Management of Duane retraction syndrome

Background: Duane retraction syndrome is the most common cause of congenital aberrant ocular innervation. We report referral practices, clinical characteristics and complications, prevalence of congenital and familial anomalies, and management outcomes from a clinic-based series. Methods: Retrospective clinic-based study of 65 patients with Duane retraction syndrome seen between January 1994 and March 2004. Results: The majority of patients were girls with type I Duane retraction syndrome associated with esotropia in primary gaze. Twenty percent of cases were complicated by absent binocular stereoacuity and 16.9% had amblyopia. Family history of any ocular disorder was reported in 38.5% of cases, while an associated congenital abnormality was found in 46% of patients. There was a significant delay between the age when ocular abnormalities were first noticed and the age when patients presented at our tertiary referral center (P <.001). Twenty-two percent of patients underwent surgical correction at a mean age of 6 years. Most procedures were unilateral or bilateral medial or lateral rectus recessions. Postoperative ocular alignment < 15 prism diopters (PD) was achieved in 86% of cases, with 50% of cases having < 5 PD. Conclusions: Amblyopia and absent binocular stereo vision affected one in five patients with Duane retraction syndrome. There was significant delay between identification of an abnormality and presentation at the eye clinic.