A Case Report of the Congenital Fibrosis of Extraocular Muscles

ABSTRACT

PURPOSE: We report the clinical findings and surgical results of congenital fibrosis of extraocular muscles in a 5-year-old boy. METHODS: For a 5-year-old boy diagnosed with congenital fibrosis of extraocular muscles, we performed myectomy with disinsertion of both lateral rectus muscles and inferior rectus muscles and the conjunctival recession. We checked the position and motility of the eyeball before and after the operation, computerized tomography(CT) preoperatively, and the muscle biopsy postoperatively. RESULTS: In a 5-year-old boy complaining of blepharoptosis and ophthalmoplegia of both eyes from birth, and gaze limitation in all direction except abduction, atrophy of the extraocular muscles was found in CT. After myectomy with disinsertion of both lateral rectus muscles and inferior rectus muscles and conjunctival recession, the eyeball position improved but lower eyelid retraction occurred. The biopsy of the inferior rectus muscle confirmed the diagnosis of fibrosis of extraocular muscles. CONCLUSIONS: Congenital fibrosis of extraocular muscles shows ophthalmoplegia and blepharoptosis from birth. With clinical findings, extraocular muscle atrophy in CT is a useful finding in diagnosis. In treatment, extraocular muscle recession or disinsertion can be done but the treatment of postoperative lower lid retraction is required. Treatment of combined blepharoptosis can also be required.