AN APPROACH TO CASE OF PROPTOSIS

AN APPROACH TO CASE OF PROPTOSISAarti Kerketta

PROPTOSIS

Forward displacement of the eye (H.I.E Saunders.Dorlands 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 )

DEFINITION

Protrusion of the globe secondary to non-endocrine causes (Henderson JW. Orbital Tumors. 3rd ed. New York: Raven Press(1994)

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By Hertels Exophthalmometer

Distance between lateral orbital margin and corneal apex greater than:13 to 15mm in Asians21 mm adult Caucasians23 mm adult African- American

On CT scansGlobe protrusion > 21 mm anterior to the inter-zygomatic line on midaxial scans at the level of the lens. Asymmetry between the two sides >2mm

Sarinnapakoran V et al,Proptosis in normal Thai samples and thyroid patients.J Med Ass Thai 2007;90(4):679-83BeugerDG et al,Proptosis In: Ophthalmic secrets.Vander JF, GualtJA, Philadelpia,Pennsylvania,2002Chapter 36,p269Naik MN et al, Interpretation of computed tomography imaging of eye and orbit.A systemic approach.Indian JOphthalmology 2002;50(4):339-53

Lateral orbital rim to the corneal apex measures14 to 21 mm in adultsProtrusion greater than 21 mm or a 2mm difference is generally abnormal

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Orbit is pyramidal shaped cavity bounded by four OPEN anteriorly

Contents-

Surrounded by

Volume of orbit - 30 cc

PATHOPHYSIOLOGYSINUSESGLOBE,MUSCLES FASCIA,VESSELS,NERVES,FAT,LACRIMAL GLAND,LACRIMAL SAC CRANIUMBONY WALLS

EFFECT OF PROPTOSIS

COSMETIC

FUNCTIONALDISABILITY

SYMPTOMS

Forward protrusion or displacement of eyeVisual disturbances- diminution of vision, diplopiaDiscomfort- grittiness, watering, painDifficulty in closing eyes fully while sleeping or blinkingIncrease in visible white part of eye

PROMINENT APPEARING GLOBE

PSEUDOPROPTOSIS

ENLARGED GLOBEEXTRAOCULAR MUSCLE WEAKNESS CONTRALATERALENOPHTHALMOSASYMMETRIC PALPEBRAL FISSURESHIGH AXIAL MYOPIABUPHTHALMOS

IPSILATERAL EYELID RETRACTIONCONTRALATERAL PTOSIS

TRUE PROPTOSISHistory and physical examination

SHALLOW ORBITNORMAL ORBIT

CRANIOFACIAL DYSOSTOSISTRAUMA

ENDOCRINAL DISEASEVASCULARMALFORMATIONINFLAMMATORYNEOPLASIA

INFECTIOUSORBITAL CELLULITISCAVERNOUS SINUS THROMBOSISPARASITIC SINUS DISEASENON INFECTIOUS

MYOSITISIDIOPATHIC ORBITAL EYE DISEASE

SECONDARYPRIMARYMALIGNANTBENIGN

CAROTID-CAVERNOUS FISTULAARTERIOVENOUS (A-V) MALFORMATIONORBITAL VARIXTHYROID EYE DISEASE(TED)

HOW TO APPROACH A CASE OF PROPTOSIS

HISTORY EXAMINATIONINVESTIGATION

HISTORY

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:

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AGE OF ONSET

AT BIRTHCHILDHOOD/ YOUNG ADULTMIDDLE AGED/ ELDERLY

UNILATERALBILATERAL

CRANIOFACIAL DYSOSTOSISCROUZONS SYNDROMEAPERTS SYNDROME

INTERMITENT/PULSATILENON PULSATILECEPHALOCELEA-V MALFORMATIONS

ORBITAL TUMORS

Vascular -Capillary hemangiomaTeratoma

BIRTH TRAUMA

CHILDHOOD/ YOUNG ADULTMIDDLE AGED/ ELDERLYAGE OF ONSET

PROGRESSIONACUTE(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

PULSATILEh/o Trauma,surgery (headache,tinnius,diplopia)

