1.PROPTOSISAdultsDr. Yousaf JamalFCPS Resident Ophthalmology UnitHayatabad Medical Complex 20-02-2010Mar 25, 2013

2. CONTENTS Introduction General Causes Clinical approach + Differentiating features Causes in Adults Common causes discussion Summary Take home message MCQs Page 2Proptosis in Adults Mar 25, 2013 3. IntroductionProptosis * Forward displacement or bulging, especially eyeballExophthalmos Abnormal protrusion of eyeball * Proptosis secondary to endocrinological dysfunction ** * Dorlands medical dictionary 26th edition ** Henderson JW.Orbital Tumors.3rded.New York:Raven Press;1994.Page 3 Proptosis in Adults Mar 25, 2013 4. General Causes Congenital Acquired Dermoid cyst Inflammatory Orbital teratoma Infectious Congenital cystic Traumaeyeball Vascular Cysts Neoplastic Page 4Proptosis in AdultsMar 25, 2013 5. Causes Inflammatory Thyroid-related orbitopathy Idiopathic orbital inflammatory disease (pseudotumor) Sarcoidosis Wegeners granulomatosis Systemic amyloidosis Histiocytosis Page 5 Proptosis in AdultsMar 25, 2013 6. Infectious Orbital cellulitis Subperiosteal abscess Gumma Mucormycosis Trauma Orbital blow-out fracture Retrobulbar hemorrhage Carotid-cavernous fistula Retained foreign body Page 6 Proptosis in Adults Mar 25, 2013 7. Vascular Orbital varices aneurysms Cysts Hydatid cyst Cysticercus Sinus related Mucocele Tumors Page 7Proptosis in Adults Mar 25, 2013 8. Neoplastic Capillary Optic nerve gliomahemangioma Optic nerve sheath Cavernous meningiomahemangioma Retinoblastoma Rhabdomyosarcoma osteoma Neurofibroma Pleomorphic lacrimalgland adenoma Leukemia,Lymphoma Lac. Gland metastasiscarcinoma Lymphangioma Page 8 Proptosis in AdultsMar 25, 2013 9. Clinical approach History Examination Investigation Page 9 Proptosis in Adults Mar 25, 2013 10. History Age of onset Nature of onset Sudden Gradual Duration Continuous Intermittent postural / valsalva Pulsatile Page 10Proptosis in Adults Mar 25, 2013 11. Associated symptoms Vision loss Pain Diplopia Past history Thyroid disorder Malignancy Lungs breasts Prostate Page 11 Proptosis in Adults Mar 25, 2013 12. Examination Local examination Inspection Palpation Auscultation Tests Systemic examination Page 12Proptosis in Adults Mar 25, 2013 13. Inspection True or pseudoproptosis ?? Laterality UnilateralDermoid cysts, orbital teratoma, congenital cystic eyeballOrbital hemorrhage, traumatic aneurysmCellulitis/abscess, cavernous sinus thrombosisTED, pseudotumorVarices, cysts, tumors Page 13 Proptosis in Adults Mar 25, 2013 14. Inspection Bilateral Cranifacial synostosis, osteitis deformans, rickets TED, Mickuliczs syndrome Histiocytosis, Amyloidosis, wegeners granulomatosis TumorsPage 14Proptosis in AdultsMar 25, 2013 15. Inspection Direction of displacement Axial Non-axial Lateral Inferonasal Superior Lid signs Page 15Proptosis in Adults Mar 25, 2013 16. Palpation Orbital rims Regional lymph nodes Page 16 Proptosis in Adults Mar 25, 2013 17. Auscultation Globe/temporal region for bruit Page 17Proptosis in Adults Mar 25, 2013 18. Other tests Transillumination Visual acuity Pupillary reactions Ocular motility Forced duction test Tonometry Fundoscopy Exophthalmometry Page 18Proptosis in Adults Mar 25, 2013 19. Systemic examination GPE For systemic disease Thyroid evaluation Primary neoplasm ?? Otolaryngological examination Page 