Infantile Esotropia: Background, Pathophysiology, Epidemiology

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Sections Infantile Esotropia
Overview
Presentation
- History
- Physical
- Causes
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DDx
Workup
Treatment
Medication
Follow-up
References

Overview

Background

Strabismus is one of the most relevant health problems of the world, and infantile esotropia is perhaps the most visually significant yet the least understood. Infantile esotropia is the inward deviation of the eyes noted before the patient reaches age 6 months. It is associated with maldevelopment of stereopsis, motion processing, and eye movements. Amblyopia is a frequent consequence of infantile esotropia. To date, its exact cause has yet to be identified, and an effective treatment strategy is yet to be formulated.

Pathophysiology

The exact cause of infantile esotropia remains unknown. While some opine that esotropia is due to excessive tonic convergence, few agree on what accounts for such conditions. Worth strongly believed that esotropia is an inborn and irreversible defect of fusion. As such, it is a primary dysfunction in the normal development of binocular sensitivity. This was countered by Chavasse who asserted that the neural components necessary for normal binocular vision are present in strabismic individuals at birth, but the development of fusion is eventually impeded by abnormalities of optical input (eg, monocular cataracts) or muscular output (eg, cranial nerve palsies).

The origins of infantile esotropia are just as undefined. A few authors have implicated practically everything from and between the extraocular muscles to the visual cortex in the causation of infantile esotropia.

Although understanding the mechanisms behind infantile esotropia has come a long way, there is still a lot of ground to cover to unearth and clearly understand such an elusive condition.

Frequency

United States

Strabismus is one of the most prevalent ocular problems among children, affecting 5 in every 100 US citizens, or some 12 million people in a population of 245 million. Infantile esotropia accounts for 28-54% of all esotropias. A population-based study from 1965 to 1994 reports the birth prevalence of infantile esotropia to be 25 per 10,000 or 1 in 403 live births.^[1]It is thought to affect about 1% of full-term, healthy newborns and a much higher percentage of newborns with perinatal complications due to prematurity or hypoxic/ischemic encephalopathy.

In an attempt to determine whether esotropia is present at birth or develops later in infancy, Nixon et al observed 1,219 alert infants in a normal newborn nursery at a city hospital and noted that only 40 babies (3.2%) had esotropia (intermittent esotropia in 17 patients, with 14 patients varying between esotropia and exotropia, and 9 patients with variable esotropia). In addition, no infant displayed characteristic features of infantile esotropia.^[2]As such, infantile esotropia is not believed to be connatal but rather develops in the first few weeks or months after birth.

Greenberg et al reported an annual age- and gender-adjusted childhood esotropia incidence of 111 per 100,000 patients younger than 19 years.^[3]This rate corresponds to a cumulative prevalence of approximately 2% of all children younger than 6 years, with a significant decrease in older ages. The incidence of childhood esotropia from this population-based study is comparable to the prevalence rates among Western populations. Esotropia is most common during the first decade of life, with the accommodative and acquired nonaccommodative forms occurring most frequently.

Mortality/Morbidity

Exotropia in infancy is believed to be associated with an increased prevalence of coexisting neurologic, ocular, and craniofacial abnormalities. To a lesser degree, infantile esotropia also has been associated with a high prevalence of systemic disorders, including prematurity, neurologic, and genetic disorders. Reports of coexisting brain lesions (eg, periventricular leukomalacia, enlargement of the lateral ventricles with hypoplasia of the corpus callosum, myelination delay at the anterior horn adjacent of the lateral ventricles) have been published.

Age

By definition, infantile esotropia is seen in infants before age 6 months.

