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Final seminar on proptosis
1. Proptosis refers to the forward displacement of the eye within the orbit. It can be caused by conditions affecting the orbit or endocrine system. 2. A thorough history and physical examination are important to determine the cause, characteristics, progression of proptosis, and assess for signs of inflammation, infection, or underlying systemic disease. 3. Imaging studies like CT and MRI help identify the location, size, and nature of any orbital lesions causing proptosis and guide management. Biopsy may be needed to confirm diagnosis in some cases.
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Final seminar on proptosis
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- 2. PROPTOSIS Forward displacement ofthe eye (H.I.E Saunders.Dorland’s Medical Dictionary 26th Ed. Harcourt International Edition (2001) EXOPHTHALMOS Proptosis secondary to endocrinological dysfunction (Henderson JW. Orbital Tumors 3rd ed. New York: Raven press;1994 )
- 3. By Hertel’s Exophthalmometer Distance betweenlateral orbital margin and corneal apex greater than: •13 to 15mm in Asians¹ •21 mm adult Caucasians² •23 mm adult African- American² On CT scans³ •Globe protrusion > 21 mm anterior to the inter- zygomatic line on mid axial scans at the level of the lens. •Asymmetry between the two sides >2mm ¹Sarinnapakoran V et al,Proptosis in normal Thai samples and thyroid patients.J Med Ass Thai 2007;90(4):679-83 ²BeugerDG et al,Proptosis In: Ophthalmic secrets.Vander JF, GualtJA, Philadelpia,Pennsylvania,2002Chapter 36,p269 ³Naik MN et al, Interpretation of computed tomography imaging of eye and orbit.A systemic approach.Indian J Ophthalmology 2002;50(4):339-53
- 4. Orbit ispyramidal shaped cavity bounded by four OPEN anteriorly Contents- Surrounded by Volume of orbit - 30 cc PATHOPHYSIOLOGY SINUSE S GLOBE,MUSCLES FASCIA,VESSELS, NERVES,FAT, LACRIMAL GLAND, LACRIMAL SAC CRANIUM BONY WALLS
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- 6. SYMPTOMS Forward protrusionor displacement of eye Visual disturbances- diminution of vision, diplopia Discomfort- grittiness, watering, pain Difficulty in closing eyes fully while sleeping or blinking Increase in visible white part of eye
- 7. PROMINENT APPEARING GLOBE PSEUDOPROPTOSIS ENLARGED GLOBE EXTRAOCULAR MUSCLE WEAKNESS CONTRALATERAL ENOPHTHALMOS ASYMMETRIC PALPEBRAL FISSURES •HIGH AXIAL MYOPIA •BUPHTHALMOS•IPSILATERAL EYELID RETRACTION •CONTRALATERAL PTOSIS TRUE PROPTOSIS History and physical examination SHALLOW ORBIT NORMAL ORBIT •CRANIOFACIAL DYSOSTOSIS •TRAUMA ENDOCRINAL DISEASE VASCULAR MALFORMATION INFLAMMATORY NEOPLASIA INFECTIOUS •ORBITAL CELLULITIS •CAVERNOUS SINUS THROMBOSIS •PARASITIC •SINUS DISEASE NON INFECTIOUS MYOSITIS IDIOPATHIC ORBITAL EYE DISEASE SECONDARYPRIMARY MALIGNANTBENIGN •CAROTID- CAVERNOUS FISTULA •ARTERIOVENOUS (A-V) MALFORMATION •ORBITAL VARIX THYROID EYE DISEASE (TED)
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- 9. • Age ofpatient at onset • Progression • Pain • Visual disturbance • Laterality(unilateral / bilateral) • Nature- pulsatile/ intermittent / variable
- 10. • redness, dryeye, discomfort • Lid changes • Headache, nausea, fever • Systemic / extra-ocular disease • Medications • Previous trauma / surgery to orbit / face
- 11. AGE OF ONSET ATBIRTH CHILDHOOD/ YOUNG ADULT MIDDLE AGED/ ELDERLY UNILATERALBILATERAL CRANIOFACIAL DYSOSTOSIS •CROUZON’S SYNDROME •APERT’S SYNDROME INTERMITENT/PULSATILE NON PULSATILE •CEPHALOCELE •A-V MALFORMATIONS ORBITAL TUMORS •Vascular -Capillary hemangioma •Teratoma BIRTH TRAUMA
