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Congenital Infantile
Esotropia
Presenter - Dr. Om Patel
Moderator – Dr. Varshini Shanker
Introduction
 The term is derived from 2 Greek words: ésò, meaning
inward, and trépò, meaning turn
 In esotropia, the eyes are crossed; that is, while one eye
looks straight ahead, the other eye is turned in toward the
nose
 Esodeviations are the most common type of ocular
misalignment
 Represents 50% of ocular deviations in pediatric age group
Introduction
 Three commonly recognized forms
 Esophoria
 Intermittent Esotropia
 Esotropia
 Latent esodeviation that is controlled by fusional mechanisms
 Eyes remain properly aligned under normal binocular viewing
conditions
Esophoria
 Is an esodeviation that is intermittently controlled by fusional
mechanisms
 Manifest under certain conditions such as fatigue,illness ,stress or
tests that interfere with the maintenance of normal fusional abilities
{such as covering one eye}
Intermittent Esotropia
Esotropia
 Esodeviation that is not controlled by fusional mechanisms so that
deviation is constant
Types of Esotropia
 Congenital infantile esotropia
 Accommodative esotropia
 Acquired non accommodative esotropia
 Cyclic esotropia
 Divergence Insufficiency
 Sensory esotropia
PSEUDOSTRABISMUS
 Wide nasal bridge
 Prominent epicanthal folds
 Narrow interpupillary
distance
 With the formation of the
bridge of the nose, pseudo-
strabismus disappears
Definition & Incidence
 Large angle esotropia present before 6 months of age
 Prevalence: 27/ 10,000
Associated factors
 Essential Congenital esotropia : most common form of
strabismus
 Incidence : 1 to 2 per cent
 Sex 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
 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
Etiology
 WORTH THEORY
 Congenital absence of cortical fusion potential
 Lack of binocular fusion
 CHAVASSE THEORY
Have potential for high grade stereopsis
Primary motor misalignment
Disruption of binocular vision
Clinical Features
 Within first 6 months of life
 Large angle esotropia
 Alternate fixation:
 Target to right, fixates with the left eye
 Target to the left, fixates with the right eye
 Apparent limitation of abduction
Clinical Features
 Associated with vertical divergence & inferior oblique
over-action over the period of time
 Mild to moderate amblyopia
 Small degree of hyperopia
Examination
 Amblyopia assessment
 Check extra ocular movements to rule out VI N
palsy
 Verify abduction deficit with vestibular
stimulation:
 Dolls head phenomenon test
 Rotating the child gently
Examination
If it persists - perform Abduction saccades
 Brisk saccades - LR functioning but restricted
 Absent saccades - weak LR due to VI nerve palsy
 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
~ Fixing eye in adduction
~ Nystagmus on attempted abduction, not adduction
~ Face turn to side of fixing eye
CIANCIA SYNDROME
Congenital Esotropia Observational Study
(CEOS)
 Infantile esotropia < 40 pd and intermittent or variable
frequently resolves spontaneously at less than 20 weeks
of age
 Cases with a constant deviation > 40 pd presenting after
10 weeks of age -- spontaneous resolution less likely
Treatment
Non – Surgical Measures
• Correction of refractive errors
• More than +2.00 D, should be corrected in small
angle cases
• More than +3.00 D, should be corrected in large angle
esotropia
• Miotics in uncooperative infants
• Amblyopia therapy
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 favour early surgery as soon as diagnosis is
made
 Wait upto 6 months of age in intermittent esotropia or
small to moderate angle deviations
Before proceeding to surgery :
 Deviation should be constant and stable
