Duane syndrome: Clinical features and surgical management

To further define the clinical features of Duane syndrome and analyze the surgical outcomes in a subset of these patients.

Retrospective chart review.

A retrospective chart review of 75 patients with Duane syndrome was performed.

Complete ophthalmologic and orthoptic data were extracted from the patients' charts, and patients were classified into group I, II, or III based on forced primary position (PP) deviation. Eighteen patients had strabismus surgery to correct the ocular misalignment with its related abnormal head posture.

Fifty-six percent of the cases were in group I, 16% in group II, and 28% in group III. Fifty-seven percent were female, 91% were unilateral, and 66% had left eye involvement. Ninety-six percent had fissure-narrowing/globe retraction on adduction; 67% had an upshoot, downshoot, or both; and 63% had an A, V, or X pattern. The A patterns correlated with downshoots in 100% of cases, V patterns with upshoots in 88%, and X patterns with upshoots/downshoots in 100%. In the 18 surgical candidates, there were 14 patients in group I, 4 in group II, and 0 in group III. An excellent surgical outcome occurred in 78% of patients and a fair-to-poor outcome in 22%. Upshoots/downshoots and fissure-narrowing/globe retraction patients showed modest improvement after surgery.

There was a consistent relationship among the type of strabismus, head position, and motility defect. The type of upshoot/downshoot correlated strongly with the A, V, or X pattern. Postoperative improvements of abnormal head posture and PP alignment were achieved in the surgical subset of patients.