Clinical Presentation

Louwagie CR, Diehl NN, Greenberg AE, Mohney BG. Is the incidence of infantile esotropia declining?: a population-based study from Olmsted County, Minnesota, 1965 to 1994. Arch Ophthalmol. 2009 Feb. 127(2):200-3. [Medline]. [Full Text].
Nixon RB, Helveston EM, Miller K, et al. Incidence of strabismus in neonates. Am J Ophthalmol. 1985 Dec 15. 100(6):798-801. [Medline].
Greenberg AE, Mohney BG, Diehl NN, Burke JP. Incidence and types of childhood esotropia: a population-based study. Ophthalmology. 2007 Jan. 114(1):170-4. [Medline].
Tychsen L. Infantile esotropia: Current neurophysiologic concepts. In: Clinical Strabismus Management: Principles and Surgical Techniques. 1999:117-138.
Weakley DR, Urso RG, Dias CL. Asymmetric inferior oblique overaction and its association with amblyopia in esotropia. Ophthalmology. 1992 Apr. 99(4):590-3. [Medline].
Mohindra I, Zwaan J, Held R, et al. Development of acuity and stereopsis in infants with esotropia. Ophthalmology. 1985 May. 92(5):691-7. [Medline].
Haefliger IO, Safran AB, Mermillod B, Roth A. Inferonasal quadrant of the visual field is not constricted in patients with infantile esotropia when evaluated by means of automated perimetry. J Clin Neuroophthalmol. 1990 Jun. 10(2):118-20. [Medline].
Khan AO, Shinwari J, Al Sharif L, Khalil D, Al-Gehedan S, Tassan NA. Infantile esotropia could be oligogenic and allelic with Duane retraction syndrome. Mol Vis. 2011. 17:1997-2002. [Medline]. [Full Text].
McNeer KW, Spencer RF, Tucker MG. Observations on bilateral simultaneous botulinum toxin injection in infantile esotropia. J Pediatr Ophthalmol Strabismus. 1994 Jul-Aug. 31(4):214-9. [Medline].
McNeer KW, Tucker MG, Spencer RF. Botulinum toxin management of essential infantile esotropia in children. Arch Ophthalmol. 1997 Nov. 115(11):1411-8. [Medline].
Scott AB, Magoon EH, McNeer KW, Stager DR. Botulinum treatment of childhood strabismus. Ophthalmology. 1990 Nov. 97(11):1434-8. [Medline].
Tejedor J, Rodriguez JM. Early retreatment of infantile esotropia: comparison of reoperation and botulinum toxin. Br J Ophthalmol. 1999 Jul. 83(7):783-7. [Medline].
Ing MR. Botulinum alignment for congenital esotropia. Ophthalmology. 1993 Mar. 100(3):318-22. [Medline].
Biglan AW, Burnstine RA, Rogers GL, Saunders RA. Management of strabismus with botulinum A toxin. Ophthalmology. 1989 Jul. 96(7):935-43. [Medline].
Rowe FJ, Noonan CP. Botulinum toxin for the treatment of strabismus. Cochrane Database Syst Rev. 2009 Apr 15. CD006499. [Medline].
de Alba Campomanes AG, Binenbaum G, Campomanes Eguiarte G. Comparison of botulinum toxin with surgery as primary treatment for infantile esotropia. J AAPOS. 2010 Apr. 14(2):111-6. [Medline].
Gursoy H, Basmak H, Sahin A, Yildirim N, Aydin Y, Colak E. Long-term follow-up of bilateral botulinum toxin injections versus bilateral recessions of the medial rectus muscles for treatment of infantile esotropia. J AAPOS. 2012 Jun. 16(3):269-73. [Medline].
Donahue SP. Botulinum toxin treatment for esotropia. Am Orthopt J. 2013. 63:29-31. [Medline].
Lueder GT, Galli M, Tychsen L, Yildirim C, Pegado V. Long-term results of botulinum toxin-augmented medial rectus recessions for large-angle infantile esotropia. Am J Ophthalmol. 2012 Mar. 153(3):560-3. [Medline].
Polling JR, Eijkemans MJ, Esser J, Gilles U, Kolling GH, Schulz E, et al. A randomised comparison of bilateral recession versus unilateral recession-resection as surgery for infantile esotropia. Br J Ophthalmol. 2009 Jul. 93(7):954-7. [Medline].
Spierer O, Spierer A. Comparison of hang-back and conventional bimedial rectus recession in infantile esotropia. Graefes Arch Clin Exp Ophthalmol. 2010 Jun. 248(6):901-5. [Medline].
Bayramlar H, Karadag R, Yildirim A, Oçal A, Sari U, Dag Y. Medium-term outcomes of three horizontal muscle surgery in large-angle infantile esotropia. J Pediatr Ophthalmol Strabismus. 2014 May-Jun. 51(3):160-4. [Medline].
Chang YH, Ryu IH, Han SH, et al. Intraoperative adjustment in strabismus surgery under topical anesthesia. Yonsei Med J. 2006 Oct 31. 47(5):667-71. [Medline].
Simonsz HJ, Kolling GH, Unnebrink K. Final report of the early vs. late infantile strabismus surgery study (ELISSS), a controlled, prospective, multicenter study. Strabismus. 2005 Dec. 13(4):169-99. [Medline].
Zak TA, Morin JD. Early surgery for infantile esotropia: results and influence of age upon results. Can J Ophthalmol. 1982 Oct. 17(5):213-8. [Medline].
Yagasaki T, Yokoyama YO, Maeda M. Influence of timing of initial surgery for infantile esotropia on the severity of dissociated vertical deviation. Jpn J Ophthalmol. 2011 Jul. 55(4):383-8. [Medline].
Wong AM. Timing of surgery for infantile esotropia: sensory and motor outcomes. Can J Ophthalmol. 2008 Dec. 43(6):643-51. [Medline].
Shirabe H, Mori Y, Dogru M, Yamamoto M. Early surgery for infantile esotropia. Br J Ophthalmol. 2000 May. 84(5):536-8. [Medline].
Birch EE, Fawcett S, Stager DR. Why does early surgical alignment improve stereoacuity outcomes in infantile esotropia?. J AAPOS. 2000 Feb. 4(1):10-4. [Medline].
Çerman E, Eraslan M, Ögüt MS. The relationship of age when motor alignment is achieved and the subsequent development of stereopsis in infantile esotropia. J AAPOS. 2014 Jun. 18(3):222-5. [Medline].
