Monofixation Syndrome 

  • Author: Balaji K Gupta, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: May 8, 2012
 

Background

Monofixation syndrome is a form of subnormal binocular vision without bifixation characterized by small-angle strabismus, unilateral absolute facultative central suppression scotoma of less than 3º, and peripheral fusion. While monofixation syndrome can be a primary disorder of binocular vision, it is more commonly a secondary sensory status resulting from a variety of primary causes.

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Pathophysiology

The main defect is a central suppression scotoma, which prevents bifixation.

The central retina has small receptive fields and is more sensitive to image blur or image disparity than the peripheral retina. Conditions that cause a suppression scotoma in the central retina but allow for peripheral fusion cause monofixation syndrome. Studies in macaque monkeys have demonstrated that 2 adjacent neurons in the visual cortex could join receptive fields up to 5°, which correlates very well to the maximum deviation of 8 pd of monofixation syndrome.[1]

Some patients have an inherited inability to bifixate.

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Epidemiology

Frequency

United States

The prevalence of monofixation syndrome in the general population is 1%.

Age

Monofixation syndrome is recognized mainly in children but is present at all ages.

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Contributor Information and Disclosures
Author

Balaji K Gupta, MD  Clinical Assistant Professor, Department of Ophthalmology and Visual Sciences, University of Chicago

Balaji K Gupta, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, and American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor 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, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

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, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

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, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Tychsen L. Can ophthalmologists repair the brain in infantile esotropia? Early surgery, stereopsis, monofixation syndrome, and the legacy of Marshall Parks. J AAPOS. Dec 2005;9(6):510-21. [Medline].

  2. Botet RV, Calhoun JH, Harley RD. Development of monofixation syndrome in congenital esotropia. J Pediatr Ophthalmol Strabismus. Mar-Apr 1981;18(2):49-51. [Medline].

  3. Kushner BJ. The Occurrence of Monofixational Exotropia After Exotropia Surgery. Am J Ophthal. Mar 13 2009;[Medline].

  4. Scott MH, Noble AG, Raymond WR 4th, Parks MM. Prevalence of primary monofixation syndrome in parents of children with congenital esotropia. J Pediatr Ophthalmol Strabismus. Sep-Oct 1994;31(5):298-301; discussion 302. [Medline].

  5. Cibis GW. Video vision development assessment in diagnosis and documentation of microtropia. Binocul Vis Strabismus Q. 2005;20(3):151-8. [Medline].

  6. Arthur BW, Smith JT, Scott WE. Long-term stability of alignment in the monofixation syndrome. J Pediatr Ophthalmol Strabismus. Sep-Oct 1989;26(5):224-31. [Medline].

  7. Hunt MG, Keech RV. Characteristics and course of patients with deteriorated monofixation syndrome. J AAPOS. Dec 2005;9(6):533-6. [Medline].

  8. Choi DG, Isenberg SJ. Vertical strabismus in monofixation syndrome. J AAPOS. Feb 2001;5(1):5-8. [Medline].

  9. Parks MM. The monofixation syndrome. Trans Am Ophthalmol Soc. 1969;67:609-57. [Medline].

  10. Siatkowski RM. The decompensated monofixation syndrome (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc. Dec 2011;109:232-50. [Medline]. [Full Text].

  11. Tomac S. Monofixation syndrome and anisometropia. Ophthalmology. Jan 2002;109(1):3-4. [Medline].

  12. Wright K. Visual development, amblyopia, and sensory adaptations. In: Pediatric Ophthalmology and Strabismus. St Louis, Mo: Mosby; 1995:119-138.

 
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