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Congenital Exotropia

  • Author: Mounir Bashour, MD, PhD, CM, FRCSC, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Mar 22, 2016
 

Background

The term congenital exotropia is typically reserved for patients presenting in the first year of life with a large, constant angle.

However, as Hunter et al (2001) state, no published study provides a rationale for this restrictive definition.[1] In their study, they evaluated differences between infants, aged younger than 1 year, with constant exotropia versus intermittent exotropia at presentation.[1] They found that "half of infantile exotropia patients may present with intermittent exotropia, with similar clinical outcomes regardless of presentation."[1] In their study, surgical intervention resulted in successful alignment in most cases.[1] More than half the patients developed measurable stereopsis, but none achieved bifixation.[1]

In a 2008 study of 12 patients with intermittent exotropia treated with bilateral lateral rectus recession, stereoacuity, as seen in the late follow-up period, measured at 40 arcsec in 2 patients, 100 arcsec in 3 patients, 140-400 arcsec in 2 patients, and none in 5 patients.[2]

True congenital exotropia (with a fixed exotropia) is an extremely rare form of strabismus and may occur with systemic disease in as many as 60% of patients. Patients with craniofacial syndromes, ocular albinism, midline defects, and cerebral palsy may present with congenital exotropia.

See related CME at Highlights of the American Association for Pediatric Ophthalmology and Strabismus Annual Meeting.

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Pathophysiology

The pathophysiology is unknown, although strabismus does occur in families, suggesting a multifactorial autosomal dominant pattern with incomplete penetrance.

A good recent review of risk factors and genetics in common comitant strabismus can be found here.[3]

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Epidemiology

Frequency

United States

Congenital exotropia is extremely rare in the United States.

International

The worldwide incidence of congenital exotropia is unknown.

Mortality/Morbidity

There is a higher incidence of amblyopia in congenital exotropia than in other forms of exotropia.

Race

No known racial predisposition to congenital exotropia exists.

Sex

No known sexual predilection exists.

Age

Congenital exotropia presents in infants younger than 6 months. Children who are born premature are at higher risk of developing strabismus; however, congenital exotropia does not occur at a higher rate in premature children.

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Contributor Information and Disclosures
Author

Mounir Bashour, MD, PhD, CM, FRCSC, FACS Assistant Professor of Ophthalmology, McGill University Faculty of Medicine; Clinical Assistant Professor of Ophthalmology, Sherbrooke University; Medical Director, Cornea Laser and Lasik MD

Mounir Bashour, MD, PhD, CM, FRCSC, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American College of International Physicians, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Mechanical Engineers, American Society of Ophthalmic Plastic and Reconstructive Surgery, Biomedical Engineering Society, Canadian Medical Association, Canadian Ophthalmological Society, Contact Lens Association of Ophthalmologists, International College of Surgeons US Section, Ontario Medical Association, Quebec Medical Association, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Coauthor(s)

C Corina Gerontis, MD Consulting Staff, Departments of Pediatrics and Ophthalmology, Schneider Children's Hospital/Long Island Jewish Medical Center

C Corina Gerontis, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

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.

References
  1. Hunter DG, Kelly JB, Buffenn AN, et al. Long-term outcome of uncomplicated infantile exotropia. J AAPOS. 2001 Dec. 5(6):352-6. [Medline].

  2. Saunders RA, Trivedi RH. Sensory results after lateral rectus muscle recession for intermittent exotropia operated before two years of age. J AAPOS. 2008 Apr. 12(2):132-5. [Medline].

  3. Maconachie GD, Gottlob I, McLean RJ. Risk factors and genetics in common comitant strabismus: a systematic review of the literature. JAMA Ophthalmol. 2013 Sep. 131(9):1179-86. [Medline].

  4. Kim E, Kim JH, Hwang JM, Choi BS, Jung C. MR Imaging of Congenital or Developmental Neuropathic Strabismus: Common and Uncommon Findings. AJNR Am J Neuroradiol. 2012 May 17. [Medline].

  5. Biglan AW, Davis JS, Cheng KP, et al. Infantile exotropia. J Pediatr Ophthalmol Strabismus. 1996 Mar-Apr. 33(2):79-84. [Medline].

  6. Brodsky MC, Baker RS, Hamed LM. Pediatric Neuro-ophthalmology. 1996.

  7. 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].

  8. Matsuo T, Yamane T, Ohtsuki H. Heredity versus abnormalities in pregnancy and delivery as risk factors for different types of comitant strabismus. J Pediatr Ophthalmol Strabismus. 2001 Mar-Apr. 38(2):78-82. [Medline].

  9. Mohney BG, Huffaker RK. Common forms of childhood exotropia. Ophthalmology. 2003 Nov. 110(11):2093-6. [Medline].

  10. von Noorden GK. Binocular Vision and Binocular Motility: Theory and Management of Strabismus. 1996.

  11. Wright KW, Buckley EG, Del Monte MA. Pediatric Ophthalmology and Strabismus. 1995.

 
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