PROPTOSIS:How to approach?

History, clinical examination, investigations and differntial diagnosis

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DEFINITION:• PROPTOSIS: Forward displacement of bulging

especially that of eye.• Abnormal protrusion of the globe beyond the

orbital margins with the patient looking straight ahead

• Word EXOPHTHALMOS synonymous – but is more specific for the. eye

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Causes of pseudoproptosis:Simulation of abnormal protrusion of the eye or a true

abnormal protrusion of eye or a true abnormal protrusion that doesn’t originate from a mass, inflammation or a vascular disorder

1.u/l high axial myopia2.u/l congenital glaucoma3.u/l secondary glaucoma resulting from ocular trauma

during childhood4.shallow c/l orbit as in crouzon’s ds(craniosynostosis)5.hypoplastic supra-orbital ridges as in trisomy186.assymetry of body of orbits7.facial asymmetry 8. Lid retraction, ptosis or enopthalmos.

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CLASSIFICATIONS

Etiology dysthyroid orbitopathy inflammatory tumours & cysts

Laterality unilateral bilateral Direction. Axial non-axial

Time of onsetchildhood-congenital acquired adulthood

DurationAcuteSubacutechronic

Clinical course StationaryProgressiveRegressivePulsatingIntermittentpositional

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CAUSES OF PROPTOSIS:• Inflammation

Acute-orbital cellulitis Chronic(nongranulomatous)-

pseudotumour Chronic(granulamatous)TB,sarcoid,syphilis,parasites,Aspergill

osis Benign lymphoepitheliallesion (Mikulicz’s ds)

Injuries

-foreign body -orbital hemorrhage

• Vascular disorders -collagen ds-SLE or PAN -cranial arteritis -allergic vasculitis -thrombophlebitis -AV aneurysm or varices

Systemic disease -Thyroid disorder -Myasthenia gravis -Acute intracranial

hypertension

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TUMOURSPRIMARY

a.Dermoid b.Hemangioma c.lymphangioma d.Phakomatoses 1.neurofibromatoses 2.Sturge-weber ds 3.tuberous sclerosis e.Lipoma f. Fibrous xanthoma g.Rhabdomyosarcoma h.Amputation neuroma I.Neurilemmoma j.Glioma of optic nerve k.Meningioma l.Lacrimal gland lesions m. Lymphoma &leukemia n.Hand-Schuller-Christian ds o.Juvenile xanthogranuloma

SECONDARY

• 1.Direct extension from- a.intraocular region:malignant

melanoma,retinoblastoma b.eyelid:bcc,scc,malignant

melanoma,mucoepidermoid ca c.conjunctiva:scc,malignant

melanoma,mucoepidermoid ca d.intracranium:meningioma e.PNS:frontal,ethmoid,maxillary

tumours• 2.Metastatic lesion -neuroblastoma(child) -primary in lung,

breast,prostrate(adults) -malignant melanoma of skin

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EVALUATION OF THE PATIENT

• HISTORY -Age of onset -nature of onset -duration -progression -symptoms -associated symptoms/systemic symptoms

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AGE OF ONSET:• NEWBORN -orbital sepsis -orbital neoplasm NEONATAL -infections of maxilla EARLY CHILDHOOD(upto 1 yr) -dermoid -hemangioma -orbital extension of

retinoblastoma -Hand-Schuller-Christian ds

• 1-5 Yrs OF AGE -dermoid -orbital extension of

retinoblastoma -hemangioma -metastatic

neuroblastoma -glioma of optic n.

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• Young Adult -pseudotumour -thyroid ophthalmopathy -mucocele -meningioma -fibrous dysplasia -osteoma -undifferentiated

sarcoma -lacrimal gland tumour

• Old age: -pseudotumour -sino-orbital mucocele -malignant lymphomas&

leukemias -meningioma -ca of palpebral or

epibulbar region -metastatic ca

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NATURE OF ONSET:Sudden onset

-orbital emphysema -rupture&infection of ethmoidal mucocele -retrobulbar hemorrhage&infection

Gradual onset -benign tumour -ASPERGILLOSIS

Rapidly expanding orbital masses -rhabdomyosarcoma,neuroblastoma,eosinophilic granuloma,

capillary hemangioma,traumatic hematoma, orbital cellulitis/abcess,pseudotumour

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PROGRESSION:Continuous progression