19Proptosis in Adults Mar 25, 2013 20. Investigations LABORATORY INVESTIGATIONS FBC, TLC, DLC TFTs Casonis test Urine analysis Page 20Proptosis in Adults Mar 25, 2013 21. InvestigationsRADIOLOGY X-rays CT Orbital USG MRI Angiography Page 21Proptosis in Adults Mar 25, 2013 22. Causes in Adults Thyroid Exophthalmos Pseudotumor Orbital cellulitis Trauma Meningioma Lymphoma Histiocytoma Cavernous hemangioma Page 22Proptosis in Adults Mar 25, 2013 23. Osteoma Varices Carotid-cavernous fistula Tumors extending from adjacent areas Lacrimal gland Sinuses lids Metastasis Page 23 Proptosis in Adults Mar 25, 2013 24. Page 24 Proptosis in Adults Mar 25, 2013 25. TED PSEUDOTUMOR LYMPHOMAPathologyHypertrophy of EOM, Hypocellular lymphoid Hypercellular lesion, GAGs accumulation lesion, mature lymphs immature malignant B cellsDemographics 3rd-4th decade, 3rd-6th decade, adults &Old age commonest cause inchildren adultsAssociated Thyroid disease - lymph nodesPresentation F>M, painful, lid Painful, acute onset, Usually bilateral, retraction, lid lag, axial, axial, unilateral inpainless, nonaxial, uni/bilateral, attentiveadults, bilateral in child, rubbery palpable gaze, heat intolerance, congestion, mass, adnexal tachycardia, diminshedinvolvement, reflexesRadiologyFusiform appearance ofIll defined orbital Soft tissue mass, EOM opacification bone erosionTx Steroids, radiotherapy, NSAIDS, steroids, Raiotherapy, decompression radiotherapy, infliximabchemotherapy antimetabolites,Page 25Proptosis in AdultsMar 25, 2013 26. CAPILLARY ON SHEATH CCFHEMANGIOMAMENINGIOMAPathology Vascular hemartomaTubular enlargement of AbnormalON, origin-meninges, communication btwpsammoma bodiescarotid artery & cavernous sinusDemographics Infant, commonest benign Late middle aged traumaticmen,orbital tumor in childhood, womanSpontaneous..women,spontaneous involutionAssociatedVisceral, nasopharyngeal- HTN, atnerosclerosis,hemangioma, kasabach- trauma, pregnancy,merit syndromeconnecive tissuedisorderPresentationNonaxial, +ve Valsalva, Axial, gaze evokedPulsatile proptosis,superficial/deepamaurosis, EOMwhooshing sound,impaired, optociliary ptosis,shuntsunilateral/bilateral,chemosis, ant segmentischemiaRadiology Intra/extraconal mass, Tubular enlargment,Ophthslmic veins,poorly defined hyperdense, ON cavernous sinus sheath enhancement enlargementTx Observation, surgeryConservative,surgeryPage 26Proptosis radiotherapy, surgery in AdultsMar 25, 2013 27. THYROTOXICOSIS Autoimmune disorder 3rd 4th decade, F > M 25-30% pts present TED Females 5 times more affected Inflammation of EOM Inflammatory cellular infiltration Page 27 Proptosis in Adults Mar 25, 2013 28. Clinical manifestations Soft tissue involvement Lid retraction Proptosis Optic neuropathy Restrictive myopathy Page 28 Proptosis in Adults Mar 25, 2013 29. Proptosis in TED Axial Unilateral/bilateral Symmetrical/asymmetrical Complications Exposure keratopathy Corneal ulceration Infection Compressional optic neuropathy Page 29 Proptosis in Adults Mar 25, 2013 30. Page 30 Proptosis in Adults Mar 25, 2013 31. Work-up Complete Hx Known thyroid disease Smoking Visual changes Examination CT orbit Visual fields / Hess chart Thyroid function tests Page 31Proptosis in Adults Mar 25, 2013 32. Treatment of Proptosis General Lubricants Taping eyelids Cold compresses Page 32Proptosis in