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Review
. 2017 Oct 30:11:1917-1930.
doi: 10.2147/OPTH.S127481. eCollection 2017.

Duane retraction syndrome: causes, effects and management strategies

Ramesh Kekunnaya  1 , Mithila Negalur  1
Affiliations
Review

Duane retraction syndrome: causes, effects and management strategies

Ramesh Kekunnaya et al. Clin Ophthalmol. .

Abstract

Duane retraction syndrome (DRS) is a congenital eye movement anomaly characterized by variable horizontal duction deficits, with narrowing of the palpebral fissure and globe retraction on attempted adduction, occasionally accompanied by upshoot or down-shoot. The etiopathogenesis of this condition can be explained by a spectrum of mechanical, innervational, neurologic and genetic abnormalities occurring independently or which influence each other giving rise to patterns of clinical presentations along with a complex set of ocular and systemic anomalies. Huber type I DRS is the most common form of DRS with an earlier presentation, while Huber type II is the least common presentation. Usually, patients with unilateral type I Duane syndrome have esotropia more frequently than exotropia, those with type II have exotropia and those with type III have esotropia and exotropia occurring equally common. Cases of bilateral DRS may have variable presentation depending upon the type of presentation in each eye. As regards its management, DRS classification based on primary position deviation as esotropic, exotropic or orthotropic is more relevant than Huber's classification before planning surgery. Surgical approach to these patients is challenging and must be individualized based on the amount of ocular deviation, abnormal head position, associated globe retraction and overshoots.

Keywords: Duane syndrome; esotropia; overshoots; strabismus surgery.

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Conflict of interest statement

Disclosure The authors report no conflicts of interest in this work.

Figures

Figure 1
Figure 1
Demonstrating an upshoot (A) and downshoot (B) on attempted adduction.
Figure 2
Figure 2
Clinical grading system for globe retraction and overshoots. Notes: (A) Grade 1 retraction and upshoot; (B) grade 2 retraction; (C) grade 3 retraction and upshoot; (D) grade 4 retraction and upshoot (pumpkin seed sign).
Figure 3
Figure 3
Outcome of medial rectus recession with superior rectus transposition in type I esotropic DRS. Note: (A) Preoperative and (B) postoperative nine-gaze photograph following right medial rectus recession combined with superior rectus transposition. Abbreviation: DRS, Duane retraction syndrome.
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