Lueder GT, Norman AA. Strabismus surgery for elimination of bifocals in accommodative esotropia. Am J Ophthalmol. 2006 Oct. 142(4):632-5. [Medline].
Ticho BH, Ticho KE, Kaufman LM. Combined strabismus and lens surgery. J AAPOS. 2006 Oct. 10(5):430-4. [Medline].
Godts D, Trau R, Tassignon MJ. Effect of refractive surgery on binocular vision and ocular alignment in patients with manifest or intermittent strabismus. Br J Ophthalmol. 2006 Nov. 90(11):1410-3. [Medline].
Sarwar H, Waqar S. Surgery for infantile esotropia: is timing everything?. J Perioper Pract. 2013 May. 23(5):107-9. [Medline].
Uretmen O, Civan BB, Kose S, Yuce B, Egrilmez S. Accommodative esotropia following surgical treatment of infantile esotropia: frequency and risk factors. Acta Ophthalmol. 2008 May. 86(3):279-83. [Medline].
Birch E, Stager D, Wright K, Beck R. The natural history of infantile esotropia during the first six months of life. Pediatric Eye Disease Investigator Group. J AAPOS. 1998 Dec. 2(6):325-8; discussion 329. [Medline].
Birch EE, Stager DR. Monocular acuity and stereopsis in infantile esotropia. Invest Ophthalmol Vis Sci. 1985 Nov. 26(11):1624-30. [Medline].
Clarke WN, Noel LP. Vanishing infantile esotropia. Can J Ophthalmol. 1982 Jun. 17(3):100-2. [Medline].
Drover JR, Stager DR Sr, Morale SE, Leffler JN, Birch EE. Improvement in motor development following surgery for infantile esotropia. J AAPOS. 2008 Apr. 12(2):136-40. [Medline]. [Full Text].
Friendly DS. Management of infantile esotropia. Int Ophthalmol Clin. 1985. 25(4):37-52. [Medline].
Gerth C, Mirabella G, Li X, Wright T, Westall C, Colpa L, et al. Timing of surgery for infantile esotropia in humans: effects on cortical motion visual evoked responses. Inv Ophth Vis Sci. Aug 2008. 49(8):3432-7. [Full Text].
Hiles DA, Watson BA, Biglan AW. Characteristics of infantile esotropia following early bimedial rectus recession. Arch Ophthalmol. 1980 Apr. 98(4):697-703. [Medline].
Hunter DG, Ellis FJ. Prevalence of systemic and ocular disease in infantile exotropia: comparison with infantile esotropia. Ophthalmology. 1999 Oct. 106(10):1951-6. [Medline].
Ing MR. The timing of surgical alignment for congenital (infantile) esotropia. J Pediatr Ophthalmol Strabismus. 1999 Mar-Apr. 36(2):61-8; quiz 85-6. [Medline].
Khan AO. Cycloplegic refractions as a function of age in children with infantile esotropia. Binocul Vis Strabismus Q. 2009. 24(1):39-42. [Medline].
Lueder GT, Galli ML. Effect of preoperative stability of alignment on outcome of strabismus surgery for infantile esotropia. J AAPOS. 2008 Feb. 12(1):66-8. [Medline].
Major A, Maples WC, Toomey S, DeRosier W, Gahn D. Variables associated with the incidence of infantile esotropia. Optometry. 2007 Oct. 78(10):534-41. [Medline].
O'Keefe M, Abdulla N, Bowell R, Lanigan B. Binocular function and amblyopia after early surgery in infantile eosotropia. Acta Ophthalmol Scand. 1996 Oct. 74(5):461-2. [Medline].
Ohtsuki H, Yoshifumi K, Hasebe S, et al. Comparative study of brain lesions detected by magnetic resonance imaging between strabismus and nonstrabismus in infancy. Ophthalmologica. 2000. 214(2):105-10. [Medline].
Paul TO, Hardage LK. The heritability of strabismus. Ophthalmic Genet. 1994 Mar. 15(1):1-18. [Medline].
Pott JW, Sprunger DT, Helveston EM. Infantile esotropia in very low birth weight (VLBW) children. Strabismus. 1999 Jun. 7(2):97-102. [Medline].
Pratt-Johnson JA, Tillson G. Sensory results following treatment of infantile esotropia. Can J Ophthalmol. 1983 Jun. 18(4):175-7. [Medline].
Prieto-Diaz J, Prieto-Diaz I. Long term outcome of treated congenital/infantile esotropia: does early surgical binocular alignment restoring (subnormal) binocular vision guarantee stability?. Binocul Vis Strabismus Q. 1998. 13(4):249-54. [Medline].
Robb RM, Rodier DW. The variable clinical characteristics and course of early infantile esotropia. J Pediatr Ophthalmol Strabismus. 1987 Nov-Dec. 24(6):276-81. [Medline].
Rowe FJ. Long-term postoperative stability in infantile esotropia. Strabismus. 2000 Mar. 8(1):3-13. [Medline].
Scheiman M, Ciner E, Gallaway M. Surgical success rates in infantile esotropia. J Am Optom Assoc. 1989 Jan. 60(1):22-31. [Medline].
Shauly Y, Miller B, Meyer E. Clinical characteristics and long-term postoperative results of infantile esotropia and myopia. J Pediatr Ophthalmol Strabismus. 1997 Nov-Dec. 34(6):357-64. [Medline].
Shauly Y, Prager TC, Mazow ML. Clinical characteristics and long-term postoperative results of infantile esotropia. Am J Ophthalmol. 1994 Feb 15. 117(2):183-9. [Medline].
Simonsz HJ, Eijkemans MJ. Predictive value of age, angle, and refraction on rate of reoperation and rate of spontaneous resolution in infantile esotropia. Strabismus. 2010 Sep. 18(3):87-97. [Medline].
Stager DR, Birch EE. Preferential-looking acuity and stereopsis in infantile esotropia. J Pediatr Ophthalmol Strabismus. 1986 Jul-Aug. 23(4):160-5. [Medline].
Tolun H, Dikici K, Ozkiris A. Long-term results of bimedial rectus recessions in infantile esotropia. J Pediatr Ophthalmol Strabismus. 1999 Jul-Aug. 36(4):201-5. [Medline].
Webber AL, Wood JM, Gole GA, Brown B. The effect of amblyopia on fine motor skills in children. Invest Ophthalmol Vis Sci. 2008 Feb. 49(2):594-603. [Medline].
Wong AM. Timing of surgery for infantile esotropia: sensory and motor outcomes. Can J Ophthalmol. 2008 Dec. 43(6):643-51. [Medline].

