Duane Retraction Syndrome

At a glance

Neurophthalmologic condition with an onset at birth and characterized by abnormal ocular movement such as eye movement limitations in the horizontal plane (adduction), outward toward the ear (abduction), or in both directions. It is usually not accompanied by other congenital anomaly; however, it has been also associated with other well-defined syndromes such as Okihiro’s, Wildervanck, Holt-Oram, Goldenhar and Moebius Syndromes (see below “Other conditions to be considered”). It is clinically characterized by a congenital strabismus that is accompanied with the inability of the eye to move outward.

Synonym

Stilling Turk Duane Syndrome; DR Syndrome; Duane Radial Ray Syndrome; Duane’s Retraction Syndrome; Eye Retraction Syndrome; Retraction Syndrome; Duane Syndrome.

History

This medical condition was originally described in 1887 by Jakob Stilling (1842-1915), a German ophthalmologist, and characterized by Siegmund Türk (1863-1931), a Swiss Ophthalmologist, in 1896. It was named after Alexander Duane (1858-1926), an American ophthalmologist, who was responsible to describe more specifically this disorder in 1905.

Classification

Duane Syndrome is clinically divided into three subtypes. They consist of:

• Duane Syndrome Type 1: Limited eye abduction with or without esotropia. It accounts for 85% of the affected individuals. It is characterized by the ability to adduct the affected eye normally (toward the nose) whereas the abduction is limited (toward the ear). During this movement, the pressure applied by the palpebral fissure onto the eye pushes it inside the socket.

• Duane Syndrome Type 2: Limited eye adduction with or without exotropia. It is the reverse of the description presented in Duane Type I.

• Duane Syndrome Type 3: Limitation of both abduction and adduction and any form of horizontal strabismus.

Incidence

Duane Retraction Syndrome (DRS) is an unusual form of congenital strabismus that accounts for 1 to 4% of children with strabismus. It is most often diagnosed by the age to 10 years. It is slightly more common in females than in males (about 60:40). It is often bilateral (20-57%), and affects more frequently the left eye (about 3-4:1). The prevalence has been estimated in the French population at 1.9% of the population affected with strabismus. In this study, female represented more than 53.5% of patients and the disorder was unilateral in 78% with the left eye affected more frequently than the right (71.9%). It appears that in approximately 20% of cases there is a familial trend; however, as a matter of interest, these are more likely to be bilateral than nonfamilial Duane Syndrome. Duane Syndrome has no particular race predilection.

Genetic inheritance

Approximately 56 to 90% are sporadic cases. In the residual cases, a positive family history with autosomal dominant transmission has been suggested, although some cases with most likely autosomal recessive inheritance have been reported. ...