Causes and Clinical Features of Proptosis in Adults
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The document discusses various causes and clinical approaches to evaluating proptosis in adults. Common causes in adults include thyroid eye disease, pseudotumor, orbital cellulitis, trauma, meningioma, lymphoma, and cavernous hemangioma. A thorough history, examination, and imaging studies are needed to differentiate between causes and guide treatment.
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Causes and Clinical Features of Proptosis in Adults
- 1. PROPTOSIS Adults Dr. Yousaf Jamal FCPS Resident Ophthalmology Unit Hayatabad Medical Complex 20-02-2010 Mar 25, 2013
- 2. CONTENTS • Introduction • General Causes • Clinical approach + Differentiating features • Causes in Adults • Common causes discussion • Summary • Take home message • MCQs Page 2 Proptosis in Adults Mar 25, 2013
- 3. Introduction • Proptosis * – Forward displacement or bulging, especially eyeball • Exophthalmos – Abnormal protrusion of eyeball * – Proptosis secondary to endocrinological dysfunction ** * Dorland’s medical dictionary 26th edition ** Henderson JW. Orbital Tumors. 3rd ed. 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 – Trauma eyeball – Vascular – Cysts – Neoplastic Page 4 Proptosis in Adults Mar 25, 2013
- 5. Causes • Inflammatory – Thyroid-related orbitopathy – Idiopathic orbital inflammatory disease (pseudotumor) – Sarcoidosis – Wegener’s granulomatosis – Systemic amyloidosis – Histiocytosis Page 5 Proptosis in Adults Mar 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 7 Proptosis in Adults Mar 25, 2013
- 8. • Neoplastic – Capillary – Optic nerve glioma hemangioma – Optic nerve sheath – Cavernous meningioma hemangioma – Retinoblastoma – Rhabdomyosarcoma – osteoma – Neurofibroma – Pleomorphic lacrimal gland adenoma – Leukemia, Lymphoma – Lac. Gland – metastasis carcinoma – Lymphangioma Page 8 Proptosis in Adults Mar 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 10 Proptosis 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 12 Proptosis in Adults Mar 25, 2013
- 13. Inspection • True or pseudoproptosis ?? • Laterality – Unilateral • Dermoid cysts, orbital teratoma, congenital cystic eyeball • Orbital hemorrhage, traumatic aneurysm • Cellulitis/abscess, cavernous sinus thrombosis • TED, pseudotumor • Varices, cysts, tumors Page 13 Proptosis in Adults Mar 25, 2013
- 14. Inspection – Bilateral • Cranifacial synostosis, osteitis deformans, rickets • TED, Mickulicz’s syndrome • Histiocytosis, Amyloidosis, wegener’s granulomatosis • Tumors Page 14 Proptosis in Adults Mar 25, 2013
- 15. Inspection • Direction of displacement – Axial – Non-axial • Lateral • Inferonasal • Superior • Lid signs Page 15 Proptosis 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 17 Proptosis in Adults Mar 25, 2013
- 18. Other tests • Transillumination • Visual acuity • Pupillary reactions • Ocular motility • Forced duction test • Tonometry • Fundoscopy • Exophthalmometry Page 18 Proptosis in Adults Mar 25, 2013
- 19. Systemic examination • GPE – For systemic disease • Thyroid evaluation • Primary neoplasm ?? • Otolaryngological examination Page 19 Proptosis in Adults Mar 25, 2013
- 20. Investigations LABORATORY INVESTIGATIONS • FBC, TLC, DLC • TFTs • Casoni’s test • Urine analysis Page 20 Proptosis in Adults Mar 25, 2013
- 21. …Investigations RADIOLOGY • X-rays • CT • Orbital USG • MRI • Angiography Page 21 Proptosis in Adults Mar 25, 2013
- 22. Causes in Adults • Thyroid Exophthalmos • Pseudotumor • Orbital cellulitis • Trauma • Meningioma • Lymphoma • Histiocytoma • Cavernous hemangioma Page 22 Proptosis 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 LYMPHOMA Pathology Hypertrophy of EOM, Hypocellular lymphoid Hypercellular lesion, GAGs accumulation lesion, mature lymphs immature malignant B cells Demographics 3rd-4th decade, 3rd-6th decade, adults & Old age commonest cause in children adults Associated Thyroid disease - lymph nodes Presentation F>M, painful, lid Painful, acute onset, Usually bilateral, retraction, lid lag, axial, axial, unilateral in painless, nonaxial, uni/bilateral, attentive adults, bilateral in child, rubbery palpable gaze, heat intolerance, congestion, mass, adnexal tachycardia, diminshed involvement, reflexes Radiology Fusiform appearance of Ill defined orbital Soft tissue mass, EOM opacification bone erosion Tx Steroids, radiotherapy, NSAIDS, steroids, Raiotherapy, decompression radiotherapy, infliximab chemotherapy antimetabolites, Page 25 Proptosis in Adults Mar 25, 2013