-tumours & endocrinal exophthalmos

Intermittent proptosis -orbital varices -recurrent hemorrhage -vascular neoplasm -lymphangioma

Variable • pseudotumours&angiomas

Pulsating -carotidocavernous

aneurysm -large frontal mucocele -meningoencephalocele -blow out fracture of roof

of orbit

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SYMPTOMATOLOGY:PAIN:

orbital inflammatory disorders,traumatic cases with orbital hematoma, malignancy

DIPLOPIA: common symptom in orbital disorders related to paralysis of

extraocular muscles or restriction of ocular movements.

OPTHALMIC EXAMINATIONVISUAL ACUITY—provides an indicator of extent of orbital

ds.&decreased vision suggests either exposure keratitis or involvement of optic n. Loss of vision prior to proptosis in children suggests optic n. glioma.

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INSPECTION

• Important to look at the entire face in order to get a sense of facial proportion & symmetry.

1.whether proptosis is true or false 2.whether proptosis unilateral or bilateral. Inspection of eyelids-diagnostic clues• Swelling of lids with ecchymosis & chemosis of

conjunctiva-orbital cellulitis• Pediatric disorders that cause eyelid ecchymosis—

neuroblastoma,ewing’s sarcoma,leukemia, eosinophilic granuloma,lymphangioma,traumatic hematoma

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Conjunctiva

-dysthyroid orbitopathy-hyperemia near insertions of recti muscles

-orbital vascular malformation or caroticocavernous fistula-dilated,slightly tortuous larger vessels that extend to corneoscleral limbus

-idiopathic orbital inflammation-marked diffuse injection of smaller conjunctival &episcleral blood vessels

• Direction of displacement

• Ethmoidal sinus mucocele displaces globe laterally

• Mass in lacrimal fossa-downward & nasal displacement

• Axial proptosis-mass inside muscle cone eg.optic n. glioma ,meningioma, grave’s

• Maxillary sinus growth-superior displacement

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Differential Diagnosis:Causes of u/l proptosis• Congenital-dermoid cyst,orbital teratoma

etc.

• Traumatic -orbital hemorrhage,traumatic aneurysm,foreign body etc.

• Inflammatory -orbital cellulitis/absess,cavernous sinus thrombosis(proptosis is intially u/l then becomes b/l),fungal, pseudotumours

• Vascular lesions-orbital varix &aneurysm(saccular aneurysm of ophthalmic artery,carticocavernous fistula)

• Cysts of orbit-haematic cyst,parasitic cyst(hydatid cyst,cysticercosis)

• Tumours-primary,secondary or metastatic

Causes of b/l proptosis• Developmental anomalies of skull- craniofacial dysostosis eg.

Oxycephaly(tower skull)

• Osteopathies- osteitis deformans, rickets,acromegaly

• Inflammatory conditions-Mikulicz’s syndrome,late stage of cavernous sinus thrombosis

• Endocrinal exophthalmos-thyrotoxic or thyrotropic

• Tumours-lymphoma,lymphosarcoma,secondaries

• Systemic ds-histiocytosis,systemic amyloidosis,xathomatosis&wegener’s granulomatosis

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• PALPATION:• Retrodisplacement of globe should be estimated• Resistance: painful/hard• In orbital varices-complete reducibility of eyeball which

comes back on valsalva or bending of head• Thrill palpable in CCF or AV malformations• Palpation of orbital rims – to note any change in

contour or dehiscence of any orbital wall• AUSCULTATION:• for abnormal vascular communications that generate a

bruit• LYMPHADENOPATHY-preauricular,cervical neck nodes• ENT EXAMINATION

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• Pupillary reactions-presence of Marcus Gunn pupil –optic n. compression

• Fundoscopy-venous engorgement,hemorrhage, papilledema or optic atrophy is observed

• Ocular motility-restriction of ocular movements may be caused by restrictive myopathy as in thyroid ophthalmopathy,splinting of optic n. in optic sheath meningioma &neurological deficit resulting from orbital apex lesions.

• Forced duction test- to differentiate defective ocular movements due to neurological lesions from those caused by mechanical obstuction.