NON PULSATILE(h/o trauma)A-V FISTULA

RETROBULBAR HEMORRHAGE

SUDDEN SPURT IN PREEXISTING PROPTOSISBleed in vascular tumorsRupture dermoid or parasitic cystLymphangioma

CHILDHOOD/ YOUNG ADULTMIDDLE AGED/ ELDERLY

PROGRESSION

ACUTE(hours to a week)

SUB-ACUTE(1-4 weeks)

CHRONIC(>6 MONTHS)

PAINFUL

PAINLESS(+/- DOV , diplopia, redness)

UNILATERALBILATERAL

INFLAMMARTORYNON-INFLAMMATORYORBITAL CELLULITIS(fever)(Diplopia)MYOSITISTEDIOID (intermittent/recurrent)(Swelling,redness ,+/- DOV)MALIGNANTMETASTASIC TUMORCHILDHOOD/ YOUNG ADULTPRIMARY-RhabdomyosarcomaSECONDARY Retinoblastoma, sinus tumorMETASTATIC-Wilms tumour,Neuroblastoma,AML

MIDDLE AGED/ ELDERLYMETASTATIC-lung, breast, prostrateSECONDARY Intracranial tumor, sinus tumor

INFLAMMARTORY+NON-INFLAMMATORY+/-(Swelling,redness +/-DOV)

CAVERNOUS SINUS THROMBOSIS(fever ,headache,vomiting)

METASTATIC TUMOR

IDIOPATHIC ORBITAL INFLAMMATORY DISEASE

MALIGNANT TUMOR

UNILATERALBILATERAL

MIDDLE AGE/ ELDERLYTHYROID EYE DISEASE(diplopia,grittiness,watering)

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CHILDHOOD/ YOUNG ADULTMIDDLE AGED/ ELDERLY

PROGRESSION

ACUTE(hours to a week)

SUB-ACUTE(1-4 weeks)

CHRONIC(>6 MONTHS)

PAINFULPAINLESS

IDIOPATHIC INFLAMMATORY ORBITAL DISEASE(variable/recurrent)TED(diplopia)

(Corneal dryness and exposure ,with DOV )

UNILATERALBILATERAL

LARGE TUMOR/ CYSTWITH DOVWITHOUT DOV

PRECEEDING PROPTOSISOPTIC NERVE GLIOMA(children)

FOLLOWING PROPTOSIS OPTIC NERVE MENINGIOMA

BENIGN TUMOR

BENIGN TUMOR

CYSTParasiticDermoid(children,young adult)

Capillary hemangioma(children)Cavernous hemangioma (adults)Lymphangioma(intermittent)

VASCULARTUMOR

OTHERNeurofibroma(pulsatile)SchwannomaBone tumorLacrima gland tumorLymphoma(bilateral)

History of systemic / extra-ocular disease

Diabetes, immunocompromised statusSinus , dental and ENT diseasesPrior nasal surgeryPrevious or current malignancyThyroid dysfunction Trauma

EXAMINATION

INSPECTIONPALPATIONMEASUREMENT OF PROPTOSISOCULAR EXAMINATION

INSPECTION

Compensatory head postureFacial scars, deformity Ocular symmetry and position of eyeAdnexal structureSurface of eye Pulsations, valsalva maneuver Movements

Attentive gaze (Kochers sign)Infrequent blinking (Stellwags sign)

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POSITION OF EYE

Forward protrusion without displacement AXIAL PROPTOSIS

Displacement along with proptosis ABAXIAL PROPTOSIS

Axial- lesion arises from within the muscle cone (intra-conal)

Non-axial-lesion arises from without the muscle cone (extra-conal)

