Duane Retraction Syndrome
Tanya Glaser, MD
Laura B. Enyedi, MD
PREOPERATIVE CONSIDERATIONS
Duane retraction syndrome (DRS) is a spectrum of eye motility disorders caused by misinnervation of the lateral rectus muscle leading to co-contraction of the medial and lateral rectus muscles on attempted adduction with associated globe retraction. Imaging and histopathological studies have demonstrated an absent or hypoplastic 6th cranial nerve nucleus with aberrant innervation by the 3rd cranial nerve.1,2 DRS can occur in isolation but can also be associated with systemic conditions such as Goldenhar syndrome (oculo-auriculo-vertebral spectrum) or Wildervanck syndrome.
Recognizing the systemic associations is important for surgical planning purposes because they can complicate anesthesia. Specifically, the mandibular hypoplasia, cleft lip, and/or palate and craniovertebral anomalies seen in Goldenhar syndrome and the fused cervical vertebrae seen in Wildervanck syndrome can make airway management challenging.
DRS CLASSIFICATION
DRS Type 1:
Poor abduction.
Esotropia in primary position.
DRS Type 2:
Poor adduction.
Exotropia in primary position.
Least common form (7%).3
DRS Type 3:
Limited abduction and adduction.
Esodeviation, exodeviation, or no deviation in primary position.4
Uncommon (15%).3
DRS Type 4 (Synergistic Divergence):
Laterality:
Unilateral DRS more commonly affects the left eye.
DRS can occur bilaterally.
Different types can occur in each eye.
CLASSIC FINDINGS IN DRS
Limitation (complete or partial) of abduction of the affected eye.
Limitation of adduction (partial, rarely complete) of the affected eye.
Retraction and partial closure of the eyelids of the affected eye on adduction.
Oblique movements of the affected eye (up and in or down and in) on adduction, known as “upshoots” and “downshoots.”
Overshoots seen in DRS are thought to be due to a leash phenomenon caused by a tight lateral rectus muscle that slips up or down the globe when the eye adducts.
Deficiency of convergence in the affected eye.
Compensatory abnormal head posture is common:
Occurs when there is a deviation in primary gaze.
With an eso-deviation, the head turn will be toward the side of the affected eye.
With an exo-deviation, the head turn will be away from the affected eye.
Allows for binocular single vision:
Usually allows for binocularity and fusion.
Usually prevents strabismic amblyopia.
Strabismic, anisometropic, or mixed-type amblyopia occurs in about 14% of patients.6
Globe retraction and lid fissure narrowing on adduction.
Small or no eso-deviation in primary gaze despite significant limitation in abduction.
Abduction deficit is often less on elevation and depression.
Exo-deviation in extreme adduction because of the limitation in both adduction and abduction.
SURGICAL PLANNING IN DRS (FIG. 52.1)
Perform a complete eye examination:
Careful attention should be paid to the angle of deviation, anomalous head position, overshoots, and globe retraction as these findings will dictate the surgical plan.
Indications for surgical intervention:
Significant deviation in primary position.
Large face turn (>15 degrees).
Severe globe retraction.
Large overshoots.8
As with paralytic strabismus, it is unlikely that strabismus surgery can restore normal ocular motility. The goal instead is to bring the patient’s area of single binocular vision to primary gaze and eliminate or reduce anomalous head positions. Secondary goals include reducing the globe retraction and improving ductions if possible. Surgery on the unaffected eye is often needed for best results, and surgery may need to be staged.
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