Review of the major findings about Duane retraction syndrome (DRS) leading to an updated form of classification

In view of all the reported evidence by electromyography in the 1970s, by histology in the 1980s, and by cerebral imagery since the 2000s, Duane retraction syndrome (DRS) has been described as the consequence of a congenital anomaly of the 6th cranial nerve nuclei with aberrant innervations by supply from the 3rd cranial nerve. Both genetic and environmental factors are likely to play a role when the cranial nerves and ocular muscles are developing between the 4th and the 8th week of gestation. New data from eye movement recordings contributed to better understanding the binocular control of saccades. Modeling of saccades in DRS seems promising for the quantification of the innervational deficit and the mechanical properties of the eye plant. The usual clinical classification of DRS needs to be updated in order to match more accurately the underlying dysinnervation of the extra ocular muscles and to illustrate the continuum that exists between the various forms. This review aims to summarize the major findings about DRS and to guide the clinician in the surgical management of this particular form of strabismus.

Research highlights► Congenital anomaly of the 6th cranial nerve nuclei. ► Aberrant innervations by supply from the 3rd cranial nerve. ► Various types of clinical patterns of Duane retraction syndrome (DRS). ► Study of saccades infers additional information about the innervation signals. ► All collected data lead to update the previous classification of DRS patients.