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Duane Retraction Syndrome

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Practical Management of Pediatric Ocular Disorders and Strabismus

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Abstract

Duane retraction syndrome is a unilateral or bilateral condition characterized by abnormal innervation to the extraocular muscles that results in limitation of eye movement and co-contraction of muscles that are normally antagonists (Duane, Arch Ophthalmol 34:133–59, 1905). Depending on the pattern of dysinnervation, a variety of anomalous ocular motility patterns arise. Patients can have limited adduction, abduction, or both. Co-contraction of opposing muscles results in globe retraction. Patients may present with esotropia, exotropia, or orthotropia, and many have a compensatory head posture. There may also be associated upshoot or downshoot movements on attempted adduction or abduction. Classic type I Duane syndrome is often attributed to an absence of the abducens nucleus and nerve on the affected side with anomalous innervation of the lateral rectus muscle by a branch of the oculomotor nerve (Parsa et al., Am J Ophthalmol 125:399–401, 1998; Demer et al., Invest Ophthalmol Vis Sci 48:5505–11, 2007; Denis et al., J AAPOS 12:91–3, 2008; DeRespinis et al., Surv Ophthalmol 38:258–88, 1993). Most cases are sporadic, although familial cases have been reported, usually in an autosomal dominant pattern. An important consideration in the clinical management of patients with limited abduction is the differentiation of Duane syndrome from a cranial sixth nerve palsy, which would necessitate further neurologic evaluation. Surgical intervention is indicated in cases of large primary gaze deviation, significant torticollis, marked upshoots or downshoots, or significant globe retraction. Many patients are able to adapt to their limited motility and do not require surgery.

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Author information

Authors and Affiliations

  1. Department of Ophthalmology, University of Missouri—Kansas City School of Medicine, 2411 Holmes St, Kansas City, MO, 64108, USA

    Ashley Abraham MD

  2. Cole Eye Institute, Cleveland Clinic, i32, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Elias I. Traboulsi MD, MEd

  3. Department of Ophthalmology, Children’s Mercy Hospitals and Clinics, 3101 Broadway St, Kansas City, MO, 64111, USA

    Michelle M. Ariss MD

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  1. Ashley Abraham MD
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  2. Elias I. Traboulsi MD, MEd
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  3. Michelle M. Ariss MD
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Correspondence to Michelle M. Ariss MD .

Editor information

Editors and Affiliations

  1. Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio, USA

    Elias Traboulsi

  2. Abrahamson Pediatric Eye Institute, Cincinnati Children's Hospital, Cincinnati, Ohio, USA

    Virginia Utz

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Abraham, A., Traboulsi, E.I., Ariss, M.M. (2016). Duane Retraction Syndrome. In: Traboulsi, E., Utz, V. (eds) Practical Management of Pediatric Ocular Disorders and Strabismus. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2745-6_52

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  • DOI: https://doi.org/10.1007/978-1-4939-2745-6_52

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