DOUBLE VISION,
ACQUIRED, and CONGENITAL
STRABISMUS
Robert Cordero, M.D., F.A.C.S
Central Florida Eye Specialists, P.L.
October 30, 2010
• www.theeyespecialists.com
• www.aapos
• www.aao
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New, Updated, Revised, Better
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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
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Georg Bartisch (1535-1606)
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
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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
Questions to Ask
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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
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Work-up Diplopia
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History, History, Histroy
Old Records and Studies
Compare Measurements
Impression(s)
Plan: Short and Long Term
Communication
Tools of the Trade
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Patience, Efficiency and Insight
Lensometer readings
Best Corrected Vision, Pinhole
Stereopsis
Prism Bars, Occluder, Pupil Light
Clip-ons, Trial Set, Cycloplegia
Basic Review
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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
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Tests for stereopsis
Titmus
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Polaroid spectacles
Figures seen in 3-D
TNO random dot test
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Red-green spectacles
‘Hidden’ shapes seen
Lang
Frisby
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No spectacles
‘Hidden’ circle seen
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No spectacles
Shapes seen
Tests for sensory anomalies
Worth four-dot test
a - Prior to use of glasses
b - Normal or ARC
c - Left suppression
d - Right suppression
e - Diplopia
Bagolini striated glasses
a - Normal or ARC
b- Diplopia
c - Suppression
d - Small suppression scotoma
Synoptophore
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Grading of binocular vision
Detection of suppression and ARC
Measurement of angle
Measurement of fusional amplitudes
Hirschberg test
• Rough measure of deviation
• Note location of corneal light reflex
• 1 mm = 7 or 15
Reflex at border of pupil = 15
Reflex at limbus = 75
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Motility tests
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Tests versions and ductions
Grades under/overaction
Left inferior oblique overaction
Left lateral rectus underaction
Cover tests
• Cover test detects heterotropia
• Uncover test detects heterophoria
• Prism cover test measures tropia
• Alternate measures total tropia and phoria
• Alternate cover test detects total deviation
Amblyopia
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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
The Pediatric Eye Disease
Investigator Group (PEDIG)
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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
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Types of Turns
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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
Small and steep curvature
Larger and ellipsoidal
Globus cone
Largest
Progressive iris atrophy
Progressive stromal iris atrophy
Broad-based PAS
Displacement of pupil
towards PAS
Isolated familial ectopia lentis
Autosomal recessive
Pupil may be normal
Pupil may be displaced in opposite
direction (ectopia lentis et pupillae)
Classification according to maturity
Immature
Hypermature
Mature
Morgagnian
Implant displacement
Decentration
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May occur if one haptic is inserted
into sulcus and other into bag
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Remove and replace if severe
Optic capture
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Reposition may be necessary
Binocular Diplopia
No Misalignment
INCOMITANT
Mechanical (Restrictive)
Diplopia
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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
• Unilateral lid retraction
• Unilateral proptosis
• Lid lag in downgaze
Restrictive myopathy
• Occurs in about 40%
• Due to fibrotic contracture
Elevation defect - most common
Depression defect - uncommon
Abduction defect - less common
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)
• Non-neoplastic, non-infectious orbital lesion (pseudotumour)
• Involves any or all soft-tissue components
• Presentation - 20 to 50 years with abrupt painful onset
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Usually unilateral
Periorbital swelling and chemosis
Proptosis
Ophthalmoplegia
Clinical course and treatment of IOID
1. Early spontaneous remission without sequelae
Treatment - nil
2. Prolonged intermittent activity with eventual remission
Treatment options - steroids, radiotherapy or cytotoxics
3. Severe prolonged activity causing a ‘frozen orbit’
Left involvement resulting in ophthalmoplegia and ptosis
Orbital myositis
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Subtype of IOID
Involvement of one or more extraocular muscles
Clinical course is usually short - treat with NSAIDs
Presentation - sudden onset of pain on ocular movement
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Underaction of left lateral rectus
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Worsening of pain on attempted left gaze
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CT shows fusiform enlargement
of left lateral rectus
Cavernous haemangioma
