The surgical outcome of strabismus in patients with general fibrosis syndrome

Background: This study investigates the clinical features of strabismus in patients with general fibrosis syndrome (GFS) and the results of surgery performed on such patients. Methods: We conducted a retrospective review of patients with GFS who visited our clinic at Chang Gung Memorial Hospital between 1 August 1992 and 31 January 2002. After evaluating the family histories of 9 patients with GFS, patients were given a complete ophthalmic evaluation. Myectomies of the inferior rectus muscle were performed to correct hypotropia and recessions and/or resections of medial rectus and/or lateral rectus were performed to correct esotropia or exotropia, respectively. The post-operative conditions of the survey patients were reviewed for at least 6 months after the completion of the procedures. Results: Six patients (67%) were identified with inherited autosomal dominance. All patients displayed the characteristic 'chin-up' position, limited extraocular muscle movement and eye abnormalities. High astigmatism (>=-2.0 diopter) was noted in 9 eyes (50%) and amblyopia was noted in all cases. With regard to vertical eye deviation, 11 eyes (61%) were corrected through myectomy of the inferior rectus muscle to within 5° as measured using a Hirschberg test. Furthermore, three cases (33%) were complicated by lower scleral show. Conclusion: The presence of GFS complicates the surgical correction of strabismus making procedure results more difficult to predict. Strabismus surgery has been demonstrated to reduce eye deviation in the primary position, thus improving patients' head posture. The resulting improvements to cosmetic appearance and functionality lead us to recommend that strabismus surgery be performed in conjunction with ptosis surgery for GFS patients.