presentation on infantile esotropia

1. INFANTILE ESOTROPIA
Moderator : Dr Kavitha V
Presenter : Dr Padmapriya PG

2. INTRODUCTION
Orthotropia The normal alignment where both eyes are
aligned on the same object of regard, retinal images fall
appropriately on each fovea
Strabismus Misalignment of the eyes, with the fovea of one
eye aligned with the fixation target and fovea of the other
eye off the target
Heterophoria tendency of the eyes to deviate is kept latent
by fusion reflex during binocular vision
Heterotropia Manifest deviation of visual axes

3. DEFINITION OF SQUINT
Normally visual axis of two eyes are parallel to each other in ‘primary
position of gaze’ and this alignment is maintained in all positions of
gaze.
Orthophoria- Position of eyes in which primary lines of sight intersect
at the fixation point.
A misalignment of the visual axes of two eyes is called Squint Or
Strabismus
3

4. • Esodeviation - inward turning of the eye, image in
deviated eye falls nasal to the fovea
• Esodeviations are the most common type of
ocular misalignment
• Represents 50% of ocular deviations in pediatric
age group
• Esodeviations often present as a constant esotropia
occurring in infancy or early childhood

5. Stages of development
Stage of latent deviation(Esophoria)
Stage of intermittent deviation(Intermittent Esotropia)
Stage of constant deviation(Esotropia)

6. Clinical types
ESOTROPIA
INCOMITANT
ESOTROPIA
COMITANT
ESOTROPIA

7. COMITANT ESOTROPIAS
Infantile (congenital) esotropia
Accommodative esotropia
• Refractive accommodative
• Non refractive accommodative
• Hypo accommodative
• Mixed or partially accommodative

8. Acquired non accommodative esotropia
• Essential acquired
• Acute concomitant esotropia
• Microtropia
• Nystagmus blockage syndrome
• Cyclic esotropia
• Divergence paralysis
• Stress induced esotropia
• Esotropia in myopia
• Esotropia due spasm of near reflex
Sensory esotropia
Consecutive esotropia

9. INCOMITANT ESOTROPIA
1.Paralytic esotropia
• Lateral rectus paralysis
• Divergence paralysis
• Mobius syndrome.
2.A and V pattern
3.Restrictive esotropia
• Duane`s retraction syndrome
• Thyroid myopathy
• Medial orbital wall fracture
• Srabismus fixus

10. ESSENTIAL INFANTILE ESOTROPIA

11. • Large angle esotropia present before 6 months of
age
• Most common form of strabismus
• Incidence : 1 to 2 per cent
• Prevalence: 27/ 10,000
• Sex distribution and race distribution :uniform
• Family history positive but no well defined
genetic pattern
• Concordance in monozygous twins to be 81 per
cent, compared with 9 per cent in dizygotic

12. • Incidence -- higher in patients with cerebral palsy,
hydrocephalus (30% )
• Maternal cigarette smoking and low birth weight linked to the
development of esotropia
• More prevalent in low birth weight, premature infants, perinatal
hypoxia
• Increased risk of development of mental illness by early
adulthood

13. ETIOLOGY
WORTH THEORY
• Congenital absence of cortical fusion potential
• Lack of binocular fusion
• States there is no hope for obtaining good binocular function.
CHAVASSE THEORY
• Primary motor misalignment
• Disruption of binocular vision
• Binocular cortical potential for high-grade stereopsis and fusion

14. • Early weakness of the lateral rectus muscle,
• Hypermetropia causing accommodative convergence,
• Abnormalities of muscle anatomy,
• Lack or immaturity of cortical fusion,
• Immaturity of sixth nerve function

15. CLINICAL FEATURES
• Timeofonset-Within first 6 months
• Angle of deviation - Large angle constant
esotropia , >40PD
• Small to moderate and variable or intermittent
occurring in first few months - CEOS
• Fixation pattern :
Alternate fixation in primary gaze
Crossed fixation in lateral gaze
• Apparent limitation of abduction

16. Visual acuity
• Normal and equal in BEs in patients who freely alternate fixation
• If one eye is preferred amblyopia will develop in other eye
• Amblyopia occurs in 2 to 40% patients
• Incidence of amblyopia seems to be proportional to the duration of the esotropia
Refractive errors
• Not prominent, but consistent with age groups.

17. Associated Motor Abnormalities
Inferior oblique overaction (70%)
Dissociated Vertical Deviation (75%)
Dissociated Horizontal Deviation (10%)
Latent Nystagmus (Fusion maldevelopment nystagmus
(FMN) – 50%
Smooth pursuit asymmetry

18. Dissociated vertical deviation (DVD):
• Intermittent slow deviation of
nonfixing eye consisting of upward
excursion, excyclotorsion and
slight abduction.
• Two eyes are independent of each
other and do not follow the
Hering’s law of ocular motility.
• Incidence: 75%

19. Dissociated Horizontal Deviation
• Exo component of DVD exaggerated
• Occurs in patients who have had previous surgery for congenital
esotropia
• Detected in 50% patients who develop consecutive exotropia
following surgery for congenital esotropia
Inferior oblique over action (IOOA):
• Results in elevation of the involved eye as it moves nasally
• Incidence: 75%

20. Overaction of inferior oblique muscles.
(A) Right inferior oblique overaction with left eye fixing in left gaze.
(B) Left inferior oblique overaction with right eye fixing in right gaze.

