Diplopia strabismus ppt-talk_10-2010

DOUBLE VISION, ACQUIRED, and

CONGENITAL STRABISMUSRobert Cordero, M.D., F.A.C.S

Central Florida Eye Specialists, P.L.

October 30, 2010

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• www.theeyespecialists.com• www.aapos• www.aao• Google

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New, Updated, Revised, Better• potions, purification, diet, lancets

• Hammurabi’s Code 1800 B.C.

• Egyptian Papyrus 1600 - 1300 B.C.

• Susruta father of Hindu surgery

• Hebrew Talmud 1300 B.C.- 70 A.D.

• Greece, Alexandria, and Rome

• Middle Ages (395-1492) and Renaissance 14-16th century

Georg Bartisch (1535-1606)

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Chevalier John Taylor 1739

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First Muscle Operations

• 1838 Stromeyer, an Orthopedic surgeon on a cadaver Hanover, Germany

• 1839 J.F. Diffenbach in Berlin, by 1842 had done 1,200 strabismus operations, which consisted of tenotomy of the medial rectus muscle

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Eye Massager 1890

Double Vision=/=Diplopia

• Diplopia = Seeing one object as two. Must rule out Vertigo, Syncope, Hysteria, etc.

• Double the Time

• Double the Effort

• Double the Cost

• Double the reward

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Questions to Ask

• Does the DV go away by covering one eye

• Abnormal head posture seen in old photos

• Pain, facial numbness, circulatory issues

• Eyelid malposition and endocrine disease

• Fatigability, dysarthria, dysphagia, SOB

• FH, Meds, MH, SH, smoking, ETOH, etc

• Compliance and socioeconomic

Work-up Diplopia

• History, History, Histroy

• Old Records and Studies

• Compare Measurements

• Impression(s)

• Plan: Short and Long Term

• Communication

Tools of the Trade

• Patience, Efficiency and Insight

• Lensometer readings

• Best Corrected Vision, Pinhole

• Stereopsis

• Prism Bars, Occluder, Pupil Light

• Clip-ons, Trial Set, Cycloplegia

Basic Review

• Anatomy

• Eye Movements

• Binocular Vision

• Strabismus

• Motor Evaluation

• Sensory Evaluation

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SENSORY

• Simultaneous perception- brains ability to perceive images from OU at same time

• Suppression-brain shuts off information• Fusion- cortical integration of separate retinal

images into single sensory perception• Stereopsis- to perceive the relative distance of

objects•

Tests for stereopsisTitmus

• Red-green spectacles

TNO random dot test

• ‘Hidden’ shapes seen • Polaroid spectacles• Figures seen in 3-D

Lang

• No spectacles

Frisby

• ‘Hidden’ circle seen

• No spectacles• Shapes seen

Tests for sensory anomaliesWorth four-dot test

a - Prior to use of glassesb - Normal or ARCc - Left suppression

Bagolini striated glasses

a - Normal or ARCb- Diplopiac - Suppression

d - Right suppressione - Diplopia

d - Small suppression scotoma

Synoptophore

• Measurement of fusional amplitudes

• Detection of suppression and ARC• Measurement of angle

• Grading of binocular vision

Hirschberg test• Rough measure of deviation

• Note location of corneal light reflex

Reflex at border of pupil = 15 Reflex at limbus = 75

• 1 mm = 7 or 15

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Motility tests• Tests versions and ductions• Grades under/overaction

Left inferior oblique overaction Left lateral rectus underaction

Cover tests

• Cover test detects heterotropia • Prism cover test measures tropia• Alternate measures total tropia and phoria

• Alternate cover test detects total deviation

• Uncover test detects heterophoria

Amblyopia

• Strabismus

• Anisometropic (sph or cyl) > 1.5 D

• Deprivation (media opacity >1 mm in size

• or ptosis < 1 mm margin reflex distance)

• Cost Effectiveness Tx gain from $2053 to

• $2509 ($/ QALY) <20K especially good

• www:aao.org/ppp cost-utility analysis

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The Pediatric Eye Disease Investigator Group (PEDIG)

