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