This slide is simplified approach to case of proptosis
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APPROACH TO A CASE OF PROPTOSIS Presenter: Dr. Laxmi Dhawal Dr. Ritesh Mehta 1 st Year Resident LEIRC
LAYOUT BRIEF ANATOMY INTRODUCTION PATHOPHYSIOLOGY CLASSIFICATION APPROACH ALGORITHM HISTORY CLINICAL AND OCCULAR EXAMINATION INVESTIGATION IMAGING
ANATOMY OF ORBIT Quadrangular , truncated pyramids situated between anterior cranial fossa and maxillary sinuses below. Each orbit is made Of 7 bones:- Frontal Ethmoidal Lacrimal Palatine Maxilla Zygomatic Sphenoid
DIMENSION OF ORBIT Depth:42mm Along Medial Wall,50 mm Along Lateral Wall Base:40mm Width,35 mm Height Intraorbital Width :25 mm Extraorbital Width:100 Mm Volume Each Orbit:29-30 ml
WALLS OF ORBIT
SURGICAL SPACES Subperiosteal Space: potential Space Between Orbital Bones & Periorbita. Extraconal Space: lies Between The Periorbita & The Muscle Cone. Subtenon’s Space:potential Space Between Sclera And Tenon’s Capsule. Sub-arachnoid Space:lies Between Optic Nerve & The Nerve Sheath. Intraconal Space:lies Within Muscle Cone
INTRODUCTION Proptosis: Abnormal Forward Displacement Or Protrusion Of Globe. Anterior Displacement Of Globe By >21mm From Lateral Orbital Rim. >2mm Difference Between Apex Of Cornea and lateral orbital rim of 2 Eyes
Exophthalmos :Abnormal Forward Displacement Or Protrusion Of Globe In The Presence Of Endocrine Disorder
Exorbitism: Angle Between The Lateral Orbital Walls That Is Greater Than 90 ,which May Also Be Associated With Shallow Orbital Depth. Enophthalmos: Retrodisplacement Of Eye Into The Orbit. Pseudo-proptosis:it Is Either The Simulation Of Abnormal Prominence Of The Eye Or A True Asymmetry That Is Not The Result Of Increased Orbital Contents.
PATHOPHYSIOLOGY
CLASSIFICATION On The Basis Of Etiology Infectious Inflammatory Vascular Neoplastic Idiopathic On The Basis Of Onset Acute Subacute Intermittent Chronic
CONT… On The Basis Of Globe Displacement Axial : Caused By Lesion Within The Muscle Cone Such As optic Nerve Tumours , thyroid Eye Disease. Non-axial: Caused By Extraconal Lesions In Which Direction Of Proptosis Is Determined By The Site Of The Lesion .
CONT.. On The Basis Of Laterality Unilateral Bilateral
Infant Children Adult Congenital lesions Orbital cellulitis Thryoid exophthalmos Retinoblastoma Dermoid cyst Orbital cellulitis Capillary hemangioma Capillary hemangioma Trauma Juvenile xanthogranuloma Optic nerve glioma Meningioma Metastatic neuroblastoma Rhabdomyosarcoma Lymphoma Retinoblastoma Histiocytoma Leukemia Cavernous hemangioma Lymphangioma Varices Metastasis Carotid cavernous fistula Tumor extension from adjacent area metastasis
Approach:
History A thorough ocular and medical history It should include: Onset Course Duration of symptoms:pain,altered sensation,diplopia,changes in vision Prior disease( eg TED,sinus disease)and therapy Injury(head or facial) Systemic condition( eg cancer,inflammatory disease) Family history Old photographs for establishing a timeline of the process
7 P’S OF PROPTOSIS 7Ps
Pain Severe pain Infection and inflammation eg orbital cellulitis Orbital abscess Myocysticercosis High flow CCF lymphangioma Acute presentation of TAO Metastatic lesion Moderate pain Idiopathic orbital inflammatory disease Trauma Ruptured dermoid cyst Dull boring pain Bone erosion in neoplasm 1. Pain
Progression: The proptosis may be progressive,static or waxing wanning Rarely intermittent proptosis can be seen in case of dumbbell dermoid(orbital and temporal fossa component) 2. Progression
ABRUPT WITHIN HOURS OVER DAYS TO WEEKS OVER MONTHS TO YEARS BLEEDING IN LYMPHANGIOMA IDIOPATHIC ORBITAL INFLAMMATORY DISEASE DERMOIDS ORBITAL EMPHYSEMA ORBITAL CELLULITIS BENIGN MIXED TUMOURS FRACTURE OF MEDIAL WALL OF ORBIT THROMBOPHLEBITIS NEUROGENIC TUMOURS RETROBULBAR HEMORRHAGE RHABDOMYOSARCOMA CAVERNOUS HEMANGIOMA TRAUMATIC HAEMATOMA THYROID OPHTHALMOPATHY LYMPHOMA RUPTURED DERMOID NEUROBLASTOMA FIBROUS DYSPLASIAS METASTATIC TUMOR OSTEOMA Based on duration :
