Monofixation Syndrome Clinical Presentation

Updated: Apr 27, 2016
  • Author: Balaji K Gupta, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

Because monofixation syndrome is an asymptomatic condition, the diagnosis is often based on the physical examination.

Patients should be asked whether they have a history of muscle surgery, amblyopia, or retinal disease, as well as a family history of strabismus.

Adults with subnormal stereovision, mild anisometropia, and subtle asymmetry in best-corrected vision may have undiagnosed monofixation syndrome.

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Physical

See the list below:

  • Prism cover tests
    • Patients should display less than 8 prism diopters of heterotropia with a simultaneous prism cover test.
    • Alternate prism cover testing may result in 2-3 times greater measurement than simultaneous prism cover testing due to fusional vergence mechanisms.
    • Up to one third of patients have no deviation with alternate cover.
  • Stereoacuity tests
    • Patients with monofixation syndrome have reduced stereopsis, ranging from 300 arc seconds to 67 arc seconds.
    • Stereopsis tends to be better at near than at distance.
  • Central suppression scotoma tests
  • Worth 4-dot test
    • At near (one-third meter), the standard flashlight tests peripheral fusion by subtending 6 º of the retina.
    • When performed at a distance of 6 meters, the patient with monofixation syndrome should only visualize from one eye because the target subtends only 1.25 º and slips into the suppression scotoma.
  • 4-Prism diopter base-out test
    • While having the patient read letters at 20 feet, a 4-prism diopter base-out is placed over one eye.
    • This is repeated on the other eye.
    • The test is positive when the eye that is suspected of having a suppression scotoma does not move after being covered with the prism.
    • This test has a high number of false negatives, especially if no manifest deviation is present.
  • Bagolini lenses
    • Lenses are placed over the eyes, and the patient views a small handlight that is located one-third meter away in a normally lit room.
    • The eye with the suppression scotoma should have a line with a central interrupted portion.
  • Binocular visual fields
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Causes

Corrected strabismus

Patients with corrected strabismus comprise the largest subgroup of patients with monofixation syndrome seen in clinical practice.

Patients who are surgically treated for esotropia more commonly develop monofixation syndrome than exotropia.

Of patients with congenital esotropia, 40-50% will develop monofixation syndrome if corrected to less than 8 prism diopters of deviation by age 2 years. [3]

Late correction dramatically decreases the chances of developing monofixation syndrome.

Patients with congenital esotropia who develop monofixation syndrome are almost twice as likely to maintain long-term alignment. [3]

While monofixation syndrome can be an acceptable outcome for patients with preexisting esotropia, many clinicians feel that it is an undesirable outcome for patients with intermittent exotropia. However, monofixation syndrome in postoperative patients with exotropia may have had underlying primary monofixation prior to surgery. [4]

Anisometropia

Blurred image on one macula due to uncorrected refractive error leads to unilateral suppression scotoma and mild-to-moderate amblyopia.

Almost 50% of such patients show no manifest deviation.

Adult patients who have no microtropia, uncorrected mild anisometropia (meridional or hyperopic anisometropia), subnormal stereovision, and a subtle difference in visual acuity likely have monofixation syndrome.

Primary monofixation syndrome

Most patients with primary monofixation syndrome are difficult to diagnose because of the lack of symptoms.

These patients have an inherent defect in central fusion, and most have mild amblyopia.

Prevalence of primary monofixation syndrome is 9% in families who have children with congenital esotropia. [5]

Monofixation syndrome may represent the forme fruste of congenital esotropia.

Macular lesion

Patients with organic macular lesions have a central scotoma.

They often maintain alignment because of peripheral fusion; they are the only group without a facultative scotoma.

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