- 26. CAPILLARY ON SHEATH CCF HEMANGIOMA MENINGIOMA Pathology Vascular hemartoma Tubular enlargement of Abnormal ON, origin-meninges, communication btw psammoma bodies carotid artery & cavernous sinus Demographics Infant, commonest benign Late middle aged traumatic…men, orbital tumor in childhood, woman Spontaneous..women, spontaneous involution Associated Visceral, nasopharyngeal - HTN, atnerosclerosis, hemangioma, kasabach- trauma, pregnancy, merit syndrome connecive tissue disorder Presentation Nonaxial, +ve Valsalva, Axial, gaze evoked Pulsatile proptosis, superficial/deep amaurosis, EOM whooshing sound, impaired, optociliary ptosis, shunts unilateral/bilateral, chemosis, ant segment ischemia Radiology Intra/extraconal mass, Tubular enlargment, Ophthslmic veins, poorly defined hyperdense, ON cavernous sinus sheath enhancement enlargement Tx Observation, surgery Conservative, surgery Page 26 Proptosis radiotherapy, surgery in Adults Mar 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 31 Proptosis in Adults Mar 25, 2013
- 32. Treatment of Proptosis • General – Lubricants – Taping eyelids – Cold compresses Page 32 Proptosis 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 33 Proptosis 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 35 Proptosis 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 41 Proptosis in Adults Mar 25, 2013
- 42. • Anti metabolites – If resistant to steroids and radiations • Methotrexate • Mycophenolate mofetil • Systemic infliximab Page 42 Proptosis in Adults Mar 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 reported 1. Henderson GW. Vascular hamartomas, hyperplasias, and neoplasms. In: Henderson GW, ed. Orbital Tumors. New York: 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 43 Proptosis 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 44 Proptosis in Adults Mar 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 45 Proptosis 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 Adults Mar 25, 2013
- 47. Work-up CT – Well circumscribed, round or oval isointense mass – Usually in lateral part of muscle cone USG – High echogenecity on A-scan...reflect septae within the lesion Page 47 Proptosis in Adults Mar 25, 2013
- 48. Page 48 Proptosis in Adults Mar 25, 2013
- 49. Treatment Surgical • En-bloc removal • Approach • Lateral orbitotomy…lateral lesions • upper eyelid or a transcaruncular-based medial orbitotomy…medial aspect lesions Page 49 Proptosis in Adults Mar 25, 2013
- 50. Page 50 Proptosis in Adults Mar 25, 2013
- 51. CAROTID-CAVERNOUS FISTULA • An abnormal communication btw cavernous and 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 of ICA & cavernous sinus • High arterial blood flow • Causes – Spontaneous…25% • Middle aged woman, atherosclerosis, HTN, pregnancy • Collagen vascular disease, Connective tissue disorders – Trauma (basal or facial skull fracture)…75% – Iatrogenic Page 53 Proptosis in Adults Mar 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 54 Proptosis 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 55 Proptosis in Adults Mar 25, 2013
- 56. • Epibulbar injection • Ptosis • Pulsatile proptosis with bruit • Increased IOP • Anterior segment ischemia • Corneal edema • Aqueous flare • Iris atrophy • Rubeosis iridis • Cataract Page 56 Proptosis 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 62 Proptosis in Adults Mar 25, 2013
- 63. Treatment Medical • Exposure keratopathy • Glaucoma • NVG Page 63 Proptosis in Adults Mar 25, 2013