- 12. CHILDHOOD/ YOUNG ADULTMIDDLE AGED/ ELDERLY AGE OF ONSET PROGRESSION ACUTE (hours to a week) SUB-ACUTE (1-4 weeks) CHRONIC (>6 MONTHS) PAINFUL with/without DIMINUTION OF VISION INFLAMMATORY (Immuno- compromised state,redness , edema,fever) NON- INFLAMMATORY ORBITAL CELLULITIS PULSATILE h/o Trauma, surgery (headache,tinnius, diplopia) NON PULSATILE (h/o trauma) •A-V FISTULA •RETROBULBAR HEMORRHAGE SUDDEN SPURT IN PREEXISTING PROPTOSIS •Bleed in vascular tumors •Rupture dermoid or parasitic cyst •Lymphangioma
- 13. CHILDHOOD/ YOUNG ADULTMIDDLE AGED/ ELDERLY PROGRESSION ACUTE (hours to a week) SUB-ACUTE (1-4 weeks) CHRONIC (>6 MONTHS) PAINFUL PAINLESS (+/- DOV , diplopia, redness) UNILATERAL BILATERAL INFLAMMARTORY NON- INFLAMMATORY ORBITAL CELLULITIS (fever) (Diplopia) MYOSITIS TED IOID (intermittent/ recurrent) (Swelling,redness ,+/- DOV) MALIGNANTM ETASTASIC TUMOR CHILDHOOD/ YOUNG ADULT PRIMARY- Rhabdomyosarcoma SECONDARY – Retinoblastoma, sinus tumor METASTATIC-Wilm’s tumour,Neuroblastoma, AML MIDDLE AGED/ ELDERLY •METASTATIC-lung, breast, prostrate •SECONDARY – Intracranial tumor, sinus tumor INFLAMMARTORY + NON- INFLAMMATORY +/- (Swelling,redness +/-DOV) CAVERNOUS SINUS THROMBOSIS (fever , headache, vomiting) METASTATIC TUMOR •IDIOPATHIC ORBITAL INFLAMMATORY DISEASE •MALIGNANT TUMOR UNILATERAL BILATERAL MIDDLE AGE/ ELDERLY THYROID EYE DISEASE (diplopia,grittiness, watering)
- 14. CHILDHOOD/ YOUNG ADULTMIDDLE AGED/ ELDERLY PROGRESSION ACUTE (hours to a week) SUB-ACUTE (1-4 weeks) CHRONIC (>6 MONTHS) PAINFUL PAINLESS IDIOPATHIC INFLAMMATORY ORBITAL DISEASE (variable/recurrent) TED(diplopia) (Corneal dryness and exposure ,with DOV ) UNILATERAL BILATERAL LARGE TUMOR/ CYST WITH DOV WITHOUT DOV PRECEEDING PROPTOSIS OPTIC NERVE GLIOMA (children) FOLLOWING PROPTOSIS OPTIC NERVE MENINGIOMA BENIGN TUMOR BENIGN TUMOR CYST •Parasitic •Dermoid (children, young adult) •Capillary hemangioma (children) •Cavernous hemangioma (adults) •Lymphangioma (intermittent) VASCULAR TUMOR OTHER •Neurofibroma (pulsatile) •Schwannoma •Bone tumor •Lacrima gland tumor •Lymphoma(bilateral)
- 15. History of systemic/ extra-ocular disease Diabetes, immunocompromised status Sinus , dental and ENT diseases Prior nasal surgery Previous or current malignancy Thyroid dysfunction Trauma
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- 17. INSPECTION Compensatory headposture Facial scars, deformity Ocular symmetry and position of eye Adnexal structure Surface of eye Pulsations, valsalva maneuver Movements
- 18. POSITION OF EYE Forward protrusion without displacement AXIAL PROPTOSIS Displacement along with proptosis ABAXIAL PROPTOSIS
- 19. AXIAL PROPTOSIS Lesions ofintraconal space Optic nerve glioma Optic nerve sheath meningioma Cvernous hemangioma, Orbital varix Schwannoma Neurofibroma Cystic lesion Thyroid associated orbitopathy Idiopathic orbital inflammation DOWN AND IN Orbital mass of superotemporal quadrant •Lacrimal gland tumor •Dermoid •Other benign or malignant neoplasia DOWN AND OUT Orbital mass of superonasal quadrant •Frontoethmoidal mucocele •Fungal granulomas • Benign or malignant mass LATERAL DISPLACEMENT •Tumor arising from ethmoid sinus •Lacrimal sac tumors •Lethal midline granuloma •Nasopharyngeal tumor UPWARD DISPLACEMENT Mass from maxillary sinus •Neoplasia •Fungal granuloma •Dumbbell dermoid Cyst or tumor of inferior quadrant
- 20. 1.Nafziger’s Method 2.Clear plasticruler 3. Luedde’s exophthalmometer 4. Hertel’s exophthalmometer 5.Naugle’s exophthalmometer 6.Measure dystopia (2 scale)
- 21. INSPECTION OF ADNEXALSTRUCTURES SURFACE LID POSITIONMASS EYELIDS AND PERIBULBAR TISSUE COLOUR EDEMA SINUS OR FISTULA SITE NUMBER SIZE SURFACE MARGINS EXTENT COMPONENT – LID AND ORBITAL PTOSIS LID RETRACTION LID LAG
