Proptosis in adults

PROPTOSIS
Adults

Dr. Yousaf Jamal
FCPS Resident
Ophthalmology Unit
Hayatabad Medical Complex
20-02-2010

Mar 25, 2013

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

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.


General Causes

• Congenital • Acquired
– Dermoid cyst – Inflammatory
– Orbital teratoma – Infectious
– Congenital cystic – Trauma
eyeball – Vascular
– Cysts
– Neoplastic


Causes

• Inflammatory
– Thyroid-related orbitopathy
– Idiopathic orbital inflammatory disease
(pseudotumor)
– Sarcoidosis
– Wegener’s granulomatosis
– Systemic amyloidosis
– Histiocytosis


• Infectious
– Orbital cellulitis
– Subperiosteal abscess
– Gumma
– Mucormycosis
• Trauma
– Orbital blow-out fracture
– Retrobulbar hemorrhage
– Carotid-cavernous fistula
– Retained foreign body


• Vascular
– Orbital varices
– aneurysms
• Cysts
– Hydatid cyst
– Cysticercus
• Sinus related
– Mucocele
– Tumors


• 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

Clinical approach

• History
• Examination
• Investigation


History

• Age of onset
• Nature of onset
– Sudden
– Gradual
• Duration
– Continuous
– Intermittent postural / valsalva
– Pulsatile


• Associated symptoms
– Vision loss
– Pain
– Diplopia
• Past history
– Thyroid disorder
– Malignancy
• Lungs
• breasts
• Prostate

Examination

• Local examination
– Inspection
– Palpation
– Auscultation
– Tests
• Systemic examination


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


Inspection

– Bilateral
• Cranifacial synostosis, osteitis deformans, rickets
• TED, Mickulicz’s syndrome
• Histiocytosis, Amyloidosis, wegener’s granulomatosis
• Tumors


Inspection

• Direction of displacement
– Axial
– Non-axial
• Lateral
• Inferonasal
• Superior
• Lid signs


Palpation

• Orbital rims

• Regional lymph nodes


Auscultation

• Globe/temporal region for bruit


Other tests

• Transillumination
• Visual acuity
• Pupillary reactions
• Ocular motility
• Forced duction test
• Tonometry
• Fundoscopy
• Exophthalmometry


Systemic examination

• GPE
– For systemic disease
• Thyroid evaluation
• Primary neoplasm ??
• Otolaryngological examination


Investigations

LABORATORY INVESTIGATIONS

• FBC, TLC, DLC
• TFTs
• Casoni’s test
• Urine analysis


…Investigations

RADIOLOGY
• X-rays
• CT
• Orbital USG
• MRI
• Angiography


Causes in Adults

• Thyroid Exophthalmos
• Pseudotumor
• Orbital cellulitis
• Trauma
• Meningioma
• Lymphoma
• Histiocytoma
• Cavernous hemangioma


• Osteoma
• Varices
• Carotid-cavernous fistula
• Tumors extending from adjacent areas
– Lacrimal gland
– Sinuses
– lids
• Metastasis


Proptosis in Adults Mar 25, 2013

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,

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

THYROTOXICOSIS

• Autoimmune disorder
• 3rd – 4th decade, F > M
• 25-30% pts present TED
– Females 5 times more affected
• Inflammation of EOM
• Inflammatory cellular infiltration


Clinical manifestations

• Soft tissue involvement
• Lid retraction
• Proptosis
• Optic neuropathy
• Restrictive myopathy


Proptosis in TED

• Axial
• Unilateral/bilateral
• Symmetrical/asymmetrical
• Complications
– Exposure keratopathy
– Corneal ulceration
– Infection
– Compressional optic neuropathy


Work-up

• Complete Hx
– Known thyroid disease
– Smoking
– Visual changes
• Examination
• CT orbit
• Visual fields / Hess chart
• Thyroid function tests


Treatment of Proptosis

• General
– Lubricants
– Taping eyelids
– Cold compresses


Systemic steroids
• Oral prednisolone = 60-80mg/day
– Tapered over 2-8 weeks
• Intravenous methylprednisolone
– 0.5g/200ml isotonic saline infusion – 30 min


