PROPTOSIS: How to approach? History, clinical examination, investigations and differntial diagnosis
8/12/2011
1
DEFINITION:
PROPTOSIS: Forward displacement of bulging especially that of eye. Abnormal protrusion of the globe beyond the orbital margins margins with the patient looking straight ahead Word EXOPHTH OPHTHALMOS ALMOS sy synon nonymous ymous but is more specific for the. eye
8/12/2011
2
Causes
of pseudoproptosis:
Simulation of abnormal protrusion of the eye or a true abnormal protrusion of eye or a true abnormal protrusion that doesnt originate from a mass, inflammation or a vascular disorder 1.u/l high axial myopia 2.u/l congenital glaucoma 3.u/l secondary glaucoma resulting from ocular trauma during childhood 4.shallow c/l orbit as in crouzons ds(craniosynostosis) 5.hypoplastic supra-orbital ridges as in trisomy18 6.assymetry of body of orbits 7.facial asymmetry 8. Lid retraction, ptosis or enopthalmos. 8/12/2011
3
CLASSIFICATIONS Etiology dysthyroid orbitopathy inflammatory tumours & cysts Laterality unilateral bilateral Direction. Axial non-axial
8/12/2011
Time of onset childhood-congenital acquired adulthood Duration Acute Subacute chronic Clinical course Stationary Progressive Regressive Pulsating Intermittent positional 4
CAUSES
OF PROPTOSIS:
Inflammation
Acute-orbital cellulitis Chronic(nongranulomatous)-
pseudotumour
Chronic(granulamatous)
TB,sarcoid,syphilis,parasites,Aspergill osis Benign lymphoepithelial lesion (Mikuliczs ds)
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
Injuries
-foreign body -orbital hemorrhage
8/12/2011
5
TUMOURS PRIMARY 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
SECONDARY
1.Direct extension froma.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
n.Hand-Schuller-Christian ds o.Juvenile xanthogranuloma
8/12/2011
6
EVALUATION OF
THE PATIENT
HISTORY -Age of onset -nature of onset -duration -progression -symptoms -associated symptoms/systemic symptoms 8/12/2011
7
AGE OF ONSET: NEWBORN -orbital sepsis -orbital neoplasm NEONATAL -infections of maxilla EARLY CHILDHOOD(upto
1 yr)
1-5 Yrs OF AGE -dermoid -orbital extension of retinoblastoma -hemangioma -metastatic neuroblastoma -glioma of optic n.
-dermoid -hemangioma -orbital extension of retinoblastoma -Hand-Schuller- Christian ds
8/12/2011
8
Young Adult -pseudotumour -thyroid ophthalmopathy -mucocele -meningioma -fibrous dysplasia -osteoma -undifferentiated sarcoma -lacrimal gland tumour 8/12/2011
Old age: -pseudotumour -sino-orbital mucocele -malignant lymphomas& leukemias -meningioma -ca of palpebral or epibulbar region -metastatic ca
9
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
8/12/2011
10
PROGRESSION: Continuous progression -tumours & endocrinal exophthalmos
Intermittent
proptosis -orbital varices -recurrent hemorrhage -vascular neoplasm -lymphangioma
8/12/2011
Variable
pseudotumours&angiomas
Pulsating
-carotidocavernous aneurysm -large frontal mucocele -meningoencephalocele -blow out fracture of roof of orbit
11
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 EXAMINATION VISUAL ACUITYprovides 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.
8/12/2011
12
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,ewings sarcoma,leukemia, eosinophilic granuloma,lymphangioma,traumatic hematoma 8/12/2011
13
Conjunctiva
Direction of displacement
-dysthyroid orbitopathy-hyperemia near insertions of recti muscles
Ethmoidal sinus
-orbital vascular malformation or caroticocavernous fistuladilated,slightly tortuous larger vessels that extend to corneoscleral limbus
Mass in lacrimal fossa-downward & nasal displacement
-idiopathic orbital inflammationmarked diffuse injection of smaller conjunctival &episcleral blood vessels
8/12/2011
mucocele displaces globe laterally
Axial proptosis-mass inside muscle cone eg.optic n. glioma ,meningioma, graves Maxillary sinus growth-superior displacement
14
Differential Diagnosis: Causes of u/l proptosis
Causes of b/l proptosis
etc.
anomalies of skullcraniofacial dysostosis eg. Oxycephaly(tower skull)
Traumatic -orbital hemorrhage,traumatic aneurysm,foreign body etc.
Osteopathiesosteitis deformans, rickets,acromegaly
Congenital-dermoid
cyst,orbital teratoma
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
8/12/2011
Developmental
Inflammatory conditions-Mikuliczs syndrome,late stage of cavernous sinus thrombosis Endocrinal
exophthalmos-thyrotoxic or thyrotropic Tumourslymphoma,lymphosarcoma,secondaries Systemic ds-histiocytosis,systemic amyloidosis,xathomatosis&wegeners granulomatosis
15
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 8/12/2011
16
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)
8/12/2011
17
Exophthalmometry(proptometry) Worms
eye view Standard Hertals exophthalmometer-measures both eyes simultaneously with lateral orbital rim as reference point Leuddes exophthalmometermeasures each eye separately with lateral orbital rim as reference pt. Mutch exophthalmometermeasures each eye separately with cheek or brow as reference pt. Normal values: 10-21 mm Absolute reading of >21mm suggests proptosis Difference of >2mm between eyes also indicates proptosis 8/12/2011
18
VEP
Flash VEP Pattern VEPfull field hemi field central field partial field Chromatic patterned stimuli-best method of separating red, green , and blue coloured channels. Helpful in detecting colour blindness. 8/12/2011
19
Normal Data: P 100 LATENCY ( m sec ) = 102 Amplitude (V) =10 s 4.2 Duration = 63 s 8.7
s
5
Criteria for abnormailty LATENCY 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.
8/12/2011
20
General systemic examination
Lab investigations
conducted to rule out complete hemogram, proptosis (esp. when b/l) peripheral blood smear & associated with systemic BM examination ds such as thyroid function tests amylodosis,histiocytosis or wegeners granulomatosis
8/12/2011
21
RADIOLOGICAL INVESTIGATIONS: X-ray orbit PA view(caldwell
view) -orbital fractures -calcification inside tumours- meningioma -phleboliths-varices -erosion of bony wallsmalignancies -paranasal sinusitis/mucocele X-ray lateral view orbital roof fracture,pituitary ds,frontal sinus ds 8/12/2011
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,neurofibroma s etc.
22
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.
8/12/2011
23
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
8/12/2011
MRI
Superior in evaluating intracanalicular,chiasmal&p ost 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
24
Carotid angiography Done in selected cases-suspected vascular shunts or intracranial vascular anomaly, tumour.
Orbital Venography Limited indications Invasive Sup opthalmic vein-= most consistent landmark.
-should be performed in all cases of pulsating exophthalmos&in cases associated with bruit/thrill Eg: angiofibroma,carotid cavernous fistula.
8/12/2011
25
HISTOPATHOLOGI CAL STUDIES: FNAC
under direct vision in an obvious mass; CT or USG guided in retrobulbar mass D/v-scanty cellular material CORE BIOPSY 3 part instrument consists of a trephine,an obturator&a tissue fixator. Endoscopic biopsy Incisional Biopsy Not 8/12/2011
preferred 26
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
8/12/2011
27