Adults Mar 25, 2013 33. Systemic steroids Oral prednisolone = 60-80mg/day Tapered over 2-8 weeks Intravenous methylprednisolone 0.5g/200ml isotonic saline infusion 30 min Page 33Proptosis in Adults Mar 25, 2013 34. Radiotherapy Alone or with steroids Combined therapy Radiations + Azathioprine + steroids Surgical decompression Page 34 Proptosis in Adults Mar 25, 2013 35. PSEUDOTUMOR Idiopathic orbital inflammatory disease Non-infectious, non-Neoplastic May involve any of soft orbital tissue Muscles Lacrimal gland Tenon capsule sclera Page 35Proptosis in Adults Mar 25, 2013 36. Presentation Unilateral adults, may be bilateral children 3rd 6th decade Acute Painful Periorbital swelling Redness/chemosis Page 36 Proptosis in Adults Mar 25, 2013 37. Congestive Proptosis Ophthalmoplegia Optic nerve dysfunction Palpable mass Page 37 Proptosis in Adults Mar 25, 2013 38. Page 38 Proptosis in Adults Mar 25, 2013 39. Work-up Hx Examination CT orbit Ring sign (contrast) Biopsy FNAC In persistent cases Page 39 Proptosis in Adults Mar 25, 2013 40. Course Spontaneous remission Intermittent episodes Prolonged inflammation Fibrosis Frozen orbit Ophthalmoplegia Ptosis Visual impairment Page 40 Proptosis in Adults Mar 25, 2013 41. Treatment Observation Oral NSAIDs Systemic steroids Oral prednisolone = 60-80mg/day Radiotherapy If no response to steroids Page 41Proptosis in Adults Mar 25, 2013 42. Anti metabolites If resistant to steroids and radiations Methotrexate Mycophenolate mofetil Systemic infliximab Page 42 Proptosis in AdultsMar 25, 2013 43. CAVERNOUS HEMANGIOMA Most common intraorbital tumor of adults 3rd to 5th decade Mostly unilateral Incidence = 4.3% among orbital neoplasms 1 No ethnic/racial predilection F:M = 7:32 but 8:71 also reported1.Henderson GW.Vascular hamartomas, hyperplasias, and neoplasms.In: Henderson GW, ed. Orbital Tumors.NewYork:Raven Press;1994:94-100.2.Harris GJ, Jakobiec FA.Cavernous hemangioma of the orbit: a clinicopathologic analysis of sixty-six cases.In: Jakobiec,ed.Ocular and Adnexal Tumors.Birmingham, Ala:1978:741-81. Page 43Proptosis in Adults Mar 25, 2013 44. Pathophysiology Benign, slowly growing vascular lesions Increase intraorbital volume to cause proptosis Morbidity Compressive optic neuropathy Extraocular muscle dysfunction Cosmetic disfigurement Death from bleeding during surgical removal Page 44Proptosis in AdultsMar 25, 2013 45. Presentation Middle aged Slowly growing unilateral axial proptosis Decrease in VA Diplopia Rarely gaze evoked amaurosis fugax May be accelerated by pregnancy Page 45Proptosis in Adults Mar 25, 2013 46. Signs Dilated & tortuous epibulbar vessels Other defects Color vision Visual field RAPD Fundus Optic disc edema & choroidal folds Page 46 Proptosis in AdultsMar 25, 2013 47. Work-up CT Well circumscribed, round or oval isointense mass Usually in lateral part of muscle coneUSG High echogenecity on A-scan...reflect septae within the lesionPage 47Proptosis in Adults Mar 25, 2013 48. Page 48 Proptosis in Adults Mar 25, 2013 49. Treatment Surgical En-bloc removal Approach Lateral orbitotomylateral lesions upper eyelid or a transcaruncular-based medialorbitotomymedial aspect lesions Page 49 Proptosis in AdultsMar 25, 2013 50. Page 50 Proptosis in Adults Mar 25, 2013 51. CAROTID-CAVERNOUS FISTULA An abnormal communication btw cavernousand