on the severity u Systemic and laboratory evaluation is mandatory

12. Jump to first page Unilateral proptosis, pain, conjunctival injection, and motility disturbances in an adult? n Orbital inflammatory pseudotumor u nonspecific idiopathic inflammatory u localized to muscle, lacrimal gland, sclera vs. diffuse u eyelid erythema or edema u palpable mass u decreased vision u uveitis u hyperopic shift u optic nerve edema u Bilateral disease more common in children u CT scan F thickening 1+ EOM (inc. tendons) F lacrimal gland enlargement F thickening of the posterior sclera u Treatment corticosteroids +/- radiation

13. Jump to first page Unilateral proptosis, pain, fever, decreased ocular motility, erythema, and edema of the eyelids? n Infectious orbital cellulitis u usually bacterial u extended posterior to orbital septum u meningitis u cavernous sinus thrombosis u staphylococci. streptococci. anaerobes, and Haemophilus influenza (in children under 5 years of age) u most common source -- ethmoid sinusitis u intravenous antibiotics

14. Jump to first page Persistent proptosis or progression of infection despite adequate antibiotic Rx n Orbital subperiosteal abscess n CT scan u confirm diagnosis u locate the abscess n surgical drainage and continued intravenous antibiotics

15. Jump to first page Child < 6 y.o. with gradual, painless, progressive, unilateral axial proptosis with visual loss? n Optic nerve glioma (juvenile pilocytic astrocytoma) u slow-growing tumor u Decreased visual acuity with a RAPD u CT scan or MRI F “fusiform” enlargement of the ON u associated with NF1 Dx if bilateral u Systemic evaluation and genetic counselling for NF is essential

16. Jump to first page Child with rapidly progressive unilateral proptosis, displacement of the globe inferiorly, and edema of upper eyelid? n Rhabdomyosarcoma u most common primary orbital malignancy of childhood u malignant growth of striated muscle tissue u rapidly progressive mass in the superior orbit with proptosis, globe displacement, and eyelid swelling u average age of presentation is 7 years u Prompt diagnosis with orbitotomy and biopsy is crucial u overall mortality is 60% once the disease has extended to orbital bones u Current Rx with radiation + chemo have lowered mortality rates to 5 to 10%

17. Jump to first page Most common benign orbital tumor in adults that causes unilateral proptosis? n Cavernous hemangioma u slow-growing vascular tumor u usually diagnosed in young adulthood to middle age u CT scan u intraconal well-defined orbital mass u Visual acuity is often not affected. u Treatment observation or surgical excision

18. Jump to first page Most common malignant orbital tumor in adults that causes unilateral proptosis? n Orbital lymphomas u typically superior orbit u slow onset and progression u subconjunctival “salmon-colored" mass in the fornix u CT scan F poorly defined mass conforming to the shape of the orbital bones and globe without bony erosion u orbital biopsy u definitive treatment is radiation u associated with systemic lymphoma: therefore medical consult and systemic evaluation are necessary for all patients

19. Jump to first page Tumors that are encapsulated or appear well circumscribed on neuroimaging n Cavernous hemangioma n Schwannoma n Fibrohistiocytoma n Neurofibroma n Hemangiopericytoma