Clinical Characteristics and Surgical Outcomes of Patients with Congenital Fibrosis of the Superior Rectus Muscle

INTRODUCTION: Congenital fibrosis of superior rectus muscle (CFSRM) is extremely rare and a difficult condition to manage surgically. The purpose of this report is to summarize the clinical characteristics of patients with CFSRM and analyze the effects of its surgical management. METHODS: Records of nine CFSRM patients were retrospectively reviewed and their clinical features including age, gender, visual acuity, refraction, ocular alignment, ocular motility, surgical method employed and final outcomes were analyzed. RESULTS: All cases were monocular. There were four cases (44.4%) coexistent with fibrosis of the levator palpebrae muscle and three (33.3%) with fibrosis of the superior oblique muscle. Eight cases (88.9%) presented with hypertropia, and one case showed mild hypotropia. Horizontal deviation was presented in six cases (66.7%). Surgical approaches included either superior rectus recession/tenotomy with or without traction suture; superior rectus recession and inferior rectus resection; or adhesion separation of superior rectus and levator palpebrae muscles. Excellent surgical outcomes were obtained in five cases (55.6%), good in two (22.2%), and poor outcomes were reported for two cases (22.2%). One patient (11.1%) underwent a second surgery. CONCLUSION: CFSRM was found to be mainly unilaterally infected, mostly manifesting as hypertropia, and was often associated with fibrosis of the levator palpebrae or superior oblique muscle. Weakening procedures of the superior rectus muscle were the preferred surgical technique. However, success rates were relatively low for CFSRM.