• Tonometry-IOP is usually raised in thyrotropic exophthalmos esp. in upward gaze(positional iop changes)

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Exophthalmometry(proptometry)• Worm’s eye view• Standard Hertal’s

exophthalmometer-measures both eyes simultaneously with lateral orbital rim as reference point

• Leudde’s exophthalmometer-measures each eye separately with lateral orbital rim as reference pt.

• Mutch exophthalmometer-measures each eye separately with cheek or brow as reference pt.

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Normal values: 10-21 mmAbsolute reading of >21mm suggests proptosisDifference of >2mm between eyes also indicates proptosis

VEP

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Flash VEPPattern VEP-

full fieldhemi fieldcentral fieldpartial field

Chromatic patterned stimuli-best method of separating red, green , and blue coloured channels. Helpful in detecting colour blindness.

Normal Data:• P 100 LATENCY ( m sec ) = 102 5• Amplitude (μV) =10 4.2• Duration = 63 8.7

Criteria for abnormailtyLATENCY CRITERIA• PROLONGATION > 3 SD • INTEROCULAR LATENCY OF P100>10 msec, LONGER LATENCY ABNORMAL

AMPLITUDE CRITERIA• INTEROCULAR AMPLITUDE RATIO>2• ABNOMALLY LOW OR HIGH AMPLITUDE• ABSENCE OF IDENTIFIABLE VEP FROM

MIDLINE AND LATERAL OCCIPITAL SITES.

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General systemic examination conducted to rule out

proptosis (esp. when b/l) associated with systemic ds such as amylodosis,histiocytosis or wegener’s granulomatosis

Lab investigations complete hemogram,

peripheral blood smear & BM examination

thyroid function tests

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RADIOLOGICAL INVESTIGATIONS:X-ray orbit PA view(caldwell

view) -orbital fractures -calcification inside

tumours- meningioma -phleboliths-varices -erosion of bony walls-

malignancies -paranasal

sinusitis/mucoceleX-ray lateral view

orbital roof fracture,pituitary ds,frontal sinus ds

• X-ray pns (waters’ view) -for visualisation of orbital

floor ant. 2/3&maxillary sinus

-better picture of orbital blow out fractures

X-ray optic foramen view(Rhese view)

comparison of both optic foramen-enlargement of foramina occurs in gliomas, meningiomas,neurofibromas etc.

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Ultrasonography:

• Non-radiational,non-invasive,well tolerated• A-scan-unidimensional image• B-scan-2D picture,better anatomical display. 4

patterns.• C-scan-for visualizing soft tissue of orbit in coronal

plane• USG patterns of pathological lesion depends mainly

on displacement of orbital fat.

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CT SCAN• Most valuable,non-invasive

method in diagnosis of orbital&related lesions---axial&coronal planes

• Size,shape,extent of any orbital mass lesion is seen clearly

• Bony involvement is seen clearly

• PNS pathology is seen clearly

• Main disadv-inability to distinguish b/w pathological soft tissue masses which are radiological isodense

MRI• Superior in evaluating

intracanalicular,chiasmal&post chiasmal extension of tumours

• Added adv of not being hampered by bone&proves to be more sensitive in delineating subtle differences in fat content & hydration of neural tissues

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Carotid angiography Done in selected cases- -suspected vascular shunts

or intracranial vascular anomaly, tumour.

-should be performed in all

cases of pulsating exophthalmos&in cases associated with bruit/thrill

Eg: angiofibroma,carotid cavernous fistula.

Orbital Venography• Limited indications• Invasive• Sup opthalmic vein-= most

consistent landmark.

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HISTOPATHOLOGICAL STUDIES:

FNAC• under direct vision in an obvious mass;CT or USG

guided in retrobulbar mass• D/v-scanty cellular material

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CORE BIOPSY•3 part instrument consists of a trephine,an obturator&a tissue fixator.

Endoscopic biopsy

Incisional Biopsy•Not preferred

Excisional biopsy:• Preferred to incisional biopsy in orbital masses which

are well encapsulated or circumscribed• Anterior orbitotomy:mass in ant part of orbit is

reached either by transcutaneous or transconjunctival approach

• Lateral orbitotomy:mass in post part(retrobulbar)or at apex of orbit

• Transcranial approach :when tumour extends into cranial cavity

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