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06. Proptosis by Dr. Ummara Shafiq.pdf
1. Dr. Ummara Shafiq
M.B.B.S, M.R.C.S (UK), MS Ophthalmology (King Edward Medical University)
Fellow of the International Council of Ophthalmology (FICO)
Proptosis
Consultant Ophthalmologist
National Eye Centre, Lahore (NECL)
Zarar Shaheed Trust Hospital, Lahore
Shaheen Welfare Hospital, Lahore
Assistant Professor,
Physical Therapy & Rehab Sciences,
Allied Health Sciences,
Superior University, Lahore
2. Proptosis
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Dr. Ummara Shafiq
• Abnormal protrusion of eyeball.
• Exophthalmos?
• Same as above but usually used in relation to endocrinopathies such as thyroid related
proptosis.
• >21 mm or asymmetry of >2 mm between the eyes
What is Proptosis?
3. Proptosis / Dystopia
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Dr. Ummara Shafiq
• Proptosis > forward displacement of
eyeball
• Dystopia > displacement of globe in
coronal plane (horizontal or vertical).
• It may or may not coexist with proptosis
or enophthalmos
Difference:
4. Exorbitism
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Dr. Ummara Shafiq
• Exorbitism is a protrusion of the eyeball due to a decrease in capacity of the orbital cavity, with a
normal orbital content volume.
• Abnormal skull development may lead to shallow orbits causing protrusion of the eyes and
inability to close eyes.
• Mostly bilateral e.g: craniosynostosis
5. Pseudoproptosis
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Dr. Ummara Shafiq
• It is the false impression of proptosis:
1. Buphthalmos
2. High myopia
3. Contralateral ptosis
4. Contralateral enophthalmos
5. Facial asymmetry
6. Pathophysiology
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Dr. Ummara Shafiq
• The basic pathophysiology irrespective of the aetiology is an increase in volume of the orbital
content within the fixed bony orbital confines.
• The orbit is widest anteriorly and not enclosed so the contents are displaced anteriorly resulting
in proptosis.
7. Proptosis
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Dr. Ummara Shafiq
• Visual Threatening: exposure keratopathy, compressive optic neuropathy
• Life Threatening: malignant tumors.
• Cosmetic implications
Importance Of Proptosis
8. Proptosis
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Dr. Ummara Shafiq
• Primary orbital pathology
• Secondary to systemic diseases.
V
I
T
A
M
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• Vascular: Orbital Varices, carotid cavernous fistula , lymphangioma , haemangiomas , AV fistulas
• Inflammatory: Thyroid orbitopathy , orbital inflammatory syndrome/ pseudotumor
• Trauma: orbital hemorrhage , fractures
• Aneurysm(CYSTIC LESIONS): dermoid cyst, encephlocele , mucocele
• Miscellaneous: metastatic Ca. Craniosynostosis
• Infection: Orbital Cellulitis , mucormycosis ,
• Neoplasm: optic nerve glioma, optic nerve sheath meningioma, Leukaemia, lymphoma,
rhabdomyosarcoma
Causes Of Proptosis
9. Proptosis
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Dr. Ummara Shafiq
• Unilateral/bilateral
• Acute/intermittent
• Pulsating/not
• Axial/non-axial
Classification Of Proptosis
11. Proptosis
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Dr. Ummara Shafiq
• Onset
• duration
• progression
• Constant or intermittent
• Decreased vision early/late
• Stationary/progressive
• Pain
• Double vision
• Periorbital neurosensory loss
• bruits
• symptoms aggravated by crying,coughing,straining, nose blowing
• Past h/o : Trauma ,fever , chills ,cancer, thyroid d/s
History
12. Proptosis
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Dr. Ummara Shafiq
• A sudden dramatic proptosis with conjunctival prolapse in a child with recent URTI
• Lymphangioma
• GAZE-EVOKED AMAUROSIS
• May be associated with an orbital apex tumor
• Proptosis provoked by straining
• Orbital Varices
• History of “tinnitus”
• Arteriovenous Shunt
History
13. General Physical Examination
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Dr. Ummara Shafiq
• Skin and oropharynx: cutaneous /oral vascular lesions > lymphangioma , café au lait
spots>neurofibromatosis
• Cranial Nerve Examination: periorbital and corneal sensation
• Examination of Chest and Abdomen: systemic malignancy: undiagnosed ca breast
• The regional/ generalized lymphadenopathy: lymphoproliferative disorder
15. Local Examination
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Dr. Ummara Shafiq
INSPECTION:
• Examiner looks from above standing behind patient/looks up from below with the patient’s head
tilted back
1. Type of proptosis (axial / non-axial),location of mass, visible pulsation, skin changes.
2. Lagophthalmos , conjunctival congestion/ discoloration.
3. examination of the globe and ocular adnexa * Dilated episcleral vessels: arteriovenous
shunt.