external or internal carotid arteries Classification Etiology Traumatic vs. spontaneous Hemodynamics High vs. low velocity Anatomy Direct vs. indirect (dural) Page 51 Proptosis in Adults Mar 25, 2013 52. Page 52 Proptosis in Adults Mar 25, 2013 53. Direct carotid-cavernous fistula 70-90% of all cases Communication btw intracavernous part ofICA & cavernous sinus High arterial blood flow Causes Spontaneous25% Middle aged woman, atherosclerosis, HTN, pregnancy Collagen vascular disease, Connective tissue disorders Trauma (basal or facial skull fracture)75% Iatrogenic Page 53 Proptosis in AdultsMar 25, 2013 54. Indirect carotid-cavernous fistula Communication btw cavernous sinus &meningeal branches of ICA and/or ECA Low arterial blood flow Causes Spontaneous Congenital AV-malformations Page 54Proptosis in Adults Mar 25, 2013 55. Presentation Days or weeks after head injury Hx of surgery or systemic factors Triad Pulsatile proptosis Chemosis Whooshing noise in head Decreased vision Diplopia Page 55Proptosis in Adults Mar 25, 2013 56. Epibulbar injection Ptosis Pulsatile proptosis with bruit Increased IOP Anterior segment ischemiaCorneal edemaAqueous flareIris atrophyRubeosis iridisCataract Page 56Proptosis in Adults Mar 25, 2013 57. Page 57 Proptosis in Adults Mar 25, 2013 58. Page 58 Proptosis in Adults Mar 25, 2013 59. Page 59 Proptosis in Adults Mar 25, 2013 60. Exposure keratopathy Ophthalmoplegia Fundus changes Optic disc swelling Venous dilatation Intraretinal hemorrhages CRVO Ischemic optic neuropathy NVG Page 60 Proptosis in Adults Mar 25, 2013 61. Page 61 Proptosis in Adults Mar 25, 2013 62. Work-up CT, MRI & Orbital echo Enlargement of EOM Dilation of one or both superior ophthalmic veins Enlargement of the affected cavernous sinus Arterial Angiography Definite diagnosis Page 62Proptosis in Adults Mar 25, 2013 63. TreatmentMedical Exposure keratopathy Glaucoma NVG Page 63Proptosis in Adults Mar 25, 2013 64. Surgery Indication Post traumatic fistula Failure to spontaneous closure Procedures Surgical repair of the damaged portion of theintracavernous internal carotid artery Electrothrombosis, Embolization, or Balloonocclusion of the fistula Page 64Proptosis in AdultsMar 25, 2013 65. OPTIC NERVE SHEATH MENINGIOMA Arise from meningiothelial cells of arachnoidvilli, hence attached to dura mater Primary ONSM Intraorbital or intracanalicular portion or ON Less common Secondary ONSM Extension of intracranial meningioma into orbit More common Page 65 Proptosis in AdultsMar 25, 2013 66. Presentation Age6th-8th decade F>M Chronic, painless, progressive visualimpairment Usually accompanied by proptosis Mostly unilateral Headache Double visionPage 66 Proptosis in AdultsMar 25, 2013 67. Signs Classic triad visual loss optic atrophy optociliary shunt vessels (30%) Restrictive motility defect * Axial proptosisSibony PA, Krauss HR, Kennerdell JS, Maroon JC, Slamovits TL.Optic nerve sheath meningiomas. Clinicalmanifestations.Ophthalmology.Nov1984;91(11):1313-26 Page 67Proptosis in Adults Mar 25, 2013 68. Page 68 Proptosis in Adults Mar 25, 2013 69. Page 69 Proptosis in Adults Mar 25, 2013 70. Work-up CT scan (with contrast) Fusiform appearance of ON Mostly at orbital apex Calcifications usually seen Bony involvement MRI Clearly show small tumors Intraorbital extension of intracranial