- 64. Surgery • Indication – Post traumatic fistula – Failure to spontaneous closure • Procedures – Surgical repair of the damaged portion of the intracavernous internal carotid artery – Electrothrombosis, Embolization, or Balloon occlusion of the fistula Page 64 Proptosis in Adults Mar 25, 2013
- 65. OPTIC NERVE SHEATH MENINGIOMA • Arise from meningiothelial cells of arachnoid villi, 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 Adults Mar 25, 2013
- 66. Presentation • Age…6th-8th decade • F>M • Chronic, painless, progressive visual impairment • Usually accompanied by proptosis • Mostly unilateral • Headache • Double vision Page 66 Proptosis in Adults Mar 25, 2013
- 67. Signs • Classic triad – visual loss – optic atrophy – optociliary shunt vessels (30%) • Restrictive motility defect * • Axial proptosis Sibony PA, Krauss HR, Kennerdell JS, Maroon JC, Slamovits TL. Optic nerve sheath meningiomas. Clinical manifestations. Ophthalmology. Nov 1984;91(11):1313-26 Page 67 Proptosis 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 meningioma easily detected Page 70 Proptosis in Adults Mar 25, 2013
- 71. Page 71 Proptosis in Adults Mar 25, 2013
- 72. Treatment • Observation – No intracranial extension and no vision loss Medical • Radiotherapy – Primary radiation – Radiation after surgery Page 72 Proptosis in Adults Mar 25, 2013
- 73. Chemotherarpy • Unresectable, recurrent, or previously irradiated • 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 Proptosis Unilateral/bilateral Slow growing Motility problems Lid retraction/lag Retropulsion Orbital pain Heat intolerance Axial Proptosis Diplopia Woman…20-40yrs Increased hyperopia Primary tumor Thyroid bruit/enlarged Unilateral (lung, breast) ORBITAL METASTATIC THYROID EYE DISEASE CAVERNOUS HEMANGIOMA TUMOR Page 75 Proptosis in Adults Mar 25, 2013
- 76. PROPTOSIS Orbital inflammation Hx of trauma Sudden onset/chronic Severe conjunctival Adults/children chemosis Prominent red eye Pulsatile Proptosis Pain/Diplopia Orbital bruit Trigeminal involvement ORBITAL PSEUDOTUMOR AV MALFORMATION Page 76 Proptosis 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 77 Proptosis in Adults Mar 25, 2013
- 78. Page 78 Proptosis in Adults Mar 25, 2013
- 79. MCQs 1. Features of carotid-cavernous fistula include: a. dilated superior ophthalmic vein on CT scan b. blood in the Schlemmn's canal c. Proptosis of contra lateral eye suggests bilateral carotid- cavernous fistula d. dural fistula is the commonest type seen following head injury e. traumatic fistula rarely close spontaneously a. T b. T c. F d. F e. T Page 79 Proptosis in Adults Mar 25, 2013
- 80. 2. A forty-eight year old woman presents with a several month history of slowly progressive, painless Proptosis and blurred vision in the left eye. She is otherwise healthy. Her examination was notable for choroidal folds OS Page 80 Proptosis in Adults Mar 25, 2013
- 81. a. Findings on the scan ? Ans. Well-defined, round, intraconal mass b. What is probable Dx of the scan ? Ans. Cavernous hemangioma Page 81 Proptosis in Adults Mar 25, 2013
- 82. 3. This is the orbital CT of a man with painless progressive Proptosis OS Page 82 Proptosis in Adults Mar 25, 2013
- 83. a. Findings on the scan? Ans. Enlargement of muscles without involvement of the tendinous portion b. 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 progressively worsening pain and swelling around the right eye. Page 84 Proptosis in Adults Mar 25, 2013
- 85. Q. What is the differential diagnosis? Ans. Preseptal cellulitis orbital cellulitis pseudotumor malignancy trauma Page 85 Proptosis in Adults Mar 25, 2013
- 86. 5. A 30 year old woman noticed mild Proptosis which accelerated during her pregnancy. Lesion caused hyperopia & retinal striae. CT showed well defined mass. Most probable Dx is: a. Metastatic breast carcinoma b. Cavernous hemangioma c. Thyroid related orbitopathy d. Pseudotumor Ans. b Page 86 Proptosis in Adults Mar 25, 2013
- 87. NEXT Lecture • Dr. Yousaf Jamal – Proptosis in children Journal club • Dr. Naeem KTK Mar 25, 2013
Editor's Notes
- Lid swelling , ecchymosis n chemois suggest cellulitis, lid lag n staring suggst TED.
- Retropulson also for pulsations..Lymphatic supply of retina??