21. DISSOCIATED VERTICAL DEVIATION INFERIOR OBLIQUE OVERACTION
Present in all gaze positions Present in adduction only
Does not obey the Hering’s law Obeys the Hering’s law
Slow floating abduction, elevation, excyclotorsion
movement
Rapid elevation, abduction movement
Not associated with A or V pattern Often associated with V pattern
Proportional to ambient illumination in fixing eye Not proportional to illumination in fixing eye
No objective fundus torsion Objective fundus excyclotorsion

22. Latent nystagmus
• Latent nystagmus: becomes manifest when one eye is
occluded
• Jerk type with fast component directed towards the
fixating (uncovered) eye.
Smooth pursuit asymmetry
• Poor nasal-to-temporal OKN regardless of the degree of
stereopsis or the timing of surgery
• Only seen during monocular occlusion

23. Systemic association
Down's syndrome, albinism, and cerebral palsy.

24. MANAGEMENT
• CLINICAL EVALUATION AND DIFFERENTIAL DIAGNOSIS
• NON SURGICAL TREATMENT
• SURGICAL TREATMENT
• POST SURGICAL TREATMENT.

25. DIFFERENTIAL DIAGNOSIS OF
INFANTILE ESOTROPIA.
• Pseudo- esotropia
• Infantile accommodative esotropia
• Cinancia syndrome
• Duane's syndrome
• Sensory esotropia
• Mobius syndrome
• Strabismus fixus
• Congenital sixth nerve palsy
• Infantile myasthenia gravis
• Neurological disease

26. PSEUDO- ESOTROPIA
• Wide nasal bridge
• Prominent epicanthal folds
• Narrow interpupillary distance
• Negative angle Kappa

27. INFANTILE ACCOMMODATIVE ESOTROPIA
Differentiating feature from infantile esotropia are
• Presence of straight eyes for first 2-3 months
• Hypermetropia >2D
• Variable angle of strabismus

28. CIANCIA SYNDROME
Large angle congenital esotropia with cross fixation ,in
which both eyes appear to be “stuck” in towards the nose
Features :
• Large angle deviation (>60PD)
• Bilateral limited abduction with intact abduction
saccades
• Fixing eye in adduction
• Nystagmus on attempted abduction, not in
adduction
• Face turn to side of fixingeye

29. CONGENITAL FIBROSIS SYNDROME /STRABISMUS FIXUS
• Large angle congenital esotropia with severe limitation of abduction
• Congenital restrictive strabismus
• Autosomal dominant inheritance
• OKN or doll’s head maneuver will show abduction saccadic eye
movements of brisk small amplitudes

30. MOBIUS SYNDROME
• Congenital bilateral abducent paralysis with
bilateral facial palsy
• It may be associated with other cranial nerve
palsies, orofacial dysmorphism, distal limb or axial
malformations, and intellectual impairment
• Patterns of ocular motility
(1) Orthotropia in primary position with complete
abduction and adduction deficits (41%),
(2) Large-angle esotropia with cross-fixation (50%),
and
(3) Large-angle exotropia with torticollis, absence of
convergence, and vertical eye misalignment (9%)

31. DUANE’S SYNDROME
• Congenital, usually sporadic miswiring of the
medial and lateral rectus muscles, resulting in
abnormal horizontal eye movements.
• The most common variant of this syndrome is
Duane type 1 (85%)
• More common in girls (60%) and on left side
(60%)
• Limited abduction of the involved eye and often
has a “tether” phenomenon, which consists of
overelevation or overdepression on attempted
adduction as the retracted globe escapes from
its horizontal rectus muscle restriction

32. SENSORY ESOTROPIA
• Occurs in patients with early onset retinoblastoma and other
disorders of optic nerve and retina
CYCLIC ESOTROPIA
• Intermittent esotropia
• Presents as alternate 12- to 36-hour periods of perfect alignment
followed by constant, usually large-angled (30–40Δ) esotropia.
• Age of onset - 3–4 years

33. EXAMINATION
1. Examination of anterior segment
2. Estimation of visual acuity
• Optokinetic nystagmus test
• Preferential looking test
• Visual evoked potential
• Catford drum test
• Cardiff acuity cards test
• Indirect assessment by – blink response, menace reflex , fixation behavior test ,
CSM method
3. Examination of fundus and media
4. Cycloplegic refraction

34. 5. Measurement of AC/A ratio
6. Measurement of deviation
7. Differentiation from simulated bilateral 6th nerve palsy
• Doll’s head phenomenon test
• Rotating test for demonstrating abduction
• Alternate patching

36. TREATMENT
NON –SURGICAL MEASURES
1)Correction of refractive errors
• More than +3.00 D, should be corrected in small angle cases
• More than +4.00 D, should be corrected in large angle esotropia
• Miotics in uncooperative infants
2) Amblyopia therapy

