Monofixation Syndrome

Monofixation Syndrome

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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 due to various primary causes.

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

The central retina has small receptive fields and is therefore 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 to the maximum deviation of 8 pd of monofixation syndrome. [1]

Some patients have an inherited inability to bifixate.

United States

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

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

Monofixation syndrome has been shown to remain stable over decades. Among patients who have been monitored for several years, sensory status has improved with higher-grade stereoacuity in a small percentage. A small percentage of patients may have decompensated motor loss over time. [2]

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

Ing MR. Roberts KM. Lin A. Chen JJ. The stability of the monofixation syndrome. Am J Ophthalmol. 2014 Jan. 157 (1):248-253. [Medline].

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

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

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. 1994 Sep-Oct. 31(5):298-301; discussion 302. [Medline].

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

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

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

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

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

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

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

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, American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

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.

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.

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.

Monofixation Syndrome

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