4. Corneal exposure, change in size with valsalva.
16. Local Examination
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Dr. Ummara Shafiq
PALPATION:
• Size, shape, surface, margins , consistency
• Signs of inflammation
• Tenderness
• Reducibility, motility
• Variation with valsalva
• resistance to retropulsion , Thrill
• Corneal sensation, infraorbital / supraorbital
• Any swelling around the eyeball, regional lymph nodes & orbital rim
17. Local Examination
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Dr. Ummara Shafiq
AUSCULTATION
• Abnormal vascular communications -> bruit caroticocavernous fistula
• Globe temporal region for bruit with bell
18. Local Examination
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Dr. Ummara Shafiq
TESTS
• Transillumination
• Visual acuity
• Pupillary reactions
• Ocular motility
• Forced duction test
• Tonometry
• Fundoscopy
• Exophthalmometry
22. Exophthalmometry
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Dr. Ummara Shafiq
Luedde Exophthalmometer:
• Transparent plastic mm ruler which is thicker than normal
• Fastest, easiest and least expensive method for
measuring exophthalmos
23. Exophthalmometry
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Dr. Ummara Shafiq
Naugle Exophthalmometer
• Superior and inferior orbital-rim based
instrument
• Can measures the position of the globe
accurately even after lateral orbitotomy
25. Measurement
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Dr. Ummara Shafiq
• The normal range is 12–21 mm.
• A difference greater than 2 mm between the eyes is significant.
• In children and teenagers mean measurements increase with age
• Less than 4 years old (13.2 mm)
• 5–8 years old (14.4 mm)
• 9–12 years old (15.2 mm)
• 13–17 years old (16.2 mm).
• Depending upon the configuration of the osseous orbit, a value of 15mm might be pathological
whereas 21mm might be normal. Axial Length of the eye affects.
26. Investigations
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Dr. Ummara Shafiq
Orbital Imaging
• X-ray Calcification , hyperostosis , blow out fractures
• CECT - initial choice of investigation
• MRI - orbital apex , CNS involvement , soft tissue infiltration and RB
• USG – to assess internal reflectivity of lesion & calcification in RB
• CT/MRI angiography for vascular lesions
29. Management
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Dr. Ummara Shafiq
Local Management:
• Sleep in supine position with head elevated (to reduce pressure symptoms)
• Taping of lids at night (prevent corneal exposure)
• Prisms in diplopia (conservative)
Medical Therapy:
• Topical tear substitutes
• Systemic diuretics – in vascular lesions
• Parenteral antibiotics
• Pain killers
33. Pseudotumor
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Dr. Ummara Shafiq
• Idiopathic orbital inflammatory disease
• Non-infectious, non-Neoplastic
• May involve any of soft orbital tissue
o Muscles
o Lacrimal gland
o Tenon capsule
34. Presentation
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Dr. Ummara Shafiq
• Unilateral – adults, may be bilateral – children
• 3rd – 6th decade
• Acute
• Painful
• Periorbital swelling
• Redness / chemosis
• Congestive Proptosis
• Ophthalmoplegia
• Optic nerve dysfunction
• Palpable mass
36. Treatment
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Dr. Ummara Shafiq
• Middle aged (female)
• Slowly growing unilateral axial proptosis
• Decrease in VA
• Diplopia
• Rarely gaze evoked amaurosis fugax
• May be accelerated by pregnancy
37. Signs
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Dr. Ummara Shafiq
• Dilated & tortuous epibulbar vessels
• Other defects
– Color vision
• – Visual field
• – RAPD
• Fundus – Optic disc edema & choroidal folds
38. Work-Up
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Dr. Ummara Shafiq
• 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
40. Carotid-cavernous Fistula
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Dr. Ummara Shafiq
• 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
41. Direct Carotid-cavernous Fistula
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Dr. Ummara Shafiq
• 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
45. Work-up
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Dr. Ummara Shafiq
• 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
46. Treatment
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Dr. Ummara Shafiq
• Medical
• Exposure keratopathy
• Glaucoma
• NVG
• 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
47. Optic Nerve Sheath Meningioma
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Dr. Ummara Shafiq
• 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
49. Optic Nerve Sheath Meningioma
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Dr. Ummara Shafiq