• Randomized multicenter clinical study• Patching regimens part vs full time• 2 hr/day for moderate cases(20/40-80)• Atropine 1% vs patching(6hr/day) ages 3- 7• 24% recurrence < 8 years age within one year

cessation either method• 27% improvement age 3-7 anisometropia Rx• 50% >2 lines age 7 to 12 either method, age

13 -17 47% benefit no prior vs 16% prior Tx

Types of Turns

• ESODEVIATION

• EXODEVIATION

• A and V Patterns

• Cyclovertical

Monocular Diplopia

Signs of chalazion (meibomian cyst)

Painless, roundish, firm lesion within tarsal plate

May rupture through conjunctiva and cause granuloma

Morphological classification of keratoconus

Nipple cone Oval cone Globus cone

Small and steep curvature Larger and ellipsoidal Largest

Progressive iris atrophy

Progressive stromal iris atrophy

Broad-based PAS Displacement of pupil towards PAS

Isolated familial ectopia lentisAutosomal recessive

Pupil may be normal Pupil may be displaced in opposite direction (ectopia lentis et pupillae)

Classification according to maturity

Immature Mature

Hypermature Morgagnian

Implant displacementDecentration

• May occur if one haptic is inserted into sulcus and other into bag

• Reposition may be necessary

• Remove and replace if severe

Optic capture

Binocular Diplopia

No Misalignment

INCOMITANT

Mechanical (Restrictive) Diplopia

• Grave’s Ophthalmopathy

• Brown’s Syndrome

• Orbital Pseudotumor

• Ocular Myositis

• Orbital Mass Lesions

• Orbital Trauma

Signs of eyelid retraction Occurs in about 50%

• Bilateral lid retraction • No associated proptosis

• Bilateral lid retraction • Bilateral proptosis

• Lid lag in downgaze • Unilateral lid retraction • Unilateral proptosis

• Occurs in about 40% • Due to fibrotic contracture

Restrictive myopathy

Elevation defect - most common Abduction defect - less common

Depression defect - uncommon Adduction defect - rare

Optic neuropathy• Occurs in about 5% • Early defective colour vision • Usually normal disc appearance

Caused by optic nerve compression at orbital apex by enlarged recti

Often occurs in absence of significant proptosis

Brown syndrome (right)

Normal elevation in abduction

Straight in primary position Limited elevation in adduction

Idiopathic orbital inflammatory disease (IOID)

• Usually unilateral• Periorbital swelling and chemosis• Proptosis• Ophthalmoplegia

• Non-neoplastic, non-infectious orbital lesion (pseudotumour)• Involves any or all soft-tissue components

• Presentation - 20 to 50 years with abrupt painful onset

Clinical course and treatment of IOID1. Early spontaneous remission without sequelae

2. Prolonged intermittent activity with eventual remission

Treatment - nil

3. Severe prolonged activity causing a ‘frozen orbit’

Treatment options - steroids, radiotherapy or cytotoxics

Left involvement resulting in ophthalmoplegia and ptosis

Orbital myositis

• Underaction of left lateral rectus

• Clinical course is usually short - treat with NSAIDs

• Subtype of IOID• Involvement of one or more extraocular muscles

• Presentation - sudden onset of pain on ocular movement

• Worsening of pain on attempted left gaze

• CT shows fusiform enlargement of left lateral rectus

Cavernous haemangioma

• Presents - 4th to 5th decade

Slowly progressive axial proptosis

• Most common benign orbital tumour in adults• Usually located just behind globe• Female preponderance - 70%

May cause choroidal folds

Treatment - surgical excision

Pleomorphic Lacrimal Gland Adenoma

• Painless and very slow- growing, smooth mass in lacrimal fossa• Inferonasal globe displacement

• Posterior extension may cause proptosis and ophthalmoplegia

• Smooth, encapsulated outline• Excavation of lacrimal gland fossa without destruction

Presents - 4th to 5th decade

Lacrimal gland carcinoma

• Biopsy

• Painful, fast-growing mass in lacrimal fossa• Infero-nasal globe displacement

• Posterior extension may cause proptosis, ophthalmoplegia and episcleral congestion• Trigeminal hypoaesthesia in 25%

