Duane's syndrome

Duane's syndrome is an unusual congenital form of strabismus where there is paradoxical anomalous lateral rectus innervation of the affected eye due t o misdirection of axons destined for the medial rectus. Three types of Duan e's syndrome are recognized. Most cases of Duane's syndrome are sporadic bu t up to 10% are familial, usually with autosomal dominant inheritance. Seve ral autosomal dominant syndromes with dysmorphic features are associated wi th Duane's syndrome. Chromosomal loci for genes contributing to Duane's syn drome have been suggested at 4q, 8q and 22q. Duane's syndrome is heterogene ous at multiple levels with variations in its ocular manifestations, accomp anying systemic manifestations and in the chromosomal loci with which it ma y be associated. The definition clinically and genetically of the various s ubgroups of Duane's syndrome will provide a valuable insight into brainstem axonal guidance to the extraocular muscles during human development.