meningiomaeasily detected Page 70Proptosis in Adults Mar 25, 2013 71. Page 71 Proptosis in Adults Mar 25, 2013 72. Treatment Observation No intracranial extension and no vision lossMedical Radiotherapy Primary radiation Radiation after surgery Page 72Proptosis in Adults Mar 25, 2013 73. Chemotherarpy Unresectable, recurrent, or previouslyirradiated Combinations 5-fluroouracil, folate, and levamisole intra-arterial cisplatin with intravenous doxorubicin Adriamycin and dacarbazine ifosfamide and mesna Surgery Surgical excision Page 73 Proptosis in Adults Mar 25, 2013 74. Page 74 Proptosis in Adults Mar 25, 2013 75. PROPTOSIS Hx Middle-aged woman Rapid onset ProptosisUnilateral/bilateral Slow growingMotility problemsLid retraction/lag RetropulsionOrbital painHeat intolerance Axial Proptosis DiplopiaWoman20-40yrs Increased hyperopia Primary tumorThyroid bruit/enlarged Unilateral(lung, breast) ORBITAL METASTATICTHYROID EYE DISEASECAVERNOUS HEMANGIOMA TUMORPage 75 Proptosis in AdultsMar 25, 2013 76. PROPTOSISOrbital inflammation Hx of traumaSudden onset/chronic Severe conjunctivalAdults/childrenchemosisProminent red eyePulsatile ProptosisPain/DiplopiaOrbital bruitTrigeminal involvementORBITAL PSEUDOTUMORAV MALFORMATIONPage 76Proptosis in Adults Mar 25, 2013 77. TAKE HOME MESSAGE Proptosis is alarming condition Localized or systemic cause Proper approach Thorough Hx & investigation Proper management Page 77Proptosis in Adults Mar 25, 2013 78. Page 78 Proptosis in Adults Mar 25, 2013 79. MCQs1. Features of carotid-cavernous fistula include:a. dilated superior ophthalmic vein on CT scanb. blood in the Schlemmns canalc. Proptosis of contra lateral eye suggests bilateral carotid-cavernous fistulad. dural fistula is the commonest type seen following headinjurye. traumatic fistula rarely close spontaneouslya. T b. T c. F d. F e. T Page 79 Proptosis in AdultsMar 25, 2013 80. 2. A forty-eight year old woman presents with aseveral month history of slowly progressive,painless Proptosis and blurred vision in theleft eye. She is otherwise healthy. Herexamination was notable for choroidal foldsOSPage 80Proptosis in Adults Mar 25, 2013 81. a. Findings on the scan ?Ans. Well-defined, round, intraconal massb. What is probable Dx of the scan ?Ans. Cavernous hemangiomaPage 81 Proptosis in Adults Mar 25, 2013 82. 3. This is the orbital CT of a man with painlessprogressive Proptosis OS Page 82Proptosis in Adults Mar 25, 2013 83. a. Findings on the scan?Ans. Enlargement of muscles without involvement of the tendinous portionb. What is your diagnosis?Ans. Thyroid ophthalmopathy Page 83 Proptosis in Adults Mar 25, 2013 84. 4. 54 year old man with 4 days of progressivelyworsening pain and swelling around the righteye. Page 84 Proptosis in Adults Mar 25, 2013 85. Q. What is the differential diagnosis?Ans.Preseptal cellulitis orbital cellulitis pseudotumor malignancy traumaPage 85Proptosis in Adults Mar 25, 2013 86. 5. A 30 year old woman noticed mild Proptosiswhich accelerated during her pregnancy.Lesion caused hyperopia & retinal striae.CT showed well defined mass. Mostprobable Dx is:a.Metastatic breast carcinomab.Cavernous hemangiomac.Thyroid related orbitopathyd.PseudotumorAns. b Page 86Proptosis in Adults Mar 25, 2013 87. NEXTLecture Dr. Yousaf Jamal Proptosis in children Journal club Dr. Naeem KTKMar 25, 2013