J Korean Ophthalmol Soc.  2008 Dec;49(12):2021-2026.

A Case of General Congenital Fibrosis Syndrome With A-pattern Strabismus

• Choi HW
• Park JM
• Lee SJ

Abstract

PURPOSE: To report a case of general congenital fibrosis syndrome with A-pattern strabismus, bilateral ptosis and hypotropia.
CASE SUMMARY
A six-year-old girl was presented with severe bilateral ptosis and esodeviation of the left eye since birth. No levator action was presented and her chin was elevated at an angle of 25 degrees. In both eyes, an abduction defect was observed, and no vertical movement was presented. There was a left esotropia of 20 prism diopters (PD) and bilateral hypotropia of 35PD. There was an A-pattern strabismus that presents convergence movements during attempts to look upward, and orthotropia during attempts to look downward. Diagnosed as general congenital fibrosis syndrome with A-pattern esotropia, bilateral ptosis and hypotropia, bilateral inferior rectus muscle recessions were performed. After the operation, chin elevation and hypotropia in both eyes were corrected and exodeviation increased. Thus, orthotropia was observed in the primary position, and an A-pattern strabismus occurred with an exotropia of 15PD on the downgaze.
CONCLUSIONS
A congenital fibrosis syndrome with an A-pattern strabismus was reported for the first time by the authors. On decisions concerningsurgical amounts of horizontal deviation, exodeviation should be considered to increase after bilateral inferior rectus muscle recessions.

Keyword

A-pattern strabismus; Congenital fibrosis syndrome; Inferior rectus muscle recession

MeSH Terms

Chin
Esotropia
Exotropia
Eye
Fibrosis
Muscles
Parturition
Strabismus

Figure

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  Figure 1. (A) Preoperative photograph shows bilateral ptosis and chin elevation. (B) Bell’s phenomenon was absent.

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  Figure 2. Preoperative photograph shows A-pattern esotropia that presents convergence movements on attemptsto look upward, a left esotropia of 20PD in the primary position, and orthotropia on attempts to look downward. Bilateral hypotropia of 35PD with no vertical movement, and abduction defect of both eyes were observed.

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  Figure 3. Light microscopic findingsof the inferior rectus muscle biopsy shows total displacement of muscle fibersby collagen fibers and fat (Masson-trichrome stain, ×200).

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  Figure 4. The postoperative 2-month photograph shows A-pattern strabismus that presents orthophoria in the primary position, an esotropia of 30PD in upgaze and an exotropia of 15PD in downgaze by increased postoperative exodeviation.

Reference

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