Strabismus Surgery in Congenital Fibrosis of the Extraocular Muscles: A Paradigm

Background: Congenital fibrosis of extraocular muscles (CFEOM) is a rare group of disorders with variable phenotypes that result from aberrant innervation to the EOMs leading to synergistic vertical and/or horizontal deviations. We report our experience with the surgical management of patients with CFEOM. Materials and Methods: We reviewed the clinical findings, the surgical management, and outcomes of 52 consecutive CFEOM patients operated by one surgeon at a university hospital setting between 1993 and 2014. Patients were divided into CFEOM1, 2, or 3 based on clinical and/or molecular genetic findings. Results: Thirty-seven (71.2%) cases were bilateral and 15 (28.8%) were unilateral. Six of the bilateral cases had CFEOM2, and the rest of the patients had either CFEOM1 or CFEOM3. The median age at the first surgery was 10 (1-43) years. Twenty-five were females and 27 were males. Nineteen patients had previous strabismus and/or ptosis surgeries elsewhere. The mean number of operations at our center was 1.6 +/- 0.7 (1-4). A temporary stay suture was used in eight patients and permanently in seven. Of the 40 patients with abnormal head position, 18 achieved excellent, 15 good, and seven poor outcomes and ocular alignment in primary position following the latest surgery was excellent in 19, good in 18, and poor in 14 of the patients, as defined in the "Methods'' section of the paper. Conclusions: Although patients with CFEOM present significant strabismus surgical challenges because of EOM dysinnervation, fibrosis, and/or heterotopia, satisfactory alignment and improvement of the head posture can be attained in a significant proportion of patients using an individually tailored surgical approach.