 Fixation should be alternating
 Accomodative element should be absent
 Sensory esotropia should have been ruled out
 Amblyopia should be treated optimally
Surgical Approach
 Bilateral Medial rectus recession
 Max of 8mm recession can be done
 MR recession with lateral rectus (LR ) resection
 Inferior oblique overaction : muscle weakening
procedures
 Alignment within 7-8 Δ of orthophoria: acceptable
Deviations B/l MR
recession
(mm)
Recession
of MR
Resection
of LR
15 3 3 4
20 3.5 3.5 5
30 4.5 4.5 6
40 5.5 5.5 7
50 6 6 8
60 6.5 6.5 9
70 7 7 10
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 refractive errors
Post Surgical Outcomes
Small angle residual esotropia or exotropia
 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
Post Surgical Outcomes
Large angle residual esotropia
 Due to undercorrection
 Esotropia more than 20 D
 Leads to suppresion 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
Post Surgical Outcomes
Large Angle Consecutive Exotropia
 Due to over correction
 In children below 2 years, surgery should be done early to give a
chance for development of peripheral fusion
 In patients above 8 years of age, wait for about 6 to 8 weeks,
surgery only if deviation is more than 20 PD
 Surgical Options
 LR recession with advancement of previously recessed
MR
 Bilateral LR recession
 LR recession + MR resection of the other eye
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
ACQUIRED ESOTROPIAS
 Onset in few years of life
 Insignificant refractive error & accomodative factor
 Three types :
 Basic Esotropia
 Convergence excess esotropia
 Divergence Insuffiency Esotropia
 Therapy : treatment of amblyopia and surgical treatment
of the underlying deviation
 Rule out a CNS lesion in case of acquired esotropia
NYSTAGMUS BLOCKAGE
SYNDROME
 Patients with congenital nystagmus may use
accommodative convergence to dampen their nystagmus
 Have straight eyes with congenital nystagmus on distance
fixation
 But on near fixation, they manifest a variable esodeviation
while using accommodative convergence to dampen the
nystagmus and improve vision
 Nystagmus is inversely proportional to angle of
deviation
 Appears as fixing eye moves from adduction to
abduction
 Fixation occurs with the adducting eye
 Head tilt towards the side of fixating eye
NYSTAGMUS BLOCKAGE
SYNDROME
CYCLIC ESOTROPIA
Acquired esotropia which occurs most frequently
between 2-6 years of age
 Strabismic & non strabismic phase of 24 hrs each
 Lasts for months to years
Strabismic phase :
 Large deviation(40-70PD)in early childhood
 Suppression in deviated eye
Non Strabismic phase :
 No manifest deviation, normal fusion and stereopsis
 These patients are actually esotropic but able to
maintain fusion periodically
 Correcting for the deviation therefore does not
cause over correction on orthophoric days;
patient has enough compensatory divergence
 Not associated with any phoria on normal days
no accommodation / fusional component
CYCLIC ESOTROPIA
Treatment
 B/L medial rectus recession
 MR recession + LR resection
DIVERGENCE PARALYSIS
 Commitant esotropia for distant fixation in patients with
normal ductions & versions
 Idiopathic condition
 CNS disease: polio, neoplasms
Divergence Paralysis
 Sudden onset diplopia at distant fixation
 Commitant esotropia
 Unrestricted field of vision
 Abduction & versions are normal
Treatment:
 Self limiting: disappear in 5 -6 months
 Base out prisms: relieve diplopia
 B/L LR resection if > 6months
SENSORY ESOTROPIA
 Esotropia secondary to poor visual function in one eye in
childhood
 Monocular lesions which prevent normal binocular
vision development
 Congenital cataracts, corneal opacities, retinoblastoma,
optic atrophy
Treatment
