Evidence-based Ophthalmology

Edited by Richard Wormald

Copyright 0 BMJ Publishing Group 2004

13 Infantile esotropia
Lorraine Cassidy, Jugnoo S Rahi

Background
_ _ _ _ ~ ~ _ _ _ Question
Infantile esotropia is the term used to describe a large In children with infantile esotropia does early alignment (six
angle comitant convergent squint with an onset between months or under), compared with alignment at two years,
three and six months of age, commonly associated with result in better binocular single vision, better cosmesis and
hypermetropia, cross fixation, inferior oblique overaction, better long-term alignment?
dissociated vertical deviation, latent nystagmus and
asymmetry of monocular OKN.'i2 It has a prevalence of 1%
and accounts for up to 54%of all esotropias in industrialised The evidence
c~untries.~-~ ~___ ~~~~

Once refractive errors and amblyopia have been treated, We found no randomised controlled trials that address
the main goal of surgical treatment of infantile esotropia is to this question. The Early Surgery for Congenital Esotropia
achieve some degree of binocular single vision (BSV). To Trial (ESCET) is an United States National Institute of
obtain full, normal BSV is rare:* and subnormal or gross Health funded multi-centre, randomised prospective study,
stereopsis is generally considered an acceptable re~ult.'~'~ which is currently underway and results are anticipated in
Gross stereopsis is reported to be achievable in up to 50%of 2004.22123This trial will compare the outcome of children
cases. '-I4 operated on between the ages of 1 1 to 18 weeks with those
Cosmesis is also an important outcome, as squint can operated on between the ages of 28 to 32 weeks. The
have psychosocial implications for the affected child and primary outcome is stereoacuity, and secondary outcomes
parents,I5 particularly if it remains uncorrected into are motor alignment, fusion, dissociated deviations and
adulthood.16 re-operations.
Von Noorden et a/.'7 have proposed the following
taxonomy: Comment
0 "subnormal binocular vision" being the optimal result Observational clinical and laboratory studies suggest that
0 "microtropia" being a desirable result accurate alignment before the age of two years may be more
0 a "residual small angle eso/exotropia" as a cosmetically beneficial than later surgery in terms of achieving
acceptable result b i n ~ c u l a r i t y .In~ ~a ~retrospective
~~~ study of 40 children
0 a large angle eso/exotropia as a cosmetically unacceptable with infantile esotropia, all of whom had surgery aged
result. 22 months or less and achieved alignment at a mean of
2 4 months (range 7 to 48 months), R o ~ reported e ~ ~
that 22.5% achieved binocular vision and 60% achieved a
The issues small angle (<20 prism dioptres) esotropia postoperatively.
Currently two major issues in the management of Nevertheless, surgical alignment after the age of two years
infantile esotropia are the optimal timing for surgical does not preclude the attainment of subnormal binocular
correctionla and the emerging use of botulinum toxin A vision or microtropia.26
(BTXA) as an adjunct or alternative to surgical It has been demonstrated that normal infants show an
alignment. abrupt onset of stereopsis in the time period of three to five
Over the past two decades, injection of botulinum months of age.27As this would require motor alignment, it is
toxin has become established as an important adjunct to increasingly argued that children with infantile esotropia should
surgery and as the primary treatment for some types of undergo surgery as soon as possible. However, observational
squints. It has recently been increasingly used in young data regarding the additional benefits of very early treatment
children. 19-21 in the first year of life are In an

a7
Evidence-based Ophthalmology

analysis of 16 patients, Ing found that those who underwent References

surgery at five months or younger did not have significantly
better binocularity at seven years compared with those 1. Von Noorden GK. A reassessment of infantile esotropia (XLIV Edward
undergoing surgery at older ages3’ Helveston et af.32 Jackson Memorial Lecture). Am/ Ophthalmol1988;105:1-102.
2. Norcia AM. Abnormal motion processing and binocularity; infantile
achieved measurable stereopsis in 40% of infantile esotropes esotropia as a model system for effects of early interruptions of
who were aligned at a mean of 4.4 months. This group also binocularity. Eye 1996; 10:259-65.
demonstrated that very early alignment (age two to four 3. Scobee RG. Esotropia. Incidence, etiology and results of therapy. Am
/ Ophthalmol1951;34:817.
months) did not increase the chance of achieving “fine” 4. Costenbader FD. Infantile esotropia. Trans Am Optical SOC
stereoacuity (i.e. 50 seconds of arc). I96 1;59:397-429.
5. Nordlow W. Age distribution of onset of esotropia. Br/ Ophthalmol
1953;37359.
Question 6. Taylor DM. How early is early surgery in the management of
strabismus? Arch Ophthalmol1963;70:752-6.
7. Parks MM. Congenital esotropia with a bifixation result. Report of a
In children with infantile esotropia does primary treatment case. Doc Ophthalmol1984;58:109-14.
with using botulinum toxin, compared with conventional a. Wright KW, Edelman PM, McVey JH, Terry AP, Liu M. High grade