Proptosis True/ pseudoproptosis Laterality Direction of displacement Axial Non axial 3. Proptosis
Displacement of globe indicates location of mass: Axial:intraconal mass Non-axial: lesion outside muscle cone.Displacement is generally opposite to the location of lesion Axial proptosis Non axial proptosis
Clinical assessment : Assessment of proptosis is first done in primary gaze
Worm’s eye view Examiner looks up from below with patient’s head tilted back
Bird’s eye view/Nafziger’s method:
Plastic ruler method Can measure proptosis from the lateral orbital rim to corneal apex holding ruler parallel to ground Measurement:
Exophthalmometry :- Is the measurement of the anterior posterior position of globe generally from the lateral orbital rim to the anterior surface of the cornea Clinical Radiological Digital photography
Hertel Exophthalmometry Hertel exophthalmometer measures position of globe by calculating the distance from the lateral orbital rim to the surface of the cornea
Steps: Consent Explanation of procedure Ask patient to sit such that examiner sits one arm away with examiner and patient eyes at same level Ask patient to look at the center of your forehead Place Hertel against lateral walls of patient Measure patients left eye with examiner’s right eye Make your view so that 2 red lines on prism are overlapping one another Position of corneal apex in mm noted from scale in prism
Interpretation: Asymmetry >/2mm or more between the eyes Or Protrusion greater than 13-15mm in east Asians 21mm in Caucasian adults 23mm in adult African- americans Mean normal(upper limit) Asian Caucasian African-American Male 15.5 16.5 18.5 Female 13.5 15.4 17.8 Age group Mean normal <4yrs 13.2 5-8yrs 14.4 9-12yrs 15.2 13-17yrs 16.2
Luedde’s Exophthalmometer
Naugle Exophthalmometry :
Sources of error in exophthalmometry : Failure to have patient look straight Failure to have cross bar parallel to floor Parallax error
Radiologic Exophthalmometry Measurement( Trokel and Hilal method): Horizontal line is drawn between lateral orbital rims A perpendicular line is then drawn from apex of the cornea to horizontal line and measured Abnormality: If each perpendicular line >21mm Or If asymmetry >2mm between two
Measurement of dystopia: Two ruler Method Loss of support to the eyeball and it shifts in that direction In non axial proptosis thehorizontal and vertical dystopia is measured
4. Pulsation Case of Carotid Cavernous Fistula Without Bruit With Bruit Neurofibromatosis Carotid-cavernous Fistula Meningoencephalocele Dural Arteriovenous Fistula Encephalocele Orbital Arteriovenous Fistula Result Of Surgical Removal Of Roof
Examination: Auscultation of globe/temporal region
Local temperature Tenderness Orbital margins Retropulsion of globe(simultaneous digital pressure over both eyes with closed lids) Regional Lymph Node(preauricular/cervical/supraclavicular) If Mass present:site,size,shape,surface,depth,color,temperature tenderness,reducibility,fluctuation,translucency,pulsatile,mobile/fixed to underlying structure 5. Palpation
Salmon colored mass Vascular congestion over the insertion of the rectus muscle Corkscrew conjunctival vessels S shaped eyelid Eczematous lesions of the eyelids Ecchymosis of eyelid skin Prominent temple Edematous swelling of lower eyelid Black crusted lesions in nasopharynx Facial asymmetry 6. Periorbital changes
EYELID SIGNS Lid Retraction (90%): Most Common Sign In Thyroid Eye Disease Darlympe Sign : Lid Retraction In Primary Gaze Kocher Sign : Staring And Frightened Appearance Of The Eyes Which Is Particularly Marked On Attentive Fixation. Von Graefe Sign : Retarded Descent Of The Upper Lid On Downgaze. Gifford Sign : Difficulty In Everting Upper Lid Joffroy’s Sign : Absent Creases In Forehead On Superior Gaze. Stellwag Sign : Incomplete And Frequent Blinking. Enroth Sign : Edema Of Lower Eyelid. Griffith’s Sign : Lower Lid Gaze On Upgaze .