 Evaluate the good eye fully for refractive errors
 Treat underlying causes, along with management of
amblyopia before squint surgery
 MR recession +/- LR resection
 Inform patient about recurrence chances
Consecutive esotropia
 Results from over correction of exodeviations
 For deviations <20 P.D. no active management
 Deviations > 20 PD wait for 6 months if no
improvement occurs surgical correction is required
 Use of adjustable sutures recommended in these cases
 In very large esodeviation presenting immediately after
surgery ,it is important to remember muscle detachment
from new site of insertion
Congenital infantile esotropia

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Congenital infantile esotropia

  • 1. { Congenital Infantile Esotropia Presenter - Dr. Om Patel Moderator – Dr. Varshini Shanker
  • 2. Introduction  The term is derived from 2 Greek words: ésò, meaning inward, and trépò, meaning turn  In esotropia, the eyes are crossed; that is, while one eye looks straight ahead, the other eye is turned in toward the nose  Esodeviations are the most common type of ocular misalignment  Represents 50% of ocular deviations in pediatric age group
  • 3. Introduction  Three commonly recognized forms  Esophoria  Intermittent Esotropia  Esotropia
  • 4.  Latent esodeviation that is controlled by fusional mechanisms  Eyes remain properly aligned under normal binocular viewing conditions Esophoria
  • 5.  Is an esodeviation that is intermittently controlled by fusional mechanisms  Manifest under certain conditions such as fatigue,illness ,stress or tests that interfere with the maintenance of normal fusional abilities {such as covering one eye} Intermittent Esotropia
  • 6. Esotropia  Esodeviation that is not controlled by fusional mechanisms so that deviation is constant
  • 7. Types of Esotropia  Congenital infantile esotropia  Accommodative esotropia  Acquired non accommodative esotropia  Cyclic esotropia  Divergence Insufficiency  Sensory esotropia
  • 8. PSEUDOSTRABISMUS  Wide nasal bridge  Prominent epicanthal folds  Narrow interpupillary distance  With the formation of the bridge of the nose, pseudo- strabismus disappears
  • 9. Definition & Incidence  Large angle esotropia present before 6 months of age  Prevalence: 27/ 10,000
  • 10. Associated factors  Essential Congenital esotropia : most common form of strabismus  Incidence : 1 to 2 per cent  Sex 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
  • 11.  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
  • 12. Etiology  WORTH THEORY  Congenital absence of cortical fusion potential  Lack of binocular fusion  CHAVASSE THEORY Have potential for high grade stereopsis Primary motor misalignment Disruption of binocular vision
  • 13. Clinical Features  Within first 6 months of life  Large angle esotropia  Alternate fixation:  Target to right, fixates with the left eye  Target to the left, fixates with the right eye  Apparent limitation of abduction
  • 14. Clinical Features  Associated with vertical divergence & inferior oblique over-action over the period of time  Mild to moderate amblyopia  Small degree of hyperopia
  • 15.
  • 16. Examination  Amblyopia assessment  Check extra ocular movements to rule out VI N palsy  Verify abduction deficit with vestibular stimulation:  Dolls head phenomenon test  Rotating the child gently
  • 17.
  • 18. Examination If it persists - perform Abduction saccades  Brisk saccades - LR functioning but restricted  Absent saccades - weak LR due to VI nerve palsy
  • 19.  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 ~ Fixing eye in adduction ~ Nystagmus on attempted abduction, not adduction ~ Face turn to side of fixing eye CIANCIA SYNDROME
  • 20.
