Duane syndrome (retraction syndrome; stilling syndrome; turk-stilling syndrome)    388

General: Autosomal dominant; more frequent in females; manifestations in infancy; was thought to be secondary to fibrosis of the lateral rectus muscle or abnormal check ligaments; now established to be due to congenital aberrant innervation affecting third and seventh cranial nerves.

Ocular: Narrowing of palpebral fissure on adduction, widening on abduction; primary global retraction; deficiency of medial and lateral recti motility; limitation of abduction in affected eye usually is complete; retraction of the globe with attempted adduction varies from 1 to 10 mm; convergence insufficiency; heterochromia irides; left eye is more frequently involved.

Clinical: Associated Klippel-Feil syndrome; malformation of face, ears, and teeth.

Cross HE, Pfaffenbach DD. Duane's retraction syndrome and associated congenital malformations. Am J Ophthalmol 1972; 73:442.

Duane A. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contractions of the palpebral fissure and oblique movements of the eye. Arch Ophthalmol 1905; 34:133.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Holtz SJ. Congenital ocular anomalies associated with Duane's retraction syndrome, the nevus Ota, and axial anisometropia. Am J Ophthalmol 1974; 77:729.

Kim, JH, Hwang JM: Presence of the abducens nerve according to the type of Duane’s retraction syndrome. Ophthalmology 2005; 112: 109-l13.