1. INCOMITANT ESOTROPIA
DR. YOUSAF JAMAL
FCPS RESIDENT
OPHTHALMOLOGY UNIT
29/08/2009

2. CONTENTS
 Introduction
 Important tests
 Etiology and management
 Take home message
 Mcqs
 Word for the day

3. INTRODUCTION
 When esotropia varies in horizontal gaze
 Mechanism
 neurological
 mechanical

4. Some tests
 Forced duction test
 Active force generation test
 Hess chart

5. Forced duction test

6. Hess chart
 What is it
 When to do
 How to do
 Interpretation
 Eye involved
 Mechanical vs. neurogenic
 Evolution over time

11. CAUSES OF INCOMITANT ESOTROPIA
 Sixth Nerve palsy
 Medical rectus restriction
 Special forms

12. Sixth nerve
 Abducent nerve
 Purely motor
 Supplies lateral recti
 Pathway
 Mid pons fasiculus pontomedullay-
junction intracavernous intraorbital
LR

13. CAUSES OF 6TH NERVE PALSY
(adults)
 Idiopathic  Increased ICP
 Vasculopathic (most  Cavernous sinus
common)  Thrombosis
 Diabetes  Meningioma
 Hypertension  Aneurysm
 Atherosclerosis  Metastasis
 Trauma  Multiple sclerosis
 basal skull fracture  Sarcoidosis

14. …Contd…
 Vasculitis  Invasion thru skull
 Stroke base
 Nasopharyngeal ca
 Acoustic neuroma
 Chordoma
 Meningitis  Chondrosarcoma
 Metabolic  Infectious
 Vit. B12  Lyme disease
 W-k syndrome  Syphilis

15. 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.

16. Presentation
Symptoms
 Horizontal diplopia
 Worse for distance
 Pronounced in the lateral gaze

17. Signs
 Esotropia in primary position
 Worse for distance
 Limited abduction
 Normal adduction
 Binocular diplopia
 Face turn

19. Differential Diagnosis
 Myasthenia gravis
 Restrictive thyroid myopathy
 Duane syndrome
 Medial orbital wall blowout fracture
 Convergence spasm
 Myositis
 Divergence paralysis

20. 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

21. Ophthalmic Examination
 Optic nerve functions
 VA+ BCVA
 Visual fields
 Motility test
 Restricted movements
 Ophthalmoscopy
 Papilledema

22. …Contd…
 Hess chart
 Forced duction test

24. Investigations
 BP
 FBS
 HBA1c
 Serology
 Lyme
 syphilis

25. …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

26. In children
 Emphasis on
 Trauma
 Recent illness
 Ear infections
 Otoscopic examination
 MRI brain for all children

27. Treatment
 Tx underlying cause
 Orthoptic TX
 Base out prism
 Patching or fogging
 Botulinum toxin in ipsilateral MR
 Surgery

28. Surgery
 If persists for > 6 months
 Recession/resection
 Transposition of SR/IR insertions
 Jansen procedure
 Hummelsceim procedure

29. Medial Rectus Restriction
Causes
 Thyroid myopathy
 Medial orbital wall fracture
 Excessive resection of MR

30. 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

31. Pathogenesis
 Autoimmune
 Infiltration of
 Lymphocytes
 Plasma cells
 Mast cells
 Deposition of mucopolysaccharides
especially hyaluronic acid
 Leads to edema and later fibrosis that cause
restriction

32. …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.

33. Presentation
Symptoms:
 Decreased vision
 Compressive optic neuropathy
 Double vision
 Vertical
 Horizontal

34. Signs (for myopathy)
 Often bilateral & asymmetric
 Restricted movements
 Hypotropia
 Esotropia
 Abnormal head position

36. Work-up (for myopathy)
History

Duration, pain, vision, known thyroid disease,

smoker
Ocular examination

Visual acuity
IOP measurement

Increased on attempted gaze

37. …Contd…
 Forced duction test
 Positive
 Diplopia measurement
 Prism

Cover/uncover & alternate cover test

Hess chart

38. …Contd…
 TFTs
 EMG & tensilon tests show no abnormality
 Orbital ultrasound
 CT
 Axial/coronal views
 MRI

39. Treatment
 General
 Smoking cessation
 Medical internist or endocrinologist opinion
 Prisms temporarily used for diplopia in
primary positions

40. 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.

41. …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

42. Medical
 Chemodenervation
 Botulinum toxin A in affected muscle
 1.5-5 units
 Onset of action…1-3 days
 Duration…3 months

43. Special forms
 DUANE SYNDROME
 MÖBIUS SYNDROME

44. 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

45. ….Contd..
 Mostly sporadic
 Autosomal dominant (5-10%)
 Females > Males (3:2)
 Left eye > right
 Systemic associations
 Goldenhar syndrome
 Klippel-feil syndrome
 Wilderwanck syndrome

46. 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

47. Clinical Features
 BSV intact in primary position
 Limited horizontal movements
 Restricted abduction
 Restricted adduction
 Both
 Upshoot or downshoot
 Retraction of the globe

48. 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

49. 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

50. 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

51.  Type 1 must be differentiated from 6th nerve
palsy
 Globe retraction
 Mild Esotropia
 Fissure changes
 Upshoot and downshoot

52. 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

53. 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

54. Left type I (left)
Type III (left)

55. 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

56. Surgery
 Standard management
 Indications
 Unacceptable face turn
 Significant misalignment
 Severe retraction
 Upshoot & downshoot

57. Procedures
Type 1
 Recession of MR
 Recommended for > 20 deviation
 LR resection not favorable
 Partial or full transposition of vertical recti

58. Type 2
 Recession of involved LR for small deviations
 Recession of both LR in large deviations
 Resection of MR not favorable

59. Type 3
 For Severe globe retraction
 Recession of both MR & LR

60. 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

61. 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

62.  Other systems involved
 Limbs
 Chest
 Orofacial defects

63. Presentation
 Ocular
 6th nerve palsy
 Bilateral tight MR restriction
 Esotropia or straight eyes
 Both abduction limited
 Adduction is better with convergence

64.  Systemic
 Mask like facies
 Defective lid closure
 Tongue atrophy
 Limb anomalies
 Low IQ

67. Treatment
 MR recession

68. Take Home Message
 Complete Hx
 Thorough Ophthalmic examination
 Tests interpretation
 Enough knowledge
 Physician/endocrinologist/neurologist opinion

70. MCQs
1. 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 imaging
b. Prescription of full cyclolegic refraction
c. Observation only
d. Strabismus surgery for deviation in primary position
Ans. C… case of duane syndrome with little face turn

71. 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

72. 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

73. 4. All of the following would be expected to show
restriction during forced duction testing except:
a. Thyroid associated orbitopathy
b. Internuclear ophthalmoplegia
c. Orbital fracture with IR entrapment
d. Congenital fibrosis of extra ocular muscles
Ans. b

74. a. mechanical/neurological strabismus
Ans. mechanical
b. Diagnosis
Ans. Duane syndrome

75. a. mechanical/neurological strabismus
Ans. Neurological
b. Under acting muscle
Ans. Right LR
c. Diagnosis
Ans. Right 6th nerve palsy

76. a. mechanical/neurological strabismus
Ans. mechanical
b. restricted gaze?
Ans. Left up and down gaze
c. Diagnosis
Ans. Left orbital floor fracture with IR restriction

77. 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 abduction
c. Type of strabismus
Ans. Mechanical
d. Diagnosis
Ans. Thyroid eye disease

78. WORD FOR THE DAY

79. Next lecture
Decreased vision (transient)
by
Dr mushtaq
&
Journal club
By
Dr Maria