• Most common benign orbital tumour in adults
• Usually located just behind globe
• Female preponderance - 70%
• Presents - 4th to 5th decade
Slowly progressive axial proptosis
May cause choroidal folds
Treatment - surgical excision
Pleomorphic Lacrimal Gland Adenoma
Presents - 4th to 5th decade
• Painless and very slow- • Posterior extension may • Smooth, encapsulated
growing, smooth mass in cause proptosis and
outline
ophthalmoplegia
lacrimal fossa
• Excavation of lacrimal gland
• Inferonasal globe
fossa without destruction
displacement
Lacrimal gland carcinoma
• Presents - 4th to 6th decades
• Very poor prognosis
Posterior extension may cause proptosis,
ophthalmoplegia and episcleral
congestion
• Trigeminal hypoaesthesia in 25%
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Painful, fast-growing mass in
lacrimal fossa
Infero-nasal globe displacement
Management
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Biopsy
Radical surgery and radiotherapy
Optic nerve glioma
• Typically affects young girls
• Associated neurofibromatosis -1 is common
• Presents - end of first decade with gradual visual loss
Gradually progressive proptosis
Optic atrophy
Treatment
• Observation - no growth, good vision and good cosmesis
• Excision - poor vision and poor cosmesis
• Radiotherapy - intracranial extension
Sphenoidal ridge meningioma
Presents with gradual visual loss and reactive hyperostosis
Proptosis
Fullness in temporal fossa Hyperostosis on plain x-ray
Lymphoma
Presents - 6th to 8th decades
Affects any part of orbit and Anterior lesions are rubbery May be confined to
may be bilateral
on palpitation
lacrimal glands
Treatment
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Radiotherapy - localized lesions
Chemotherapy - disseminated disease
Direct carotid-cavernous fistula
• Defect in intracavernous part of internal carotid
• Rapid flow shunt
Causes
• Head trauma - most common
• Spontaneous rupture - in hypertensive females
• Ptosis, chemosis and conjunctival injection
• Ophthalmoplegia
• Raised intraocular pressure
Direct carotid-cavernous fistula
• Pulsatile proptosis with bruit • Retinal venous congestion and haemorrhages
and thrill
• Abolished by ipsilateral
carotid compression
Indirect
carotid-cavernous
fistula
(dural
shunt)
• Indirect communication between meningeal branches of internal
or external carotids and cavernous sinus
• Slow flow shunt
Causes
• Congenital malformations
• Spontaneous rupture
• Dilated episcleral vessels
• Raised intraocular pressure with
wide pulsation
• Occasional ophthalmoplegia
and mild proptosis
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
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Ptosis
EOM Palsies
Pseudogaze Palsies
Pseudointernuclear Ophthalmoplegia
Pseudoconvergence Paresis
Lid Twitch
Quiver Movements
Orbicularis Weakness
Myasthenia Gravis
1. Clinical features
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Uncommon, typically affects young women
Weakness and fatiguability of voluntary musculature
Types: Neonatal, Congenital, Ocular, System
2. Investigations
ICE Test
•Tensilon test (edrophonium) or Prostigmine
• Antibodies to acetylcholine receptors 3 types, MuSK
(muscle-specific receptor tyrosine kinase)
• CT or MRI for presence of thymoma
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3. Treatment options
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Medical - AChE inhibitor, steroids, immunomodulators
Thymectomy, prisms, strabismus surgery
Ocular myasthenia
Ptosis
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Insidious, bilateral but asymmetrical
Worse with fatigue and in upgaze
Ptotic lid may show ‘twitch’ and
‘hop’ signs
Diplopia
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Intermittent and usually vertical
Edrophonium test
Before injection
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Measure amount of ptosis or
diplopia before injection
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Inject i.v. atropine 0.3 mg
Positive result
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Inject i.v. test dose of edrophonium
(0.2 ml-2 mg)
Inject remaining (0.8 ml-8 mg) if no
hypersensitivity
Cranial Neuropathy
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Oculomotor (3rd) Cranial Nerve
Trochlear (4th) Cranial Nerve
Abducen (6th) Cranial Nerve
Adult, Child, TITS
Anatomy of third nerve
Oculomotor nucleus
Pituitary gland
Red nucleus
Carotid artery
Cavernous sinus
Pons
III nerve
Post cerebral artery
Clivus
Basilar artery
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 part
of nerve but not pupillary
fibres ( damaged by medical
lesions )
Pupillary fibres lie dorsal and peripheral
Signs of right third nerve palsy
• Ptosis, mydriasis and cycloplegia • Normal abduction
• Abduction in primary position
• Limited adduction
• Intorsion on attempted
downgaze
• Limited elevation
• Limited depression
Important causes of isolated third nerve palsy
Idiopathic - about 25%
Vascular disease - hypertension, diabetes
Trauma
Posterior communicating aneurysm
Extradural
haematoma
Aneurysm
Chiasm
Midbrain
pushed
across
Edge of
tentorium
Prolapsing
temporal
lobe
Posterior cerebral
artery
Third nerve
Anatomy of fourth nerve
Internal carotid artery
Postr. communicating
artery
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VI
Postr.cerebral artery
Supr.cerebellar artery
Basilar artery
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Only cranial nerve to emerge dorsally
Crossed cranial nerve
Very long and slender
Signs of right fourth nerve palsy
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Right hyperdeviation in primary
position when left eye fixating
Excyclotorsion