• Radiotherapy
– Alone or with steroids
• Combined therapy
– Radiations + Azathioprine + steroids
• Surgical decompression


PSEUDOTUMOR

• Idiopathic orbital inflammatory disease
• Non-infectious, non-Neoplastic
• May involve any of soft orbital tissue
– Muscles
– Lacrimal gland
– Tenon capsule
– sclera


Presentation

• Unilateral – adults, may be bilateral – children
• 3rd – 6th decade
• Acute
• Painful
• Periorbital swelling
• Redness/chemosis


• Congestive Proptosis
• Ophthalmoplegia
• Optic nerve dysfunction
• Palpable mass


Work-up

• Hx
• Examination
• CT orbit
– Ring sign (contrast)
• Biopsy
– FNAC
• In persistent cases


Course

• Spontaneous remission
• Intermittent episodes
• Prolonged inflammation
– Fibrosis
• Frozen orbit
– Ophthalmoplegia
– Ptosis
– Visual impairment


Treatment

• Observation
• Oral NSAIDs
• Systemic steroids
– Oral prednisolone = 60-80mg/day
• Radiotherapy
– If no response to steroids


• Anti metabolites
– If resistant to steroids and radiations
• Methotrexate
• Mycophenolate mofetil
• Systemic infliximab


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.


• 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


Presentation

• Middle aged
• Slowly growing unilateral axial proptosis
• Decrease in VA
• Diplopia
• Rarely gaze evoked amaurosis fugax
• May be accelerated by pregnancy


Signs

• Dilated & tortuous epibulbar vessels
• Other defects
– Color vision
– Visual field
– RAPD
• Fundus
– Optic disc edema & choroidal folds


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


Treatment

Surgical
• En-bloc removal
• Approach
• Lateral orbitotomy…lateral lesions
• upper eyelid or a transcaruncular-based medial
orbitotomy…medial aspect lesions


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)


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


Indirect carotid-cavernous fistula

• Communication btw cavernous sinus &
meningeal branches of ICA and/or ECA
• Low arterial blood flow
• Causes
– Spontaneous
– Congenital AV-malformations


Presentation

• Days or weeks after head injury
• Hx of surgery or systemic factors
– Triad
• Pulsatile proptosis
• Chemosis
• Whooshing noise in head
– Decreased vision
– Diplopia


• Epibulbar injection
• Ptosis
• Pulsatile proptosis with bruit
• Increased IOP
• Anterior segment ischemia
• Corneal edema
• Aqueous flare
• Iris atrophy
• Rubeosis iridis
• Cataract

• Exposure keratopathy
• Ophthalmoplegia
• Fundus changes
– Optic disc swelling
– Venous dilatation
– Intraretinal hemorrhages
– CRVO
– Ischemic optic neuropathy
– NVG


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


Treatment

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


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


Presentation

• Age…6th-8th decade
• F>M
• Chronic, painless, progressive visual
impairment
• Usually accompanied by proptosis
• Mostly unilateral
• Headache
• Double vision


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


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


Treatment

• Observation
– No intracranial extension and no vision loss
Medical
• Radiotherapy
– Primary radiation
– Radiation after surgery


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


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

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


TAKE HOME MESSAGE

• Proptosis is alarming condition
• Localized or systemic cause
• Proper approach
• Thorough Hx & investigation
• Proper management


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


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


a. Findings on the scan ?
Ans. Well-defined, round, intraconal mass

b. What is probable Dx of the scan ?
Ans. Cavernous hemangioma


3. This is the orbital CT of a man with painless
progressive Proptosis OS


a. Findings on the scan?
Ans. Enlargement of muscles without involvement of the tendinous
portion

b. What is your diagnosis?
Ans. Thyroid ophthalmopathy


4. 54 year old man with 4 days of progressively
worsening pain and swelling around the right
eye.


Q. What is the differential diagnosis?
Ans. Preseptal cellulitis
orbital cellulitis
pseudotumor
malignancy
trauma


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


NEXT

Lecture
• Dr. Yousaf Jamal
– Proptosis in children

Journal club
• Dr. Naeem KTK

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Proptosis in adults

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