The etiology of Duane’s syndrome is a congenital agenesis of the sixth nerve nucleus with misdirection of part of the medial rectus nerve to aberrantly innervate the lateral rectus muscle [1]. So, the medial rectus nerve innervates both its agonist MR and the antagonist LR muscles. This pattern of innervation results in co-contraction of the medial and lateral recti on adduction causing globe retraction. Globe retraction produces lid fissure narrowing on adduction which is a classic sign of Duane’s syndrome. Another important sign is limited abduction. This is because of agenesis of the sixth nerve and the sixth nucleus. There are various subtypes of Duane’s syndrome based on the innervational pattern (see below). Duane’s syndrome is usually sporadic without systemic manifestations but some cases are familial or associated with systemic disease (Goldenhar syndrome, Klippel-Feil syndrome, and intrauterine thalidomide exposure).

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Strabismus Syndromes

  • Kenneth W. Wright,
  • Yi Ning J. Strube

摘要

The etiology of Duane’s syndrome is a congenital agenesis of the sixth nerve nucleus with misdirection of part of the medial rectus nerve to aberrantly innervate the lateral rectus muscle [1]. So, the medial rectus nerve innervates both its agonist MR and the antagonist LR muscles. This pattern of innervation results in co-contraction of the medial and lateral recti on adduction causing globe retraction. Globe retraction produces lid fissure narrowing on adduction which is a classic sign of Duane’s syndrome. Another important sign is limited abduction. This is because of agenesis of the sixth nerve and the sixth nucleus. There are various subtypes of Duane’s syndrome based on the innervational pattern (see below). Duane’s syndrome is usually sporadic without systemic manifestations but some cases are familial or associated with systemic disease (Goldenhar syndrome, Klippel-Feil syndrome, and intrauterine thalidomide exposure).