37. CONGENITAL ESOTROPIA
OBSERVATIONAL STUDY (CEOS)
• Infantile esotropia < 40PD and intermittent or variable frequently resolves
spontaneously at less than 20 weeks of age
• Cases with a constant deviation 40PD or more on 2 exams with <3D of
hypermetropia - spontaneous resolution less likely

38. SURGICAL TREATMENT
Time Of Surgery
• Standard approach - Between 6 months to 2 years to age
• Peripheral fusion is achieved in most cases if operated before 2
years of age
• Recent studies favor very early surgery as soon as diagnosis is made
• Wait upto 6 months of age in intermittent esotropia or small to
moderate angledeviations

39. EARLY SURGERY LATE SURGERY
Advantages
1. Increased potential for binocularity
2. Reduced muscle contracture
1. Stable & reliable measurement
2. Better amblyopia management
3. Lesser chances of reoperation
4. Decreased risk of general anesthesia
Disadvantages
1. Unstable measurement
2. Chances of recurrent or residual esotropia
& spontaneous resolution
3. Can not rule out accommodative
component
4. Technical difficulties due to exact
measurement of growing eyeball
1. Reduced chances of binocularity
2. Increased muscle contracture can lead to
mechanical component of squint

40. Before proceeding to surgery :
• Deviation should be constant and stable
• Fixation should be alternating
• Accomodative element should beabsent
• Sensory esotropia should have been ruled out
• Amblyopia should be treated optimally

41. Surgical Approach
• Bilateral Medial rectus recession
• Max of 8mm recession can be done
• Unilateral MR recession with lateral rectus (LR ) resection can be
done over non dominant eye
• Standard surgical charts will be used and surgery is usually based on
near deviation.
• Inferior oblique overaction : muscleweakening procedures
• Alignment within 8-10 Δ of orthophoria: acceptable

42. Deviations
(PD)
B/L MR
recession
(mm)
Recession
of MR
(mm)
Resection
of LR
(mm)
15 3 3 3.5
20 3.5 3.5 4
30 4.5 4.5 5.5
40 5.5 5.5 6.5
50 6 6 7
60 6.5 6.5 7.5
70 7 7 8
In children <5months max 6mm recession can be done

43. POST SURGICAL OUTCOMES
SUBNORMAL BINOCULAR VISION
• Best possible outcome
• Orthophoria or asymptomatic heterophoria with stable alignment
• Normal Visual Acuity
• Peripheral fusion eventually develops which allows a low grade stereopsis
• Normal retinal correspondence
• Do not require any further treatment besides amblyopia therapy and/or
correction of refractiveerrors

44. MICROTROPIA
• Deviation <10PD
• Desirable treatment result
• Inconspicuous shift or no shift on cover test .
• Peripheral fusion usually develops with low grade of stereopsis and normal
fusional vergence amplitudes
• Mild amblyopia is common
• No further treatment except amblyopia prevention

45. SMALL ANGLE RESIDUAL ESOTROPIA OREXOTROPIA
• Considered as an acceptable treatment
• Deviation less than 15 PD are cosmetically acceptable
• About 80 % patients develops anomalous retinal
correspondence
• Stereopsis is of low grade or absent
• No further treatment is required except amblyopia prevention

46. LARGE ANGLE RESIDUAL ESOTROPIA
• Due to under correction
• Esotropia more than 20 D
• Leads to suppression amblyopia
• Absent stereopsis
• Needs repeat surgery within 3 months
• Bilateral LR resection in case primary MR recession has been
done else LR resection with MR recession in the other eye

47. LARGE ANGLE CONSECUTIVE EXOTROPIA
• Due to over correction
• In children below 2 years, surgery should be done early.
• In patients above 8 years of age, wait for about 6 to 8 weeks,
surgery only if deviation is more than 20 PD
• Copper’s dictum
• Surgical Options
• LR recession with advancement of previously recessed
MR
• Bilateral LR recession
• LR recession + MR resection of the other eye

48. BOTULINUM TOXIN
• To create a incomitant deviation so that patient could adopt a
face turn and attain fusion
• Multiple injections may be required
• Alignment and sensory outcomes not comparable to surgery
• Complications include
• Secondary ptosis
• Initial consecutive exotropia lasting up to 2 to 3
months

49. SURGERIES FOR DVD AND INFERIOR OBLIQUE
OVERACTION
DVD if monocular or asymmetrical
- Recession of superior rectus muscle or resection of inferior rectus muscle
- Recession of superior rectus muscle with posterior fixation suture
INFERIOR OBLIQUE OVERACTION
- Weakening procedures
- Myectomy or recession
Simultaneous DVD and IOOA
- Anteriorzation of Inferior oblique insertion to the margin of the inferior rectus.

50. References
1) Pediatric ophthalmology and strabismus, 3rd edition, Kenneth Wright
2) Yanoff and Duker Ophthalmology, 5th edition
3) Kanski’s clinical ophthalmology, 8th edition
4) Squint and orthoptics , A K Khurana 2nd edition

51. THANK YOU