7.Photographs Photograph Is Required To Compare Before And After The Onset Of Proptosis Evaluation
Ocular Examination Systemic Examination
Ocular examination: Visual acuity:dimunition d/t optic nerve compression,corneal exposure. Colour vision & contrast sensitivity : colour desaturation is a sensitive feature, brightness appearance is diminished. Extraocular muscle movement : restrictive myopathy. Ptosis is present or not.
IOP:increase in thyroid orbitopathy(d/t restriction of movement),arteriovenous fistula(d/t elevated venous pressure). Iop is measured in 3 gaze( upgaze,straight and down gaze). Braley’s sign:positional IOP change seen in TED Increase of IOP >/6mm Hg:positive result(d/t muscle restriction) Negative result<6mm Hg rise indicated neurological lesion
Systemic Examination Skin pigmentation Café au lait spots Features of hyperthyroidism Cutaneous hemangiomas elsewhere Scalp bony lesion Organomegaly/lump in abdomen ENT examination:Paranasal sinuses and nasopharyngeal mass Neurological evaluation of III IV VI VII CN
Laboratory Studies: Routine blood investigation(TC,DC)+ESR,VDRL etc Thyroid function test Casoni’s test(echinococcosis) Stool examination for cyst and ova of parasite Urine (for Bence Jones protein in Multiple myeloma) Sr.ACE and lysozyme for diagnosis ofsarcoidosis Sr.ANCA assay for granulomatosis with polyangiitis Diagnosis is confirmed by biopsy of one or more affected organ
Imaging modalities: Non invasive:xray,USG,CT,MRI,CT and MR angiography Invasive:arteriography and venography These modalities should be based on patient’s specific condition X ray Plain x-ray Caldwell view,water’s view,lateral view and Rehse view(for optic foramina)
Computed tomography Shape,location,extentand character of lesions in orbit Refining of Differential diagnosis Relationship of lesion with spaces of orbit in case of orbitotomy More bony resolution making it technique of choice for orbital trauma and bony tumors IV contrast agent employed for tumors with increased vascularity(meningioma) or altered vascular permeability
Thyroid Orbitopathy Axial myopia Lacrimal gland mass Carotid cavernous fistula
Capillary hemangioma Retinoblastoma Choroidal osteoma Non specific Orbital Inflammation
Magnetic resonance imaging: Bettter details of: orbitocranial junction Orbitocranial tumors soft tissues of orbital apex Intracanalicular portion of optic nerve Structures in periorbital spaces IV contrast agent in case of vascular lesion
CT and MR Angiography For: AV malformations/fistula Aneurysms Invasive: Arteriography Gold standard for diagnosing arterial lesion-aneurysm and AV malformation Advantage:Both diagnostic and therapeutic Venography Diagnosis and management of orbital varices Study of cavernous sinus
Ultrasonography Determines size,shape and position of normal and abnormal orbital tissue Doppler ultrasonography:can demonstrate retrograde flow in orbital veins cases of dural cavernous fistula/AV malformation or vascular tumors with increased blood flow Limited value in assessing lesion of posterior orbit
Pathology: Plays an utmost role in: Preoperative orbital biopsy to decrease array Diagnosis of tissue obtained from orbitotomy Cell marker study Genetic analysis Types of study generally done are: FNAC Incisional Biopsy Excisional Biopsy Core biopsy
Summary: A good approach(history and examination) is mandatory The most common cause of bilateral proptosis is Graves disease Acute unilateral proptosis suggest infection or vascular disorders( eg hemorrhage,fistula,cavernous sinus thrombsis ) Apply lubricant to exposed cornea to prevent keratopathy The goal is to treat the patient and prevent vision loss
Bibliography: AAO-BSCS orbit,eyelids and lacrimal system 2022-2023 Kanski’s clinical Ophthalmology Ninth Edition Myron Yanoff /Jay S. Duker Ophthalmology Sixth Edition