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INCOMITANT ESOTROPIA
DR. YOUSAF JAMAL
FCPS RESIDENT
OPHTHALMOLOGY UNIT
29/08/2009
CONTENTS Introduction Important tests Etiology and management Take home message Mcqs Word for the day
INTRODUCTION
When esotropia varies in horizontal gaze Mechanism
neurological mechanical
Some tests
Forced duction test Active force generation test Hess chart
Forced duction test
Hess chart
What is it When to do How to do Interpretation
Eye involved Mechanical vs. neurogenic Evolution over time
CAUSES OF INCOMITANT ESOTROPIA
Sixth Nerve palsy Medical rectus restriction Special forms
Sixth nerve
Abducent nerve Purely motor Supplies lateral recti Pathway
Mid pons fasiculus pontomedullay- junction intracavernous intraorbital
LR
CAUSES OF 6TH NERVE PALSY(adults)
Idiopathic Vasculopathic (most
common) Diabetes Hypertension Atherosclerosis
Trauma basal skull fracture
Increased ICP Cavernous sinus
Thrombosis Meningioma Aneurysm Metastasis
Multiple sclerosis Sarcoidosis
…Contd…
Vasculitis Stroke Acoustic neuroma Meningitis Metabolic
Vit. B12 W-k syndrome
Invasion thru skull base Nasopharyngeal ca Chordoma Chondrosarcoma
Infectious Lyme disease Syphilis
Children
Idiopathic Birth trauma Viral infections Vaccination Increased ICP
Hydrocephalus Gradenigo syndrome Brainstem glioma*
*Harley RD. Paralytic strabismus in children. Etiologic incidence and management of the third, fourth, and sixth nerve palsies.Ophthalmology. 1980 Jan;87(1):24-43.
Presentation
Symptoms Horizontal diplopia Worse for distance Pronounced in the lateral gaze
Signs
Esotropia in primary position Worse for distance Limited abduction Normal adduction Binocular diplopia Face turn
Differential Diagnosis
Myasthenia gravis Restrictive thyroid myopathy Duane syndrome Medial orbital wall blowout fracture Convergence spasm Myositis Divergence paralysis
Work Up
History: Age of onset Prior therapy e.g.. Glasses, patching Symptoms fluctuation HTN, DM, thyroid, trauma, other causes
Examination: Neurological:
MS, increased ICP, Gradenigo syndrome, stroke, acoustic neuroma
Ophthalmic Examination
Optic nerve functions VA+ BCVA Visual fields
Motility test Restricted movements
Ophthalmoscopy Papilledema
…Contd…
Hess chart Forced duction test
Investigations
BP FBS HBA1c Serology
Lyme syphilis
…Contd…
CT MRI Brain
<45 years (if –ve then LP) 45-55 years with no hx of vasculopathy VI th nerve palsy + severe pain or neurological
signs Any Hx of Ca Bilateral VI th Nerve palsy Papilledema
In children
Emphasis on Trauma Recent illness Ear infections
Otoscopic examination MRI brain for all children
Treatment
Tx underlying cause Orthoptic TX
Base out prism Patching or fogging
Botulinum toxin in ipsilateral MR Surgery
Surgery
If persists for > 6 months Recession/resection Transposition of SR/IR insertions
Jansen procedure Hummelsceim procedure
Medial Rectus Restriction
Causes
Thyroid myopathy Medial orbital wall fracture Excessive resection of MR
THYROID MYOPATHY
Subset of Thyroid eye disease i.e. also called* Graves eye disease Thyroid ophthalmopathy Thyroid related ophthalmopathy Thyroid orbitopathy Thyroid related immune orbitopathy Thyroid eye disease
*american academy of ophthalmology. 2008-2009,Section 6
Pathogenesis
Autoimmune Infiltration of
Lymphocytes Plasma cells Mast cells
Deposition of mucopolysaccharides especially hyaluronic acid
Leads to edema and later fibrosis that cause restriction
…Contd…
Muscles may increase up to 6-8 times of normal size
Non-tendinuous part involved Frequency*
Inferior rectus (60-70%) Medial rectus (25%) Then superior and lateral rectus
*Char DH, Norman D. The use of computed tomography and ultrasonography in the evaluation of orbital masses. Surv Ophthalmol 1982;27:29.
Presentation
Symptoms: Decreased vision
Compressive optic neuropathy Double vision
Vertical Horizontal
Signs (for myopathy)
Often bilateral & asymmetric Restricted movements Hypotropia Esotropia Abnormal head position
Work-up (for myopathy)
HistoryDuration, pain, vision, known thyroid disease, smoker
Ocular examinationVisual acuityIOP measurement
Increased on attempted gaze
…Contd…
Forced duction test Positive
Diplopia measurement Prism Cover/uncover & alternate cover test Hess chart
…Contd…
TFTs EMG & tensilon tests show no abnormality Orbital ultrasound CT
Axial/coronal views MRI
Treatment General
Smoking cessation Medical internist or endocrinologist opinion Prisms temporarily used for diplopia in
primary positions
Surgery
Indications Diplopia in primary or reading positions Abnormal head position
When to do??* Angle of deviation stable for > 6 months In chronic & inactive cases After orbital decompression surgery
*Scot WF, Thalaker JA. Diagnosis an treatment of thyroid myopathy, Ophthalmology 1974;73:437.
…Contd…
Goal To achieve BSV in primary & reading position
Technique Recession is preferred Tx bcz resections worsen
the restriction Adjustable & non-absorbable sutures used Initial under correction is desirable
Medical Chemodenervation
Botulinum toxin A in affected muscle 1.5-5 units Onset of action…1-3 days Duration…3 months
Special forms
DUANE SYNDROME
MÖBIUS SYNDROME
DUANE SYNDROME
Characteristics Failure of innervation of LR by 6th nerve Innervation of LR by 3rd nerve
Imaging studies Hypoplasia / aplasia of 6th N. Nucleus
….Contd..