- 22. REDNESS Infection and inflammation Thyroid eye disease Malignantand metastatic neoplasm EDEMA Reduced venous and lynphatic drainage by mass Bluish discoloration- vascular tumor or malformation ,trauma Sinus and fistula- Orbital abcess, dermoids, mucocele
- 23. LID POSITION PTOSIS LID RETRACTION LIDLAG Mechanical Paralytic Thyroid eye disease Large orbital neoplasm Thyroid eye disease S shaped lid thickening - Plexiform neurofibroma
- 24. Pulsation –direct lateral view • Arteriovenous malformation • Cephalocele, large mucocele • Neurofibromatosis Valsalva maneuver Increase in proptosis with valsalva • AV malformations • Orbital varix
- 25. OCULAR MOVEMENTS Limitationof ocular motility due to orbital mass Restriction due to invasive process in muscle Paralytic
- 26. CONJUNCTIVA Dilated vascular channelat the canthus with chemosis- dysthyroid disease Epibulbar dark-red corkscrew vessels – increased venous pressure, AV malformation Diffuse conjunctival congestion- orbital inflammatory or infectious disease Sectoral congestion and chemosis- Myositis Subconjunctival haemorrhage- Trauma, infiltrative tumors Chemosis- inflammation, lymphatic obstruction Salmon patch- lymphoma CORNEA-exposure Iris- Lisch nodules in neurofibromatosis
- 27. EXAMINATION OF NASALCAVITY AND ORAL CAVITY IS MANDATORY IN PRESENCE OF PARANASAL SINUS INVOLVEMENT
- 28. • Local Temperature •Tenderness •Orbital tonometry, orbital margins • If mass palpable note •Position •Size,surface, attachnents •Consistency(hard , rubbery, spongy or soft) •Compressibility/ Reducibility
- 29. Tenderness Orbital infectionand inflammation Adenoid cystic carcinoma Trauma Temperature Rise in temperature of overlying skin seen in orbital infection and inflammation
- 30. Consistency of thepalpable mass Compressibility - characteristic of • Cystic mass Reducibility- characteristic of mass communicating with neighbouring cavity CYSTIC SOFT FIRMRUBBERY HARD
- 31. TRANSILLUMINATION (fluid / airfilled) AUSCULTATION Bruits- High flow arteriovenous fistula
- 32. VISUAL ACUITY PUPILLARY REACTION COLOR VISION REFRACTION VISUAL FIELD OPHTHALMOSCOPY INTRA OCULAR PRESSURE
- 33. Documentation of visualacuity is necessary Diagnostic and aids in planning management PUPILS VISUAL ACUITY Look carefully for RAPD suggestive of optic nerve damage
- 34. COLOUR VISION Inearly compression of optic nerve patient may not notice defective vision. In bilateral cases RAPD may not be elicited. Very early optic nerve compression can be missed in fundus examination IMPORTANCE
- 35. RAPD, COLOR VISION ABNORMALITIESAND VISUAL FIELD DEFECTS CAN DETECT COMPROMISE OF THE OPTIC NERVE EVEN WHEN THE VISUAL ACUITY IS NORMAL
- 36. Optic discs –edema, pallor, atrophy Choroidal folds - tumours, dysthyroid ophthalmopathy, inflammatory lesions FUNDUS EXAMINATION
- 37. Retinal vascular changes- Opticociliaryshunt vessels (optic nerve sheath meningioma, cavernous haemangioma) Venous dilatation and tortousity (a-v malformations, carotid cavernous fistula) Vascular occlusions (orbital cellulitis and optic nerve tumours
- 38. GENERAL EXAMINATION Lookfor evidence of malignancy Signs of thyroid dysfunction - thyromegaly, tachycardia, tremors of the hand etc. Sites of entry for infection -sinus disease, nasal and oral infection Lymphadenopathy
- 39. ON THE BASISOF HISTORY AND EXAMINATION DIFFERENTIAL DIAGNOSIS SHOULD BE MADE
- 40. IMAGING SPECIAL INVESTIGATIONS IMPORTANCE To knowexact location and extent of lesion Predict nature of lesion (tumor or cyst,encapsulated/infiltrating, Vascular/nonvascular,benign /malignant/metastatic) Plan proper management strategy