WORK-UP
• CT scan (with contrast)
o Fusiform appearance of ON
o Mostly at orbital apex
o Calcifications usually seen
o Bony involvement
• MRI
o Clearly show small tumors
o Intraorbital extension of intracranial
o Meningioma easily detected
50. Optic Nerve Sheath Meningioma
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Dr. Ummara Shafiq
TREATMENT
• Observation – No intracranial extension and no vision loss
• Radiotherapy – Primary radiation – Radiation after surgery
• Chemotherarpy
o Unresectable, recurrent, or previously irradiated
o Combinations – 5-fluroouracil, folate, and levamisole
• Surgical excision
51. Proptosis in Children
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Dr. Ummara Shafiq
• Orbital cellulitis
• Dermoid cyst
• Capillary hemangioma
• Optic nerve glioma
• Rhabdomyosarcoma
52. Orbital Cellulitis
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Dr. Ummara Shafiq
• Bacterial orbital cellulitis – life-threatening
• Infection of soft tissues behind septum
• More common in children
• Organisms
o S. pneumoniae
o S. aureus
o S. pyogenes
o H. influenzae
53. Orbital Cellulitis
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Dr. Ummara Shafiq
• Sinus-related – Ethmoidal (90%)
• Extension of preseptal cellulitis
o Dacryocystitis
o Facial cellulitis
o Dental infections
• Systemic bacteremia
• Post-trauma
o Penetrating septum (48- Penetrating septum
• Post-surgical
o Lacrimal
o Orbital
Etiology
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Dr. Ummara Shafiq
INDICATIONS
• Decreasing vision
• Afferent pupillary defect
• No response to antibiotics in 48-72 hrs
• Orbital/Subperiosteal abscess
• Atypical picture…..Needs biopsy
• Canthotomy/Cantholysis in emergency
• Surgical drainage of fluid
• Orbital surgery + sinusotomy
• Exenteration
Orbital Cellulitis
Surgical Treatment
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Dr. Ummara Shafiq
• A choristoma, found adjacent to suture lines
• Connected to periorbita by fibrovascular tissue
• Content
o Keratinized stratified squamous epithelium
o Blood vessels, fat, collagen, sebaceous glands, and hair follicles
o a tan oily liquid to a white or yellow substance
Dermoid Cyst
Definition
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• A choristoma, found adjacent to suture lines
• Connected to periorbita by fibrovascular tissue
• Content
o Keratinized stratified squamous epithelium
o Blood vessels, fat, collagen, sebaceous glands, and hair follicles
o a tan oily liquid to a white or yellow substance
Dermoid Cyst
Definition
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• Mostly in children
• Mostly…Superotemporal aspect of orbit
• A painless mass
• Slowly growing
• Usually < 1 cm in diameter, non-tender
• Globe displacement
• Inflammation if ruptures
• May – Compress optic nerve – Diplopia
Dermoid Cyst
Presentation
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• CT/MRI
o Indicated if posterior extent not palpated
o Cystic appearance
o Well circumscribed lesion
Dermoid Cyst
Workup
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• Most common orbital tumor of infancy
• Benign endothelial cells neoplasms
• Absent usually at birth
• Rapid growth in infancy
• Involution later on
Capillary Hemangioma
Definition
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• Thought … of placental origin
• 02 phases of growth
o Proliferative
• Rapid growth…8-18 months
• Increase in no. of endothelial & mast cells (stimulus for vessel growth)
o Involutional
• 30-50% involute by 5 years
• 75% by 7 years
Capillary Hemangioma
Pathophysiology
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• F:M…3:1
• 1-2% in neonates
• All develop by 6 months
• Typically…red spot growing in size in Periorbital area
• Inability to open lids
• Bulging of the eye
CLASSIFIED
• Cutaneous
• Purely preseptal
• Preseptal with extraconal element
• Combination of preseptal, extraconal and intraconal
Capillary Hemangioma
Presentation
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• Usually…superonasal , brow or lid lesion
• Typically blanches with pressure
• Ptosis & proptosis if posterior extension
• Visual loss
• Preseptal …Dark blue or purple
• Enlarge & change color while crying
Capillary Hemangioma
Signs
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Capillary Hemangioma
Work-up
• CT – poorly circumscribed mass with no bony erosion – Homogenous enhancing lesion
• MRI – hypointense to fat on T1-weighted & hyperintense on T2-weighted scans
• US & Doppler – For anatomic relations & extent
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Capillary Hemangioma
Mortality/Morbidity
• Kasabach-Merritt syndrome
o Coagulopathy, thrombocytopenia
o large visceral / Nasopharyngeal hemangiomas
o DIC may occur, high output CCF
