Jonathan Salvin
BASICS
DESCRIPTION
• A large angle (>35 prism diopters) esotropia developing within the first 6 months of life in an otherwise normal infant with no significant refractive error and no limitation of ocular movements.
– Associated with lower incidence of amblyopia at presentation.
– Associated with secondary findings of inferior oblique overaction, latent nystagmus, dissociated vertical deviation (DVD); often as later findings.
EPIDEMIOLOGY
(1)[A]
• 8% of all childhood esotropia
– Esotropia (all) has an incidence of 111 per 100,000 under the age of 19 years
Prevalence
2% of all children under the age of 6 years
RISK FACTORS
Family history of strabismus
PATHOPHYSIOLOGY
• Studies in primates reveal that early abnormal binocular sensory input results in the creation of infantile esotropia and its related complications
– Early restoration of normal binocular function resulted in improved alignment and decreased incidence of late findings (2)
COMMONLY ASSOCIATED CONDITIONS
• Amblyopia inferior oblique overaction
– Latent nystagmus
– Dissociated vertical deviations
– Cross fixation and pseudoabduction deficit
DIAGNOSIS
HISTORY
• Onset of inward crossing of the eyes seen at birth or soon thereafter (under the age of 6 months)
– Full extraocular movements
– Usually early cross-fixation
PHYSICAL EXAM
• Large angle of esotropia, usually over 35 prism diopters.
• Constant and comitant angle of deviation
• Refractive error usually <+2 diopters (normal for this age group).
• Ophthalmic examination is otherwise normal.
• Fixation may alternate. A lack of alternation may indicate amblyopia is present.
• Fixation with the crossed eye may suggest a lateral rectus underaction. Full abduction can be demonstrated by covering the fixating eye or Doll’s eye maneuver. (3)[A]
DIAGNOSTIC TESTS & INTERPRETATION
Imaging
Neuroimaging may be indicated if esotropia associated with abnormal ductions/versions or manifest nystagmus.
DIFFERENTIAL DIAGNOSIS
• Pseudoesotropia
– Duane syndrome Type 1 (4)
– Moebius syndrome (5)
– Congenital sixth nerve palsy
– Orbital tumor
– Nystagmus blockage syndrome
– Cianca syndrome
TREATMENT
ADDITIONAL TREATMENT
General Measures
• Strabismus surgery (6)[A]. Usually bimedial rectus recession, although larger deviations may be managed by additional lateral rectus recession
• Amblyopia treatment as indicated
Additional Therapies
• Treat underlying amblyopia if present, generally prior to surgery
• Treat high hyperopia to rule out accommodative component
SURGERY/OTHER PROCEDURES
• Alignment of the eyes should be achieved prior to 24 months of age to achieve binocularity. (3)[A], (7)[A]
• Some data suggests that early surgery is beneficial.
• NOTE: Spontaneous resolution without surgery may rarely occur.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
• Long term follow-up for recurrent strabismus, inferior oblique overaction, and DVD all of which may require later surgery
– Second strabismus surgery needed in up to 50% of patients by the age of 10 years
Patient Monitoring
• Monitor for amblyopia and refractive errors
• Secondary accommodative component may develop and need to be treated with glasses
– Monitor for recurrent/consecutive strabismus
– Monitor for late complications
PATIENT EDUCATION
• Long-term monitoring for recurrent strabismus or late associated findings
– Polycarbonate safety glasses required if dense amblyopia remains
PROGNOSIS
• Usually normal vision in both eyes
– Poorly developed stereoacuity without early intervention
COMPLICATIONS
• Recurrent or consecutive strabismus may develop and require additional surgical treatment
• Amblyopia
REFERENCES
1. Greenberg AE, Mohney BG, Diehl NN, et al. Incidence and types of childhood esotropia. A population-based study. Ophthalmology 2007;114:170–174.
2. Wong AMF, Foeller P, Bradley D, et al. Early versus delayed repair of infantile strabismus in macaque monkeys: I. ocular motor effects. J AAPOS 2003;7:200.
3. Pediatric Eye Disease Investigator Group. The clinical spectrum of early-onset esotropia: Experience of the Congenital Esotropia Observational Study. Am J Ophthalmol 2002;133(1):102–108.
4. Alexandrakis G, Saunders RA. Duane retraction syndrome. Ophthlmol Clin North Am 2001;14(3):407–417f
5. Miller MT, Strömland K. The Möbius sequence: A relook. JAAPOS 1999;3(4):199–208.
6. Ing M, Costenbader FD, Parks MM, et al. Early surgery for congenital esotropia. Am J Ophthalmol 1966;61(6):1419–1427.
7. Louwagie CR, Diehl N, Geenberg AE, et al. Long-term follow-up of congenital esotropia in a population-based cohort. J AAPOS 2009;13:8–12.
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