Management

• Radical surgery and radiotherapy

• Presents - 4th to 6th decades• Very poor prognosis

Optic nerve glioma

• Presents - end of first decade with gradual visual loss

• Observation - no growth, good vision and good cosmesis• Excision - poor vision and poor cosmesis• Radiotherapy - intracranial extension

• Typically affects young girls• Associated neurofibromatosis -1 is common

Gradually progressive proptosis Optic atrophy

Treatment

Sphenoidal ridge meningioma

Presents with gradual visual loss and reactive hyperostosis

Fullness in temporal fossa Hyperostosis on plain x-rayProptosis

LymphomaPresents - 6th to 8th decades

Affects any part of orbit and may be bilateral

Anterior lesions are rubbery on palpitation

Treatment

May be confined to lacrimal glands

• Radiotherapy - localized lesions• Chemotherapy - disseminated disease

Direct carotid-cavernous fistula

• Head trauma - most common• Spontaneous rupture - in hypertensive females

Causes

• Ptosis, chemosis and conjunctival injection• Ophthalmoplegia• Raised intraocular pressure

• Defect in intracavernous part of internal carotid• Rapid flow shunt

Direct carotid-cavernous fistula

• Pulsatile proptosis with bruit and thrill• Abolished by ipsilateral carotid compression

• Retinal venous congestion and haemorrhages

• Dilated episcleral vessels• Raised intraocular pressure with wide pulsation

Causes

• Occasional ophthalmoplegia and mild proptosis

Indirect carotid-cavernous fistula (dural shunt)• Indirect communication between meningeal branches of internal or external carotids and cavernous sinus• Slow flow shunt

• Congenital malformations• Spontaneous rupture

Incomitant Misalignment

Tensilon Test

• Tensilon (Edrophonium HCL) 10 mg/ml fast acting anti-cholinesterase

• Neostigmine (Prostigmin) IM (0.02mg/kg) alternative

• Have injectable Atropine Sulfate ready

Ophthalmic Signs of Myasthenia Gravis

• Ptosis

• EOM Palsies

• Pseudogaze Palsies

• Pseudointernuclear Ophthalmoplegia

• Pseudoconvergence Paresis

• Lid Twitch

• Quiver Movements

• Orbicularis Weakness

• Nystagmus

Myasthenia Gravis

• Uncommon, typically affects young women1. Clinical features

•ICE Test•Tensilon test (edrophonium) or Prostigmine

2. Investigations

• Medical - AChE inhibitor, steroids, immunomodulators3. Treatment options

• Weakness and fatiguability of voluntary musculature• Types: Neonatal, Congenital, Ocular, System

• Antibodies to acetylcholine receptors 3 types, MuSK (muscle-specific receptor tyrosine kinase)• CT or MRI for presence of thymoma

• Thymectomy, prisms, strabismus surgery

Ocular myasthenia

• Insidious, bilateral but asymmetrical• Worse with fatigue and in upgaze

Ptosis

• Ptotic lid may show ‘twitch’ and ‘hop’ signs

• Intermittent and usually vertical

Diplopia

Edrophonium test

• Measure amount of ptosis or diplopia before injection

• Inject i.v. atropine 0.3 mg

• Inject i.v. test dose of edrophonium (0.2 ml-2 mg)• Inject remaining (0.8 ml-8 mg) if no hypersensitivity

Before injection Positive result

Cranial Neuropathy

• Oculomotor (3rd) Cranial Nerve

• Trochlear (4th) Cranial Nerve

• Abducen (6th) Cranial Nerve

• Adult, Child, TITS

Anatomy of third nerveOculomotor nucleus

Pituitary gland

Carotid artery

Cavernous sinus

III nerveClivus

Basilar artery

Post cerebral artery

Red nucleus

Pons

Applied anatomy of pupillomotor nerve fibres

Blood vessels on pia mater supply surface of the nerve including pupillary

fibres ( damaged by compressive lesions )

Vasa nervorum supply partof nerve but not pupillaryfibres ( damaged by medicallesions )