o Mortality…30-50%
• Ophthalmic morbidity
o Space occupying lesion
o Amblyopia
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Capillary Hemangioma
Treatment
• Medical
o Observation
o Steroids – Clobetasol propionate cream – Injectable steroid formulations – Systemic
corticosteroids are used for amblyogenic lesions
o Interferon alfa-2a – Resistant to steroid treatment
• Surgery
o Laser surgery
• Carbon dioxide laser
• Argon laser
• Nd:YAG laser
o Primary excision
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Optic Nerve Glioma
Definition
• Most common primary tumor of ON
o In children…mostly benign
o In adults…Aggressive glioma
• WHO classify it as
o Grade I astrocytomas (pilocytic astrocytomas)
o slow growing & tend not to metastasize
• Generalized hyperplasia of glial cells in the nerve to complete disorganization
• 10-38% of pediatric pts with ON glioma have NF-1
• Bilateral ON gliomas…almost pathognomonic for NF-1
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Optic Nerve Glioma
Presentation
• ON gliomas are
o 4% of orbital tumors
o 2/3rd of all primary optic nerve tumors
• 20% gliomas extend to the optic chiasm or beyond into the optic radiations & aggressive
• F>M
• Painless proptosis with inferior dystopia
• Decreased vision
• Large lesion can obstruct 3rd ventricle & ICP
• Optic atrophy
• Optociliary collaterals
• CRVO
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Optic Nerve Glioma
Work-Up
• CT
o Diffuse Fusiform enlargement of the optic nerve
• MRI
o T1-weighted images – Isointense to hypointense
o T2-weighted images – Isointense to hyperintense
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Optic Nerve Glioma
Treatment
• Not required if
o Not growing
o Good vision
o No cosmetic blemish
• Surgical excision
o Large & growing lesions
o Confined to orbit
• Radiotherapy + chemotherapy
o Intracranial extension
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Rhabdomyosarcoma
Definition
EMBRYONAL SARCOMA
• Most common primary orbital malignancy of childhood
• Origin…undifferentiated mesenchymal cells
• Called Rhabdomyosarcoma if differentiate into striated muscle
• The four main histological type:-
1. Embryonal
2. Botyroid
3. Alveolar
4. Pleomorphic
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Rhabdomyosarcoma
Presentation
• Usually first decade (average…7yrs)
• Boys > girls
• Proptosis
o Rapid (days to weeks)
o Unilateral
o Painful
o Non-axial
• Mimics orbital cellulitis
• Swollen lids, bluish appearance
• Ptosis
• Chemosis
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Rhabdomyosarcoma
Presentation
• Location
o Superonasal
o Retro bulbar
o Superior
o Inferior
• Sinuses involvement & nose bleeds
• Other findings
o Disc edema
o Choroidal folds
o Decreased vision
o Cervical lymph nodes involvement
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Lymphangioma
Definition
• Introduction
o Non-Neoplastic
o Non-functional
o Abortive
o Vascular malformation
• Hemodynamically isolated
• Bleeding into lumen…chocolate cysts
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Lymphangioma
Presentation
• Early childhood (average=5-6 yrs)
• Multiple bluish soft masses
• Upper nasal quadrant
• Cystic conjunctival component
• Proptosis
o Due to spontaneous hemorrhage
o Painful
o Axial/non-axial
• Worsened by RTI
• Palat involvement
• Ptosis
• Restricted eye movements
• Optic nerve compression
• Papilledema
• Secondary glaucoma
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Lymphangioma
Work-Up
• CT
o Multilobulated cystic mass within the orbit and/or intralesional calcifications
• MRI
o Hemorrhagic cyst…Hyperintense
o Fluid level
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Lymphangioma
Treatment
• Conservative
• Intralesional injection of the sclerosing agents
• Needle aspiration
• CO2 laser
• Local radiotherapy
• Cryotherapy
• Partial surgical resection of cyst
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Summary
PROPTOSIS
Lid edema
Sinus disease
Restricted motility
Pain / Tender
Lid trauma
Fever
Little/no inflammation
Orbital lesion
Young child/adult
Slow growing
Located near bone
Sutures
ORBITAL CELLULITIS DERMOID CYST
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Summary
PROPTOSIS
Child (5 years)
Upper nasal quadrant mass
Painful
Few episodes of proptosis
Worsened by RTI
MRI shows fluid levels
Child (7-8 years)
Hx of nose bleeds
Bluish color lids
Unilateral
Lymph node involved
Painful
ORBITAL LYMPHANGIOMA RHABDOMYOSARCOMA
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Summary
PROPTOSIS
Orbital inflammation
Sudden onset/chronic
Adults/children
Prominent red eye
Pain/Diplopia
Trigeminal involvement
Hx of trauma
Severe conjunctival
Chemosis
Pulsatile Proptosis
Orbital bruit
ORBITAL PSEUDOTUMOR AV MALFORMATION