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

Choi, Hae Won; Park, Jung Min; Lee, Soo Jung

doi:2008.49.12.2021

J Korean Ophthalmol Soc > Volume 49(12); 2008 > Article

Case Report

Journal of the Korean Ophthalmological Society 2008;49(12):2021-2026.
DOI: https://doi.org/10.3341/jkos.2008.49.12.2021 Published online December 15, 2008.

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

Hae Won Choi, Jung Min Park, Soo Jung Lee

Department of ophthalmology, Maryknoll Hospital, Pusan, Korea. kris9352@hanmail.net

A형 사시를 동반한 전반적 선천섬유증증후군 1예

최해원ㆍ박정민ㆍ이수정

Department of ophthalmology, Maryknoll Hospital, Pusan, Korea

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.

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