Mostly sporadic Autosomal dominant (5-10%) Females > Males (3:2) Left eye > right Systemic associations
Goldenhar syndrome Klippel-feil syndrome Wilderwanck syndrome
History First described by
Sinclair in 1895 Bahr in 1896 Stilling in 1887 Wolff in 1900
Duane described in 1905* 54 cases and offered theories
*Duane A. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. 1905. Arch Ophthalmol. Oct 1996;114(10):1255-6; discussion 1257
Clinical Features
BSV intact in primary position Limited horizontal movements
Restricted abduction Restricted adduction Both
Upshoot or downshoot Retraction of the globe
Classification
Two types Brown* Huber**
*Brown HW., (1950) Congenital structural muscle anomalies in: Allen JH ed. Strabismus Ophthalmic Symposium. St Louis, Mosby, pp 205-36
**Huber A., (1974) Electrophysiology of the retraction syndrome. British journal of ophthalmology 58, 293-300
Brown’s Classification
Based on clinical observations Type A
Limited abduction and less limited adduction Type B
Limited abduction but normal adduction Type C
Limited adduction > limited abduction
Huber’s Classification
Type 1 (70%-80%): Inability or limited abduction Normal or minimal defect in adduction Esotropia with head straight Globe retraction & palpebral-fissure
narrowing on adduction Usual face turn to affected side
Type 1 must be differentiated from 6th nerve palsy Globe retraction Mild Esotropia Fissure changes Upshoot and downshoot
Type 2 (about 7%)
Limited adduction Normal or minimal defect in abduction Exotropia of the affected eye Globe retraction and palpebral-fissure
narrowing on adduction Face turn to normal side
Type 3 (about 15%)
Limited abduction and adduction Globe retraction and palpebral-fissure
narrowing on attempted adduction Possible upshoot and downshoot on
adduction Straight or nearly straight head position
Left type I (left)
Type III (left)
Management
General measures Prisms: up to 25 error Special seating arrangement for children in
schools Vision therapy for secondary convergence
insufficiency Special rear mirrors while driving
Surgery Standard management Indications
Unacceptable face turn Significant misalignment Severe retraction Upshoot & downshoot
Procedures
Type 1 Recession of MR Recommended for > 20 deviation LR resection not favorable Partial or full transposition of vertical recti
Type 2 Recession of involved LR for small deviations Recession of both LR in large deviations Resection of MR not favorable
Type 3 For Severe globe retraction
Recession of both MR & LR
MÖBIUS SYNDROME
Very rare Paul julius Möbius, a German neurologist, in
1888 and 1892 Both congenital facial diplegia and bilateral
Abducent nerve palsies In 1939, henderson
Congenital unilateral facial palsy
Pathology Involvement of cranial nerves
Facial nerve in all cases Abducent nerve (75%) Hypoglossal nerve… usual Glossopharyngeal, vagus & accessory nerves…
uncommon Occulomotor & trochlear nerves… rare
Other systems involved Limbs Chest Orofacial defects
Presentation Ocular
6th nerve palsy Bilateral tight MR restriction Esotropia or straight eyes Both abduction limited Adduction is better with convergence
Systemic Mask like facies Defective lid closure Tongue atrophy Limb anomalies Low IQ
Treatment
MR recession
Take Home Message
Complete Hx Thorough Ophthalmic examination Tests interpretation Enough knowledge Physician/endocrinologist/neurologist opinion
MCQs1. 9 month girl has abnormal movement of Rt eye
which started shortly after birth but stable over time. Good VA, left face turn, with face turn eyes are straight, Rt eye moves normally but Lt fails to abduct past midline. Esotropia = 20 PD, cycloplegic refraction +1.00 sphere. Next step in management should be:
a. Neurological evaluation with neuro imagingb. Prescription of full cyclolegic refractionc. Observation onlyd. Strabismus surgery for deviation in primary position
Ans. C… case of duane syndrome with little face turn
2. A 30 year-old man developed a right sixth nerve palsy and facial pain. CT scan revealed opacity of the mastoid air cells. Diagnosis is…
a. Wallenberg's syndrome
b. Millard-Gubler's syndrome
c. Gradenigo's syndrome
d. Möebius' syndrome
Ans: c
3. A 6 year-old girl had bilateral Esotropia and absent facial expression. There are also punctate corneal staining due to exposure keratopathy. Corneal sensation appears normal. Diagnosis..
a. Duane's syndrome b. Möbius syndrome c. accommodative Esotropia d. intermittent divergent squint
Ans: b
4. All of the following would be expected to show restriction during forced duction testing except:
a. Thyroid associated orbitopathyb. Internuclear ophthalmoplegiac. Orbital fracture with IR entrapmentd. Congenital fibrosis of extra ocular muscles
Ans. b
a. mechanical/neurological strabismus
Ans. mechanical
b. Diagnosis
Ans. Duane syndrome
a. mechanical/neurological strabismusAns. Neurologicalb. Under acting muscleAns. Right LRc. Diagnosis Ans. Right 6th nerve palsy
a. mechanical/neurological strabismusAns. mechanicalb. restricted gaze?Ans. Left up and down gazec. Diagnosis Ans. Left orbital floor fracture with IR restriction
a. What is the primary position of the affected eye?Ans. Left Hypotropia b. In which direction is the eye movement
affected? Ans. Left up gaze and abductionc. Type of strabismusAns. Mechanicald. DiagnosisAns. Thyroid eye disease
WORD FOR THE DAY
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