Godenhar증후군과 동반된 양안 안구후퇴 증후군 1예

초록

Purpose : Duane’s retraction syndrome is a congenital eye movement disorder caused by innervational disturbance. It is rare that Duane’s retraction syndrome is associated with Goldenhar’s syndrome which shows systemic congenital malformations. We report a case of bilateral Duane’s retraction syndrome associated with Goldenhar’s syndrome, which was treated with strabismus surgery and excision of epibulbar lipodermoid. Methods: A 14-month-old boy with exotropia showed severe limitation of adduction, mild limitation of abduction, globe retraction and palpabral fissure narrowing on attempted adduction in both eyes. He also had a diffuse small epibulabar mass under the lateral conjunctiva in the left eye. The angle of exodeviation was 45PD on alternate prism cover and uncover test. He had had cleft lip, multiple skin tags on the face and preauricular appendages since birth and undergone an operation for these abnormalities at age of 4 months. Bilateral 7.5mm recession of the lateral recti and excisional biopsy of epibulbar mass of the left eye were performed. Results: A case of Duane’s retraction syndrome associated with Goldenhar’s syndrome was found. The patient obtained orthophoria in his primary gaze after bilateral recession of lateral recti. The epibulbar mass was consistent with lipodermoid on pathologic examination.

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