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Right underaction on depression
in adduction
Vertical diplopia
Right overaction on left gaze
Positive Bielschowsky test in right fourth nerve pal
Increase in right
hyperdeviation on ipsilateral
head tilt
Absence of right
hyperdeviation on
contralateral head tilt
Anatomy of sixth nerve
Basilar artery
Medial
lemniscus
Pituitary gland
Carotid artery
4th ventricle
Cavernous sinus
Petroclinoid
ligament
Vestibular
nucleus
Clivus
VI nerve
Pyramidal tract
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 palsy
Vascular - hypertension, diabetes
Raised intracranial pressure
Acoustic neuroma
Dilated
ventricles
Petrous
tip
Brainstem pushed downwards
SUPRANUCLEAR DISORDERS OF
EYE MOVEMENT
1. Horizontal gaze palsies
• Internuclear ophthalmoplegia
• Combined internuclear and PPRF
(‘one-and-a-half syndrome’)
MLF
2. Vertical gaze palsies
• Parinaud dorsal midbrain syndrome
• Progressive supranuclear palsy
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Internuclear ophthalmoplegia
Lesion involving left MLF
Defective left adduction and ataxic
nystagmus of right eye
Normal left gaze
Convergence intact if lesion discrete
Important causes
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Demylination - usually bilateral
Vascular disease
Tumours of brainstem
‘One-and-a-half syndrome ’
Combined lesion of left MLF and PPRF
Paralytic Pontine Exotropia
• Ipsilateral (left) gaze palsy
• Defective left adduction
• Normal right abduction with ataxic
nystagmus
Parinaud dorsal midbrain syndrome
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Supranuclear upgaze palsy
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Large pupils with light-near dissociation • Convergence weakness
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Lid retracton (Collier sign)
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Normal downgaze
Convergence-retraction nystagmus
Important causes
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In children: aqueduct stenosis, meningitis and pinealoma
In young adults: demylination, trauma and a-v malformations
In elderly: vascular accidents and posterior fossa aneurysms
Progressive supranuclear palsy
( Steele-Richardson-Olszewski syndrome )
• Affects elderly
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Pseudobulbar palsy
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Extrapyramidal rigidity
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Gait ataxia
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Dementia
Initially involves downgaze
Subsequent defective up and
horizontal gaze
Medial wall blow-out fracture
Signs
Periorbital subcutaneous emphysema
Ophthalmoplegia - adduction and abduction
if medial rectus muscle is entrapped
Treatment
• Release of entrapped tissue
• Repair of bony defect
COMITANT
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Review History
Decompensated Phoria
Accomodative Esotropia
Acute Esotropia of Childhood
Vergence Paresis
Skew Deviation
Foveal Displacement Syndrome
Central Disruption of Fusion
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
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Refractive accommodative esotropia
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Presents between 18 months - 3years
Initially intermittent
Normal AC/A ratio
Excessive hypermetropia
Fully accommodative
Partially accommodative
Esotropia greater for near
Straight for distance
Straight for distance and near
Esotropia for near
Non-refractive accommodative esotropia
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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
Signs
Straight for distance
Esotropia for near
Recent right sixth nerve palsy
Right esotropia in primary position due to
unopposed action of right medial rectus
Marked limitation of right abduction due to
right lateral rectus weakness
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
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Assessment after photocoagulation
Poor involution
Good involution
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Persistent neovascularization
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Haemorrhage
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Re-treatment required
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Regression of neovascularization
Residual ‘ghost’ vessels or
fibrous tissue
Disc pallor
Choroidal neovascularization (CNV)
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Less common than atrophic AMD but more serious
Metamorphopsia is initial symptom
Most lesions are not visible clinically
Suspicious clinical signs
Pinkish-yellow subretinal lesion
with fluid
Subretinal blood or lipid
Idiopathic premacular fibrosis
Macular pucker
Cellophane maculopathy
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Translucent epiretinal
membrane
Fine retinal striae and mild
vascular distortion
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Severe retinal wrinkling and
vascular distortion
• Pucker emanating from
epicenter
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Opaque epiretinal membrane
May be associated with
macular pseudo-hole
Recession
Resection
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Summary
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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
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”
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References:
• Burde RM, Savino PJ, Trobe JD. Cliinical Decisions in NeuroOphthalmology, 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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