  • 21. Congenital Esotropia Observational Study (CEOS)  Infantile esotropia < 40 pd and intermittent or variable frequently resolves spontaneously at less than 20 weeks of age  Cases with a constant deviation > 40 pd presenting after 10 weeks of age -- spontaneous resolution less likely
  • 22. Treatment Non – Surgical Measures • Correction of refractive errors • More than +2.00 D, should be corrected in small angle cases • More than +3.00 D, should be corrected in large angle esotropia • Miotics in uncooperative infants • Amblyopia therapy
  • 23. 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 favour early surgery as soon as diagnosis is made  Wait upto 6 months of age in intermittent esotropia or small to moderate angle deviations
  • 24. Before proceeding to surgery :  Deviation should be constant and stable  Fixation should be alternating  Accomodative element should be absent  Sensory esotropia should have been ruled out  Amblyopia should be treated optimally
  • 25. Surgical Approach  Bilateral Medial rectus recession  Max of 8mm recession can be done  MR recession with lateral rectus (LR ) resection  Inferior oblique overaction : muscle weakening procedures  Alignment within 7-8 Δ of orthophoria: acceptable
  • 26. Deviations B/l MR recession (mm) Recession of MR Resection of LR 15 3 3 4 20 3.5 3.5 5 30 4.5 4.5 6 40 5.5 5.5 7 50 6 6 8 60 6.5 6.5 9 70 7 7 10
  • 27. 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 refractive errors
  • 28. Post Surgical Outcomes Small angle residual esotropia or exotropia  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
  • 29. Post Surgical Outcomes Large angle residual esotropia  Due to undercorrection  Esotropia more than 20 D  Leads to suppresion 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
  • 30. Post Surgical Outcomes Large Angle Consecutive Exotropia  Due to over correction  In children below 2 years, surgery should be done early to give a chance for development of peripheral fusion  In patients above 8 years of age, wait for about 6 to 8 weeks, surgery only if deviation is more than 20 PD  Surgical Options  LR recession with advancement of previously recessed MR  Bilateral LR recession  LR recession + MR resection of the other eye
  • 31. 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
  • 32. ACQUIRED ESOTROPIAS  Onset in few years of life  Insignificant refractive error & accomodative factor  Three types :  Basic Esotropia  Convergence excess esotropia  Divergence Insuffiency Esotropia  Therapy : treatment of amblyopia and surgical treatment of the underlying deviation  Rule out a CNS lesion in case of acquired esotropia
  • 33. NYSTAGMUS BLOCKAGE SYNDROME  Patients with congenital nystagmus may use accommodative convergence to dampen their nystagmus  Have straight eyes with congenital nystagmus on distance fixation  But on near fixation, they manifest a variable esodeviation while using accommodative convergence to dampen the nystagmus and improve vision
  • 34.  Nystagmus is inversely proportional to angle of deviation  Appears as fixing eye moves from adduction to abduction  Fixation occurs with the adducting eye  Head tilt towards the side of fixating eye NYSTAGMUS BLOCKAGE SYNDROME
  • 35. CYCLIC ESOTROPIA Acquired esotropia which occurs most frequently between 2-6 years of age  Strabismic & non strabismic phase of 24 hrs each  Lasts for months to years Strabismic phase :  Large deviation(40-70PD)in early childhood  Suppression in deviated eye Non Strabismic phase :  No manifest deviation, normal fusion and stereopsis
  • 36.  These patients are actually esotropic but able to maintain fusion periodically  Correcting for the deviation therefore does not cause over correction on orthophoric days; patient has enough compensatory divergence  Not associated with any phoria on normal days no accommodation / fusional component CYCLIC ESOTROPIA
  • 37. Treatment  B/L medial rectus recession  MR recession + LR resection
  • 38. DIVERGENCE PARALYSIS  Commitant esotropia for distant fixation in patients with normal ductions & versions  Idiopathic condition  CNS disease: polio, neoplasms
  • 39. Divergence Paralysis  Sudden onset diplopia at distant fixation  Commitant esotropia  Unrestricted field of vision  Abduction & versions are normal Treatment:  Self limiting: disappear in 5 -6 months  Base out prisms: relieve diplopia  B/L LR resection if > 6months
  • 40. SENSORY ESOTROPIA  Esotropia secondary to poor visual function in one eye in childhood  Monocular lesions which prevent normal binocular vision development  Congenital cataracts, corneal opacities, retinoblastoma, optic atrophy
  • 41. Treatment  Evaluate the good eye fully for refractive errors  Treat underlying causes, along with management of amblyopia before squint surgery  MR recession +/- LR resection  Inform patient about recurrence chances
  • 42. Consecutive esotropia  Results from over correction of exodeviations  For deviations <20 P.D. no active management  Deviations > 20 PD wait for 6 months if no improvement occurs surgical correction is required  Use of adjustable sutures recommended in these cases  In very large esodeviation presenting immediately after surgery ,it is important to remember muscle detachment from new site of insertion