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AXIAL PROPTOSIS Lesions of intraconal spaceOptic nerve gliomaOptic nerve sheath meningiomaCvernous hemangioma, Orbital varixSchwannomaNeurofibromaCystic lesionThyroid associated orbitopathy Idiopathic orbital inflammationABAXIAL PROPTOSISDOWN AND IN

Orbital mass of superotemporal quadrant

Lacrimal gland tumorDermoidOther 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 sinusLacrimal sac tumorsLethal midline granulomaNasopharyngeal tumor

UPWARD DISPLACEMENT

Mass from maxillary sinusNeoplasiaFungal granulomaDumbbell dermoid

Cyst or tumor of inferior quadrant

Measure Proptosis 1.Nafzigers Method2.Clear plastic ruler3. Lueddes exophthalmometer4. Hertels exophthalmometer5.Naugles exophthalmometer6.Measure dystopia (2 scale)

Stands behind the patient looks over forehead Raise patients 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

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INSPECTION OF ADNEXAL STRUCTURES

SURFACE

LID POSITION MASSEYELIDS AND PERIBULBAR TISSUE

COLOUREDEMASINUS OR FISTULA

SITENUMBERSIZESURFACEMARGINSEXTENTCOMPONENT LID AND ORBITAL

PTOSISLID RETRACTIONLID LAG

REDNESS Infection and inflammationThyroid eye diseaseMalignant and metastatic neoplasm

EDEMA

Reduced venous and lynphatic drainage by massBluish discoloration- vascular tumor or malformation ,traumaSinus and fistula- Orbital abcess, dermoids, mucocele

LID POSITIONPTOSISLID RETRACTIONLID LAG

MechanicalParalytic

Thyroid eye diseaseLarge orbital neoplasmThyroid eye disease

S shaped lid thickening - Plexiform neurofibroma

Pulsation direct lateral viewArteriovenous malformationCephalocele, large mucoceleNeurofibromatosis

Valsalva maneuverIncrease in proptosis with valsalva AV malformationsOrbital varix

OCULAR MOVEMENTS

Limitation of ocular motility due to orbital mass

Restriction due to invasive process in muscle

Paralytic

Thyroid associated orbitopathy, Idiopathic orbital inflammation, myocysticercosis ,fungal granulomaParalytic- Carotico cavernous fistula

Limitation of motility following trauma- soft tissue edema , entrapment of muscle in orbital fracture or injury to muscle itself

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CONJUNCTIVADilated vascular channel at the canthus with chemosis- dysthyroid disease Epibulbar dark-red corkscrew vessels increased venous pressure, AV malformationDiffuse conjunctival congestion- orbital inflammatory or infectious diseaseSectoral congestion and chemosis- MyositisANTERIOR SEGMENT EXAMINATION Subconjunctival haemorrhage- Trauma,infiltrative tumorsChemosis- inflammation,lymphatic obstructionSalmon patch- lymphomaCORNEA-exposureIris- Lisch nodules inneurofibromatosis

EXAMINATION OF NASAL CAVITY AND ORAL CAVITY IS MANDATORY IN PRESENCE OF PARANASAL SINUS INVOLVEMENT

PALPATION

Examination..... Local Temperature Tenderness Orbital tonometry, orbital margins If mass palpable note PositionSize,surface, attachnentsConsistency(hard , rubbery, spongy or soft)Compressibility/ Reducibility

Tenderness

Orbital infection and inflammation

Adenoid cystic carcinoma

Trauma

TemperatureRise in temperature of overlying skin seen in orbital infection and inflammation

Consistency of the palpable mass

Compressibility - characteristic of Cystic mass

Reducibility- characteristic of mass communicating with neighbouring cavityCYSTICSOFTFIRMRUBBERYHARD

TRANSILLUMINATION (fluid / air filled)

AUSCULTATION Bruits- High flow arteriovenous fistula

OCULAR EXAMINATIONVISUAL ACUITYPUPILLARY REACTIONCOLOR VISIONREFRACTIONVISUAL FIELDOPHTHALMOSCOPYINTRA OCULAR PRESSURE

Documentation of visual acuity is necessary Diagnostic and aids in planning management