Pupillary fibres lie dorsal and peripheral

Signs of right third nerve palsy

• Ptosis, mydriasis and cycloplegia• Abduction in primary position

• Limited depression • Limited adduction

• Normal abduction

• Limited elevation

• Intorsion on attempted downgaze

Important causes of isolated third nerve palsy

Idiopathic - about 25%

Vascular disease - hypertension, diabetesPosterior communicating aneurysmTrauma

Extraduralhaematoma

Prolapsingtemporallobe

Edge oftentorium

Aneurysm

Chiasm

Third nerve

Posterior cerebralartery

Midbrainpushedacross

Anatomy of fourth nerve

• Only cranial nerve to emerge dorsally• Crossed cranial nerve• Very long and slender

Internal carotid artery

Postr. communicating artery

IIIVI

Postr.cerebral arterySupr.cerebellar artery

Basilar arteryIV

Signs of right fourth nerve palsy

• Right overaction on left gaze

• Right underaction on depression in adduction • Vertical diplopia

• Right hyperdeviation in primary position when left eye fixating• Excyclotorsion

Positive Bielschowsky test in right fourth nerve palsy

Absence of right hyperdeviation on contralateral head tilt

Increase in right hyperdeviation on ipsilateral head tilt

Anatomy of sixth nerve

Basilar artery

Pituitary gland

Carotid artery

Cavernous sinus

VI nerve

Petroclinoidligament

Clivus

Pyramidal tract

Vestibularnucleus

Mediallemniscus

4th ventricle

Old right sixth nerve palsy

Straight in primary position due to partial recovery

Limitation of right abduction and horizontal diplopia

Normal right adduction

Important causes of isolated sixth nerve palsyVascular - hypertension, diabetes

Acoustic neuromaRaised intracranial pressure

Dilated ventricles

Petroustip

Brainstem pushed downwards

SUPRANUCLEAR DISORDERS OF EYE MOVEMENT

1. Horizontal gaze palsies

2. Vertical gaze palsies

• Internuclear ophthalmoplegia• Combined internuclear and PPRF (‘one-and-a-half syndrome’)

• Parinaud dorsal midbrain syndrome• Progressive supranuclear palsy

MLF

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Internuclear ophthalmoplegia

• Demylination - usually bilateral • Vascular disease

Important causes

• Tumours of brainstem

Defective left adduction and ataxic nystagmus of right eye

Normal left gaze

Convergence intact if lesion discrete

Lesion involving left MLF

‘One-and-a-half syndrome ’

Paralytic Pontine Exotropia

• Ipsilateral (left) gaze palsy • Defective left adduction• Normal right abduction with ataxic nystagmus

Combined lesion of left MLF and PPRF

Parinaud dorsal midbrain syndrome

• In young adults: demylination, trauma and a-v malformations

• In children: aqueduct stenosis, meningitis and pinealoma

• Supranuclear upgaze palsy

• Large pupils with light-near dissociation

• Lid retracton (Collier sign)

Important causes

• Normal downgaze

• Convergence weakness

• Convergence-retraction nystagmus

• In elderly: vascular accidents and posterior fossa aneurysms

Progressive supranuclear palsy

• Affects elderly

Initially involves downgaze

Subsequent defective up and horizontal gaze

• Pseudobulbar palsy

• Extrapyramidal rigidity

( Steele-Richardson-Olszewski syndrome )

• Gait ataxia

• Dementia

Medial wall blow-out fracture

Signs

• Release of entrapped tissue• Repair of bony defect

Periorbital subcutaneous emphysema Ophthalmoplegia - adduction and abduction if medial rectus muscle is entrapped

Treatment

COMITANT

• Review History

• Decompensated Phoria

• Accomodative Esotropia

• Acute Esotropia of Childhood

• Vergence Paresis

• Skew Deviation

• Foveal Displacement Syndrome

• Central Disruption of Fusion

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Decompensated Phoria

• latent ocular misalignment due to lose of single binocular fusion

• associated with febrile illness, head trauma, changing refractive needs, asthenopia

