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SECTION 8 – Pediatrics

Chapter 57 – Duane syndrome

Joseph L Demer

Clinical background

Duane retraction syndrome is a congenital cranial dysinnervation disorder characterized by uni- or bilateral abduction deficit, narrowing of the palpebral fissure on adduction, and globe retraction with occasional upshoot or downshoot in adduction (Box 57.1).[1] Unlike the large esotropia in abducens paralysis with which Duane syndrome shares the typical feature of abduction deficit, in Duane syndrome there is typically little to no esotropia in central gaze.

Box 57.1 

Clinical features of Duane retraction syndrome

      Limited ab- and/or adduction
      Globe retraction on adduction
      Palpebral fissure narrowing on adduction
      Up- and downshoots in adduction (common but not universal)

Huber's commonly used clinical classification of Duane syndrome consists of three groups: type 1, with limitation of abduction only; type 2, with limitation of adduction only; and type 3, with limitation of both ab- and adduction.[2,3] Type 1 Duane syndrome is most commonly encountered (Figure 57.1), followed by type 3. Type 2 Duane syndrome is rare, and often associated with exotropia. While there is wide agreement that unilateral Duane syndrome is more common …