Duane syndrome

Last revised by Assoc Prof Craig Hacking on 12 Feb 2019

Duane syndrome, also known as Duane retraction syndrome, is a rare congenital disease characterized by non-progressive strabismus. It is caused by a variable degree of abnormal development of one or both 6th cranial nerves (CN VI).

It presents during childhood and it accounts for less than 5% of cases of strabismus 1. Three subtypes of Duane syndrome are described:

  • DRS type I: most common
  • DRS type II
  • DRS type III 

The most common clinical presentation is strabismus because of hypoplasia/atrophy of the lateral rectus muscle. Other possible clinical presentation include amblyopia and blepharostenosis.

The majority of cases are unilateral, but it can be bilateral in up to 20% of patients.

MRI findings consist of the following 2,3:

  • hypoplasia/atrophy of the lateral rectus muscle
    • the degree of atrophy is variable as the lateral rectus muscle may be reinnervated by aberrant branches from the 3rd cranial nerve (CN III)
  • hypoplasia or absence of CN VI

Hypoplasia of other extra-ocular muscles have been described, depending on the subtype of the disease, especially the superior oblique muscle (DRS type I and II) 3.


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