• presence of adaptive head posture and large fusional amplitudes

Refractive accommodative esotropia

Fully accommodative

• Normal AC/A ratio• Excessive hypermetropia

Straight for distance and near

Partially accommodative

Esotropia for near

• Presents between 18 months - 3years• Initially intermittent

Esotropia greater for near Straight for distance

Non-refractive accommodative esotropia• Presents between 18 months to 3years

• High AC/A ratio - due to increased AC (convergence excess) - due to decreased A (hypoaccommodative)• No significant refractive error

Esotropia for near Straight for distance

Signs

Recent right sixth nerve palsy

Right esotropia in primary position due to unopposed action of right medial rectus

Marked limitation of right abduction due toright lateral rectus weakness

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Skew Deviation

• vertical misalignment of visual axes due to imbalance of prenuclear inputs

• vertical diplobia cannot be isolated to a single EOM(s)

• Hypertropia varies with gaze associated with downbeat nystagmus

• brainstem and cerebellar disease, MS, INO, increased ICP

Assessment after photocoagulation

• Persistent neovascularization

• Haemorrhage

Poor involution

• Re-treatment required

• Regression of neovascularization• Residual ‘ghost’ vessels or fibrous tissue

Good involution

• Disc pallor

Choroidal neovascularization (CNV)

• Metamorphopsia is initial symptom• Most lesions are not visible clinically

Suspicious clinical signs

Pinkish-yellow subretinal lesion with fluid

Subretinal blood or lipid

• Less common than atrophic AMD but more serious

Idiopathic premacular fibrosis

• Pucker emanating from epicenter

• Opaque epiretinal membrane

• May be associated with macular pseudo-hole

• Severe retinal wrinkling and vascular distortion

Macular pucker

• Translucent epiretinal membrane• Fine retinal striae and mild vascular distortion

Cellophane maculopathy

ResectionRecession

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Summary

• What is patient’s real complaint ?

• Disease process, work up, time frame

• Trial of prisms

• Is surgery an option?

• Are other referrals or treatments necessary?

• Communication between all parties

• Evidence-Based Medicine

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Evidence-based Medicine

• “ the conscientious, judicious,explicit use of the best available evidence from clinical care research in making health care decisions”

• Harvard Health Policy Review 2007: 8:145-155 Montori and Guyatt: Corruption of the evidence as threat and opportunity for evidence-based medicine

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VISION THERAPY

• 1) Orthoptic VT helpful for convergence insufficiency and binocular function

• 2) Behavioral-Perception VT unproven for visual processing and perception

• 3) Prevention or correction of Myopia unproven

• * Eye excercises do not treat learning disabilities

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Learning Disabilities

• 15-20% of the population affected with reading, math, foreign langauge problems, organizing written and spoken language

• reading disorder different from dyslexia

• 85% have dyslexia, whereby, loosing place reading due to difficulty decoding letter(s) or word combinations and/or lack of comprehension, not because of a “tracking abnormality”

References:• Burde RM, Savino PJ, Trobe JD. Cliinical Decisions in Neuro-

Ophthalmology, 2nd ed. St. Louis: Mosby-Year Book Inc., 1992.• Gorin G, History of Ophthalmology. Delaware:Publish or Perish, 1982

• Miller KM, Capo H, Mallette RA, Guyton DL. Ocular Motility and Binocular Vision. St. Louis: C.V. Mosby Co., 1989.

• Pediatric Ophthalmology and Strabismus, Section 6, Basic and Clinical Course. San Francisco: American Academy of Ophthalmology,1990-1.

• Taylor D. Pediatric Ophthalmology. Cambridge: Blackwell Scientific Publications, Inc, 1990.

• Wright KW. Text Book of Ophthalmology. Baltimore: Williams P. Wilkins, 1997.

• Kanski JJ, Bolton A. Illustrated Tutorials in Clinical Ophthalmology.

• Butterworth-Heinemann, 2001.

• American Orthoptic Journal, Volume 60, 2010

• FocalPoints Advances in the Management of Amblyopia, Kerr,NC 2010

• FocalPoints Practical Management of Amblyopia, Keech,RV Mar 2000

• FocalPoints Diplobia:Diagnosis and Management, Lee,MS Dec 2007

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