primary surgical alignment, result in better binocular single stereoacuity after early surgery for congenital esotropia. Arch
vision, better cosmesis and better long-term alignment? Ophthalmol1994;112:913.
9. Ing MR. Early surgical alignment for congenital esotropia. / Pediatr
Ophthalmol Strabismus 1983;20 1 1-1 8.
10. Hiles DA, Watson BA, Biglan AW. Characteristics of infantile eotropia
The evidence following early bimedial rectus recessions. Arch Ophthalmol 1980;
98~697-703.
11. Mohindra I, Zwaan J, Held R, Brill S, Zwaan F. Development of acuity
We found no trials comparing alignment using botulinum and stereopsis in infants with esotropia. Ophthalmology 1985;92:69 I .
toxin with surgery as the primary procedure. 12. Birch EE, Stager DR. Monocular acuity and stereopsis in infantile
esotropia. Invest Ophthalmol Ks Sci 1985;26:1624.
13. Birch EE, Stager DR, Barry P, Everett ME. Prospective assessment of
acuity and stereopsis in amblyopic infantile esotropia following early
Comment surgery. Invest Ophthalmol Ks Sci 1990;31:758-65.
14. Birch EE, Stager DR, Barry P, Everett ME. Random dot stereoacuity
To date, only observational clinical studies suggest that following surgical correction of infantile esotropia. / Pediatr
botulinum toxin may have a role as the primary approach in Ophthalmol Strabismus 1995;32:231-5.
some children with infantile e ~ o t r o p i a .Findings
~~ of a 15. Tolchin JG,Lederman ME. Congenital (infantile) esotropia: psychiatric
aspects. / Pediatr Ophthalmol Strabismus 1978; 15:160-3.
recent trial in children with residual squint following a 16. Oltisky SE, Sudesh S, Graziano A et al. The negative psychosocial
primary surgical procedure suggest that botulinum toxin A impact of strabismus in adults. /AAPOS 1999;3:209-11.
confers greater benefits than a second surgical procedure, as 17. Von Noorden GK, Isaza A, Parks MM. Surgical treatment of
congenital esotropia. Trans AmAcad Ophthalmol Otolaryngol1972;
it is a more rapid and less invasive procedure but with a 76: 1465-78.
similar outcome.34 18. Ing MR. The timing of surgical alignment for congenital (infantile)
esotropia. / Pediatr Ophthalmol Strabismus 1999;36:6 1-8.
19. Robert PY, Jeaneau-Bellego E, Bertin P, Adenis JP. Value of delayed
botulinum toxin injection in esotropia in the child as first line
Implications for practice treatment. /Fr Ophthalmol1998;21:508-14.
20. Schiavi C, Benedetti P, Scorolli L, Campos EC. Nouve indicazioni
all’uso della tossina botulinica. Atti SOC Oflalm Lonbarda
The issue of when to operate remains contentious. I 992;47:345-8.
Whether very early surgery in the first year of life confers 21. Tucker MG, NcNeer KW, Spencer RF. The incidence of latent
nystagmus in infantile esotropia patients treated early with bimedial
benefit needs to be established through ongoing and future botulinum toxin A. Invest Ophthalmol Ks Sci 1997;38:S112.
trials. The results of the ESCET trial should provide 22. Birch E, Stager D, Wright K, Beck R. The natural history of infantile
information that is currently lacking. Observational and esotropia during the first six months of life. Pediatric Eye Disease
Investigator Group. /AAPOS 1998;2:325-8.
laboratory evidence suggests a benefit of surgical alignment 23. Spiegel PH, Wright KW. Optimum timing for surgery for congenital
at age two years or under. Equally, the increasing interest in esotropia. Semin Ophthalmol1997;12: 166-70.
botulinum toxin as an alternative or adjunct to surgery 24. Taylor DM. Is congenital esotropia functionally curable? Trans Am
Optical SOCI972;70:529-76.
requires investigation through trials. 25. Rowe FJ. Long-term postoperative stability in infantile esotropia.
Strabismus2000;8:3-13.
26. Shauly Y, Prager TC, Mazow ML. Clinical characteristics and long-
term postoperative results of infantile esotropia. Am / Ophthalmol
Implications for research 1994;117:183-9.
27. Birch E. Stereopsis in infants and its development ratio to visual
acuity. In: Simon SK, ed. Ear& Ksual Development Normal
For both questions, long-term alignment and patient- and Abnormal. New York Oxford University Press, 1993:
based, subjective measures of outcome should be addressed. pp. 224-36.
 Infantile esotropia

28. Zak TA, Morin D. Early surgery for infantile esotropia: results 32. Helveston EM, Neely DF, Stidham DB, Wallace DK, Plager DA, Spru.
and influence of age upon results. Can J Ophthalmol 1982;17: Results of early alignment of congenital esotropia. Ophthalmology
21 3-1 8. l999;106: 1716-26.
29. Clarke WN. Very early v. early or late surgery for infantile esotropia. 33. McNeer KW, Tucker M, Spencer RF. Management of essential
Can J Ophthalmol1995;30:240-1. infantile esotropia with botulinum toxin A: review and
30. Nixon RB, Helveston EM, Miller K, Archer SM, Ellis FD. Incidence of recommendations. J Pediatr Ophthalmol Strabismus2000;3763-7.
strabismus in neonates. A m / Ophthalmol1985;100:798-801. 34. Teiedor J, Rodriguez JM. Early retreatment of infantile esotropia:
3 1. Ing MR. Outcome of surgical alignment before six months of age for comparison of reoperation and botulinum toxin. 5r J Ophthalmol
congenital esotropia. Ophthalmology1995;102:2041-5. I 999;a3:783-7.

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