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Infantile (Congenital) Esotropia

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Title: Infantile (Congenital) Esotropia
Author: Madison Perchik, BS, 4th Year Medical Student at the University of Tennessee College of Medicine
Author Email: madisonperchik1@gmail.com
Date: 08/28/2022
Keywords/Main Subjects: Strabismus, Eye Misalignment, Pediatric Ophthalmology, Esotropia, Pediatric Strabismus, Large Angle Esotropia, Eye Turn, Crossed Eye 

Diagnosis: Infantile Esotropia

Overview:  

Infantile esotropia is the inward turning of one or both eyes confirmed before 6 months of age in a child with no other neurological problems. This condition is also commonly referred to as congenital esotropia, but many children are not born with the esotropia, so infantile esotropia is a more appropriate term. Infantile esotropia is defined by a large, constant inward angle of the affected eye(s) (>30 prism diopters)1. Cross-fixation is common in infantile esotropia, which occurs when the left eye is used for right gaze and the right eye is used for left gaze. This eliminates the need for the constantly adducted eye to abduct. However, abduction of the affected eye can be elicited on both doll’s head maneuver and with patching of the opposite eye. The refractive errors of children with infantile esotropia are similar to children of the same age with normal eye alignment. Amblyopia is a serious and common consequence of untreated infantile esotropia2. Associated sensorimotor symptoms of infantile esotropia include dissociated vertical deviation (DVD), inferior oblique overaction, latent nystagmus, and optokinetic asymmetry3.
 

Etiology:  

Infantile esotropia has an incidence that ranges from 1 in 100 to 500 live births4,5.  The cause of infantile esotropia is still debated. In the early 20th century, Worth proposed that it is a congenital defect in the visual pathway that prevents fusion, which meant that correction would not allow children affected to achieve good binocular vision. In this case, surgical correction would not be urgent and would be mostly for cosmetic purposes6. A later theory from Chavasse suggested that the brain does have the ability to fuse, but motor dysfunction causes eye misalignment, which prevents the development of fusion capacity and stereopsis. In this theory, early surgical intervention could lead to greater potential for fusion and stereopsis development7. More recent studies have supported Chavasse’s theory, with early intervention leading to a positive sensory fusion outcome8,9. Although a specific hereditary pattern has not been clearly defined, it is common for children with infantile esotropia to have a family history of strabismus10. Children who are preterm or had low birth weights are also more likely to develop infantile esotropia11.

Evaluation:  

Important history from the family includes the following:  

• Birth weight, term/preterm, complications of birth, developmental milestones 
• Any other past medical history, especially neurological conditions 
• Review photographs taken by family if available to help determine age of onset 

• Timing: Is the deviation constant or intermittent? 
• Characteristics: Does it always turn inwards or sometimes outwards? 
• Changes in the deviation: Has the angle become progressively larger? 
• Any additional symptoms? 
• Family history of strabismus 

Cover testing if possible 

Corneal light reflex: Hirschberg and Krimsky methods helpful in estimating the angle of esotropia especially when cover testing not possible 

Evaluate for amblyopia by determining if child has preference for fixating with one eye 

Dilated exam: 

• Refractive error. If the child has a normal refractive error for his/her age, then accommodative esotropia is unlikely. If the child’s refractive error is greater than +3.00 diopters, then further work-up needs to determine if this is true infantile esotropia or accommodative esotropia12 

• Retina exam to exclude posterior causes of esotropia, such as retinoblastoma13
  

Management:  

While eye muscle surgery is the most effective treatment of infantile esotropia, Botox injections have been suggested as monotherapy for small to medium angle esotropia or as combination therapy with eye muscle surgery14. The optimal timing of surgical intervention is undecided, but there is growing evidence that early surgical intervention (<11 months) is beneficial for both sensory and motor ocular outcomes15. Additionally, multiple studies have concluded that there are no adverse outcomes long-term caused by early surgical intervention16,17. The most common surgical intervention to treat infantile esotropia is medial rectus recession. An alternate approach is to do a medial rectus recession and an ipsilateral lateral rectus resection17. The most common complications of these surgeries is undercorrection or overcorrection. Parents should be advised that multiple surgeries could be necessary. The goal of surgery is to achieve orthotropia or get as close as possible in the hopes of gaining some sensory fusion and eliminating the risk of amblyopia.
 

References:  

1. Costenbader FD. Infantile esotropia. Trans Am Ophthalmol Soc. 1961; 59:397-429. 
2. Kinori M, Robbins SL. Esotropia. Ophthalmology, Fifth Edition. 11.6, 1210-1216.e1 
3. Hiles DA, Watson A, Biglan AW. Characteristics of infantile esotropia following early bimedial rectus recession. Arch ophthalmol 1980;98:697-703. 
4. Greenberg AE, Mohney BG, Diehl NN, Burke JP. Incidence and types of childhood esotropia: a population-based study. Ophthalmology. 2007;114:170–4. 
5. Mohney BG, Erie JC, Hodge DO, Jacobsen SJ. Congenital esotropia in Olmsted County, Minnesota. Ophthalmology. 1998;105:846–50.  
6. Worth C. squint, its causes and treatment. London: Bailliere, Tindall, and Cox, 1903. 
7. Chavasse FB. Worth’s squint on the binocular reflexes and the treatment of strabismus, 7th ed. Philadelphia: P.Blakiston’s Son & Co, 1939. 
8. Wong AMF. Timing of surgery for infantile esotropia: sensory and motor outcomes. Can J Ophthalmol. 2008; 43(6): 643-651. 
9. Ing MR. Early surgical alignment for congenital esotropia. Trans Am Ophthalmol Soc. 1981; 79:625-663. 
10. Issaho, DC et al. Genetic inheritance in non-syndromic infantile esotropia. Biomed J Sci & Tech Res. 2018. 
11. Gulati S, Andrews CA, Apkarian AO, Musch DC, Lee PP, Stein JD. Effect of gestational age and birth weight on the risk of strabismus among premature infants. JAMA Pediatr. 2014; 168(9):850-856. 
12. Pollard ZF. Accommodative esotropia during the first year of life. Arch Ophthalmol. 1976 Nov;94(11):1912–1913. 
13. Kiernan M, Fabian I, Smith V, Sagooo M, Reddy M. Strabismus as a presenting sign of retinoblastoma. Journal of Pediatric Ophthalmology and Strabismus. 2021; 58 (5). 
14. McNeer KW, Spencer RF, Tucker MG. Observations on bilateral simultaneous botulinum toxin injection in infantile esotropia. J Pediatr Ophthalmol Strabismus. 1994; 31(4):214-219. 
15. Birch EE, Stager DR., Sr Long-term motor and sensory outcomes after early surgery for infantile esotropia. J AAPOS. 2006;10:409–13. 
16. Birch EE, Felius J, Stager DR, Sr, Weakley DR, Jr, Bosworth RG. Pre-operative stability of infantile esotropia and post-operative outcome. 
17. Buckley EG, Plager DA, Repka MX, Wilson ME. Plager DA, ed. Contributions by Parks MM, von Noorden GK. Ophthalmology Monographs 17. Oxford: Oxford University Press. 2004. 

Identifier: Moran_CORE_126091
Faculty Approval by: Dr. Marielle Young
Copyright statement: Copyright Madison Perchik, ©2022. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/