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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
- 1. Xia S, Li RL, Li YP, Qian XH, Chong V, Qi J. MRI findings in Duane's ocular retraction syndrome. Clinical radiology. 69 (5): e191-8. doi:10.1016/j.crad.2013.12.010 - Pubmed
- 2. Denis D, Dauletbekov D, Alessi G, Chapon F, Girard N. Duane retraction syndrome: MRI features in two cases. Journal of neuroradiology. Journal de neuroradiologie. 34 (2): 137-40. doi:10.1016/j.neurad.2007.01.002 - Pubmed
- 3. Xia S, Li RL, Li YP, Qian XH, Chong V, Qi J. MRI findings in Duane's ocular retraction syndrome. Clinical radiology. 69 (5): e191-8. doi:10.1016/j.crad.2013.12.010 - Pubmed