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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