PUPILSVISUAL ACUITY

Look carefully for RAPD suggestive of optic nerve damage

COLOUR VISION

In early 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

If colour vision is defective evaluate carefully- Pupil for RAPD

Do detailed fundus examination for

Presence of disc edema,pallor,optocilliary shunt,retinal detachment

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RAPD, COLOR VISION ABNORMALITIES AND VISUAL FIELD DEFECTS CAN DETECT COMPROMISE OF THE OPTIC NERVE EVEN WHEN THE VISUAL ACUITY IS NORMAL

Optic discs edema, pallor, atrophy

Choroidal folds - tumours, dysthyroid ophthalmopathy, inflammatory lesions

FUNDUS EXAMINATION

It is important to note that in some cases choroidal folds may precede the development of proptosis.36

Retinal vascular changes-Opticociliary shunt 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

GENERAL EXAMINATION

Look for evidence of malignancySigns of thyroid dysfunction - thyromegaly, tachycardia, tremors of the hand etc.Sites of entry for infection -sinus disease, nasal and oral infectionLymphadenopathy

ON THE BASIS OF HISTORY AND EXAMINATION

DIFFERENTIAL DIAGNOSISSHOULD BE MADE

IMAGING

SPECIAL INVESTIGATIONS

IMPORTANCETo know exact location and extent of lesionPredict nature of lesion (tumor or cyst,encapsulated/infiltrating,Vascular/nonvascular,benign /malignant/metastatic) Plan proper management strategy

Various imaging modalities in orbital disease

X-RayNon -contrast and contrast Computed TomographyMagnetic Resonance ImagingUltrasound

CT and MRI have largely replaced radiographySkull X-ray are now performed only in selective cases offacial fracture

THE CHOICE OF IMAGING STUDY SHOULD BE BASED ON CLINICAL PRESENTATION AND THE SPECIFIC PATHOLOGY BEING SUSPECTED

FINE NEEDLE ASPIRATION CYTOLOGY AND BIOPSY

IN CASE OF DOUBTAIDS IN ESHTABLISHING THE DIAGNOSIS

PLAN MANAGEMENTBiopsy is indicated for histopathological confirmation of clinical diagnosis

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.

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DECISION MAKING GOALS FOR MANAGEMENT

Prevent life threatning condition Preservation of visual functionAlleviation of pain Cosmesis

MANAGEMENT OPTIONS OF PROPTOSIS

INFLAMMATORYNON-INFLAMMATORYOTHERS

INFECTIOUSNON-INFECTIOUS(TED, IOID)

NON-SURGICALSURGICALSurgical drainage of abcessEnucleation/Exentration

SystemicAntibioticsAntifungal

ANALGESIC

NON-SURGICALSURGICALNon steroid anti inflammatorySteroidImmunosuppressentRadiotherapy

Surgical decompressionEOM surgeryLid surgery

BENIGNMALIGNANTExentrationExcisionNON-SURGICALSURGICAL

ObservationIntralesional/ systemic steroidCapillary hemangioma

Excision of mass NON-SURGICALSURGICAL

RadiotherapyChemotherapy

TRAUMAA-V MALFORMATIONMedical management of intraocular pressureClosure of fistula

Medical management of intraocular pressureSurgical decompression,explorationFracture repair

EVALUATION AND MANAGEMENT OF PROPTOSIS REQUIRES A MULTIDISCIPILINARY APPROACHIF NEEDED ALWAYS TAKE OPINION OF OTOLARYNGIOLOGIST, NEUROSURGEON, ONCOLOGIST

VISUAL FIELDLarge orbital tumor with pressure on eye produceIrregular quadrantic contractions Meningioma- peripheral field affected earlier than centralGlioma- Scotoma dispropotionately greater than proptosis