Surgical management of severe cocontraction, globe retraction, and pseudo-ptosis in Duane syndrome.

BACKGROUND: Correction of severe cocontraction and pseudo-ptosis present unique surgical challenges in patients with Duane syndrome.

METHODS: We report four Duane syndrome patients with esotropia in primary position, poor abduction, and severe cocontraction causing limitation to adduction, globe retraction, and pseudo-ptosis. All were treated with partial tendon transposition of the vertical rectus muscles augmented with Foster fixation sutures and surgical weakening of the ipsilateral lateral rectus muscle. One patient had a large recession of the lateral rectus muscle, and in three patients, the lateral rectus muscle was inactivated by removing from the globe and attaching its insertion to the lateral orbital wall.

RESULTS: Postoperatively, all patients were aligned within eight prisms diopters of orthotropia, had no face turn, and improved adduction and abduction. The two patients who had restriction to abduction on intraoperative forced ductions also had residual esotropia in primary position and underwent recession of the ipsilateral medial rectus muscle as a second procedure. Postoperative binocular single visual field was enlarged by 56 to 500% in the three patients who were tested preoperatively and postoperatively. Globe retraction and cocontraction were markedly relieved. Palpebral fissure widened 1.0 and 6.0 mm in two patients who had preoperative and postoperative measurements.

CONCLUSION: In Duane syndrome patients, severe cocontraction, globe retraction, and limitation to adduction may improve if the lateral rectus muscle is maximally recessed or its insertion is inactivated from the globe. Partial transposition of the vertical rectus muscles augmented with Foster sutures improved the angle of esotropia in primary position and abduction. Medial rectus muscle recession is indicated when the passive forced duction test reveals moderate-to-severe restriction to abduction.