부산대학교 도서관

Purpose: Successful late treatment results of Congenital Fibrosis of Extraocular Muscles syndrome (CFEOM1). Methods: Our patient was 9 years old child with a variation of CFEOM1 syndrome, first presented with abnormal head position (AHP) of 30° chin up. Horizontal movements were normal, both eyes showed no elevation and limited depression, converging on attempt to elevate and diverging on attempt to depress, absent Bell's phenomenon. She was slightly hypermetropic with no amblyopia. Ptosis was significant on both eyes with RE levator muscle function of 6 mm and no levator function on LE. MRD 1 was 1/ 0 mm. She had compensatory frontalis function on both sides. Three surgeries were performed to alleviate AHP. During the first strabismus surgery thick fibrotic inferior recti on both eyes were recessed. On second strabismus surgery, since the passive motility test showed good elevation, anteposition of hypoplastic superior recti situated at 10 mm behind the limbus was performed. Asymmetric ptosis surgery - frontalis suspension was performed on both eyes. Results: After two strabismus surgeries AHP improved, but the patient still had slight chin up 15°. After ptosis surgery residual AHP was 5°. Conclusion: Although the best results in treating congenital fibrosis syndromes are achieved by early treatment (e.g.1-2 years old), later treatment can also give satisfying results. If no amblyopia present, the most important goal is to treat AHP successfully. Because of highly asymmetric and irregular action of muscles CFEOM syndromes, special care should be taken for dosage. [ABSTRACT FROM AUTHOR]