Duane's retraction syndrome (DRS)

• Duane's retraction syndrome (DRS) (also known as Stilling-Turk-Duane syndrome)
  • prevalence of about 0.1% of the general population
  • accounts for 1% to 5% of all strabismus cases
  • DRS is a congenital oculomotor anomaly
    • characterized by the pathognomonic ocular motility pattern of globe retraction with simultaneous narrowing of the palpebral fissure on attempted adduction
    • most cases of DRS occur sporadically
      • incidence of genetic inheritance has been approximated to fall within a range between 5% and 23 %
    • syndrome includes variants permitting an abduction deficiency, adduction deficiency, or a combination of the two
    • often there is an associated 'tether' phenomenon consisting of over elevation, over depression, or both during adduction
    • globe retraction on adduction occurs secondary to aberrant innervation
      • results in cocontraction of the medial and lateral recti in the affected eye

Reference:

• (1) DeRespinis PA et al. Duane's retraction syndrome. Surv Ophthalmol 1993;38 (3):257-28.