- 41. Various imagingmodalities in orbital disease X-Ray Non -contrast and contrast Computed Tomography Magnetic Resonance Imaging Ultrasound CT and MRI have largely replaced radiography Skull X-ray are now performed only in selective cases of facial fracture
- 42. THE CHOICE OFIMAGING STUDY SHOULD BE BASED ON CLINICAL PRESENTATION AND THE SPECIFIC PATHOLOGY BEING SUSPECTED
- 43. FINE NEEDLE ASPIRATIONCYTOLOGY AND BIOPSY IN CASE OF DOUBT AIDS IN ESHTABLISHING THE DIAGNOSIS PLAN MANAGEMENT Biopsy is indicated for histopathological confirmation of clinical diagnosis
- 44. DECISION MAKING GOALS FORMANAGEMENT Prevent life threatning condition Preservation of visual function Alleviation of pain Cosmesis
- 45. MANAGEMENT OPTIONS OF PROPTOSIS INFLAMMATORY NON- INFLAMMATORY OTHERS INFECTIOUSNON-INFECTIOUS (TED, IOID) NON- SURGICAL SURGICAL •Surgical drainage of abcess •Enucleation/ Exentration Systemic •Antibiotics •Antifungal ANALGESIC NON- SURGICAL SURGICAL •Non steroid anti inflammatory •Steroid •Immunosuppressent •Radiotherapy •Surgical decompression •EOM surgery •Lid surgery BENIGN MALIGNANT •Exentration •Excision NON- SURGICAL SURGICAL •Observation •Intralesional/ systemic steroid Capillary hemangioma Excision of mass NON- SURGICAL SURGICAL •Radiotherapy •Chemotherapy TRAUMA A-V MALFORMATION •Medical management of intraocular pressure •Closure of fistula •Medical management of intraocular pressure •Surgical decompression, exploration •Fracture repair
- 46. EVALUATION AND MANAGEMENT OFPROPTOSIS REQUIRES A MULTIDISCIPILINARY APPROACH IF NEEDED ALWAYS TAKE OPINION OF OTOLARYNGIOLOGIST, NEUROSURGEON, ONCOLOGIST
- 50. Large orbitaltumor with pressure on eye produce Irregular quadrantic contractions Meningioma- peripheral field affected earlier than central Glioma- Scotoma dispropotionately greater than proptosis
Editor's Notes
- #3 Protrusion of the globe secondary to non-endocrine causes (Henderson JW. Orbital Tumors. 3rd ed. New York: Raven Press(1994)
- #4 Lateral orbital rim to the corneal apex measures14 to 21 mm in adults Protrusion greater than 21 mm or a 2mm difference is generally abnormal
- #10 In light of the fact that there are numerous causes of proptosis, and due to the lack of direct visualisation of the pathology in orbital disease, a thorough history is extremely helpful in arriving at a differential diagnosis and in the determination of appropriate investigations of the patient. Specific points which should be addressed include:
- #18 Attentive gaze (Kocher’s sign) Infrequent blinking (Stellwag’s sign)
- #19 Axial-• lesion arises from within the muscle cone (intra-conal) Non-axial-lesion arises from without the muscle cone (extra-conal)
- #21 Stands behind the patient looks over forehead Raise patient’s upper lids with index fingers from the sides Compare position of apex of cornea on each side Patient bends head forward and cornea should disappear at the same time
- #26 Thyroid associated orbitopathy, Idiopathic orbital inflammation, myocysticercosis ,fungal granuloma Paralytic- Carotico cavernous fistula Limitation of motility following trauma- soft tissue edema , entrapment of muscle in orbital fracture or injury to muscle itself
- #35 If colour vision is defective evaluate carefully- Pupil for RAPD Do detailed fundus examination for Presence of disc edema,pallor,optocilliary shunt,retinal detachment
- #37 It is important to note that in some cases choroidal folds may precede the development of proptosis.
- #44 suspected and in those with secondary neoplasms from contiguous structures. Incisional biopsy is sometimes necessary in the case of suspected pseudotumour (particularly after poor response to oral steroids) and also in cases of suspected lymphoproliferative tumours where it may be necessary to perform a biopsy of at least one gram of fat.