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Author

Vicente Victor D Ocampo, Jr, MD Head, Uveitis and Ocular Immunology Service, Veterans Memorial Medical Center, Philippines; Head, Uveitis and Ocular Immunology Service, Ospital ng Makati Medical Center, Philippines; Consulting Staff, Department of Ophthalmology, Asian Hospital and Medical Center, Philippines

Vicente Victor D Ocampo, Jr, MD is a member of the following medical societies: American Academy of Ophthalmology, Philippine Ocular Inflammation Society, Philippine Academy of Ophthalmology

Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, Sigma Xi

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Aldeyra Therapeutics (Lexington, MA); Bausch & Lomb Surgical, Inc (Rancho Cucamonga, CA); Eyegate Pharma (Waltham, MA); Novartis (Cambridge, MA); pSivida (Watertown, MA); Xoma (Berkeley, CA)<br/>Received research grant from: Alcon; Aldeyra Therapeutics; Allergan; Bausch & Lomb; Clearside Biomedical; Dompé pharmaceutical; Eyegate Pharma; Mallinckrodt pharmaceuticals; Novartis; pSivida; Santen.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Chief Editor

Donny W Suh, MD, FAAP Chief of Pediatric Ophthalmology and Strabismus, Children's Hospital and Medical Center; Associate Professor, Department of Ophthalmology and Visual Sciences, Truhlsen Eye Institute, University of Nebraska Medical Center

Donny W Suh, MD, FAAP is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association, Iowa Medical Society, National Eye Care Project

Disclosure: Received research grant from: PEDIG/NIH.

Additional Contributors

Gerhard W Cibis, MD Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Ophthalmological Society

Disclosure: Nothing to disclose.

Sections Infantile Esotropia
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Medication
Follow-up
References

Infantile Esotropia

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