Treatment of upshoot and downshoot in Duane syndrome by recession and y-splitting of the lateral rectus muscle

Abstract

Background Duane syndrome is characterized by abduction deficiency, narrowing of the palpebral fissure with retraction of globe on attempted adduction, and upshoot or downshoot, which can be the most prominent feature of the motility disorder. The upshoot and downshoot is believed to be caused by a tautness or leash effect from the lateral rectus muscle. The purpose of this study was to present the results of recession of the lateral rectus muscle with Y-splitting in the treatment of upshoot and downshoot in Duane syndrome. Methods Ten patients with Duane syndrome underwent surgery for severe upshoot or downshoot. Lateral rectus muscles recession from 5.0 to 9.0 mm and Y-splitting was accomplished in all patients. In 6 patients, the medial rectus muscles were recessed simultaneously from 5.0 to 6.0 mm to correct the associated marked globe retraction and ocular deviation. Results Mean age at the time of surgery was 9.9 ± 6.9 years (range, 3 to 20). Median follow up was 6 months (range, 3 weeks to 3 years). After surgery, all patients showed a marked decrease in upshoot or downshoot as well as improvement in globe retraction and face turn, where present. Conclusions Recession of lateral rectus muscle with Y-splitting is an extremely effective procedure in the treatment of significant upshoot and downshoot associated with globe retraction in Duane syndrome. When combined with simultaneous recession of the medial rectus muscle, it improves globe retraction and corrects ocular deviation.