Duane’s syndrome
Duane's retraction syndrome is a congenital disorder in which the lateral rectus is innervated abnormally. The sixth nerve nucleus is hypoplastic and fails to supply the lateral rectus, and fibers from the third nerve innervate the muscle instead.1,2 Duane's syndrome belongs to the congenital cranial dysinnervation disorders, a group caused by developmental disturbance of the brainstem or cranial nerves.1
The medial and lateral rectus muscles of the affected eye contract together on attempted adduction. The globe retracts and the palpebral fissure narrows.1,2 Abduction is completely or partly restricted, and adduction is usually partly restricted.1 Most patients with type I are orthotropic in primary gaze despite a complete abduction deficit. Adults with an acquired sixth nerve palsy are characteristically esotropic, and this difference separates the two conditions.2 A face turn is common and gives binocular single vision with the face in the turned position.1
The Huber classification has three types. Type I, the most common, limits abduction with normal or mildly limited adduction, and the eyes are straight or slightly esotropic in primary position.1,2 Type II, the least common, limits adduction, and type III limits both adduction and abduction.1,2 The condition is often bilateral, and about 10% of cases are familial.1
More detail
Up to half of patients have systemic defects such as deafness, external ear abnormalities, speech disorder and skeletal abnormalities.1 An up-shoot or down-shoot in adduction may occur, sometimes produced by a tight lateral rectus slipping over or under the globe.1
Most patients need no surgery, because a compensatory head posture maintains binocular single vision. Surgery is considered for a head posture that is psychosocially unacceptable or causes neck discomfort, and for cosmetically unacceptable up-shoots, down-shoots or globe retraction.1 The lateral rectus of the involved side should not be resected, as resection increases retraction.1 Amblyopia, when present, usually results from anisometropia rather than strabismus.1
Sources (6)
- Kanski's Clinical Ophthalmology (2024), ch. 18
- Neuro-Ophthalmology: Diagnosis and Management, ch. 15
- Rutstein, R., Daum, K. (1997). Anomalies of Binocular Vision: Diagnosis & Management, Mosby.
- Appukuttan, A., Gillanders, J., Josephson, J. D., Helveston, E. M., Ellis, F., & Keltner, J. L. (1978). Duane's retraction syndrome. Journal of pediatric ophthalmology and strabismus, 15(4), 245-249.
- Parikh, R. S., Desai, A. D., Raj, V., Kaliki, S., Naik, M. N., & Murthy, R. (2012). Duane's retraction syndrome: series of 102 cases. Indian journal of ophthalmology, 60(1), 9-14.
- Hotchkiss, M. G., Miller, N. R., Clark, A. W., & Green, W. R. (1980). Bilateral Duane's retraction syndrome: a pathologic case study. Archives of Ophthalmology, 98(5), 870-874.
Reviewed October 2026