Duane's Retraction Syndrome

Duane's retraction syndrome.

Signs and symptoms: Duane's retraction syndrome (DRS) is a congenital disorder of ocular motility characterized by lim ited abduction, adduction or both. It is unilateral in 80% of cases.¹

The hall mark clinical signs are the classic retraction of the globe and narrowing of the palpebral fissure on eye movement. Studies reveal a preponderance of cases (66%) involving the left eye.^1-7 Other observations include exotropia in primary gaze (35%),³ crocodile-tearing phenomena (tearing while chewing, 12%),³ abduction deficit, globe retraction on attempted adduction, and compensatory face turn to maintain single simultaneous binocular vision. Undiagnosed or uncorrected DRS can lead to amblyopic vision loss.

DRS has three presentations. Type 1 occurs in 85% of cases and predominantly in females (60%).^2-4 It presents with an abduction palsy with globe retraction on attempted adduction. Type II DRS occurs in 14% of cases, ^2-4 and presents as an adduction palsy, globe retraction on attempted adduction and intact abduction. Type-III DRS comprises 1% of cases; both abduction and adduction limitations with any form of horizontal strabismus characterize this type. All cases may present with the "tether" phenomenon, with over-elevation or over-depression in adduction.

Thirty percent of cases have systemic associations, which include limb abnormalities, cardiac abnormalities, neurosensory deafness, Goldenhar's syndrome, Klippel-Feil syndrome, congenital gustolacrimal reflex and Wildervanck association.

Pathophysiology: Abnormal development of the cells in the abducens nucleolus characterizes DRS, resulting in restricted or absent abduction, and erroneous innervation of the lateral rectus by branches emanating from oculomotor nuclei.

Some neuropathologists have reported that DRS patients lack a cranial nerve VI nucleolus and nerve. Others maintain that lateral rectus dual innervation is present in at least the type I and II variants. While the exact pathophysiology remains controversial, mechanically DRS involves the development of an abnormal communication with the lateral rectus via the inferior division of cranial nerve III. This "miswiring" produces a dual electromyographic firing of the recti upon attempted adduction, resulting in globe retraction and palpebral fissure narrowing.

Management: Patients with DRS often develop peculiar but effective sensory adaptations. Surgery is only required when there is a risk of significant amblyopia.

The amount of esotropia or exotropia with DRS rarely exceeds 30.00D prism. Remedies include medial rectus recession, lateral rectus resection or both. Clinicians can improve the "tether" phenomenon by placing horizontal rectus positioning sutures or by splitting the lateral rectus into a "Y" and resuturing sections above and below the original axis. Full-correction spectacles, patch therapy or accommodative facility training can help avoid or mitigate amblyopia.

Clinical Pearls

• DRS exists in 0.1 % of the general population and accounts for 1-5 % of all strabismus cases. ^2-5
• Because systemic abnormalities exist in many cases, a complete health examination with blood work, auditory testing and EKG is essential.
• Differential diagnosis includes epicanthal folds, congenital esotropia, convergence excess, accommodative excess, excessive hyperopia with resultant esotropia, Brown's syndrome, double elevator palsy, Möbius syndrome, congenital fibrosis syndrome, CN VI palsy, Grave's disease and orbital pseudotumor.
• Children benefit most from surgical intervention. Typically, surgery is appropriate for adults only when the strabismus is cosmetically unacceptable. 

1. Moster M. Paresis of Isolated and Multiple Cranial Nerves and Painful Ophthalmoplegia. In : Yanoff M, Duker JS. Ophthalmology. Philadelphia: Mosby 1999:11.16.1 ­ 11.16.12.
2. Diamond G. Esotropia. In : Yanoff M, Duker JS. Ophthalmology. Philadelphia: Mosby 1999:6.6.1 ­ 6.6.8.
3. Zhang F. Clinical features of 201 cases with Duane's retraction syndrome. Chin Med J (Engl) 1997;110( 10):789 - 91.
4. Glaser JS, Bachynski B. Congenital Motor and Sensory Anomalies. In: Glaser JS. Neuroophthalmology 2nd ed. Philadelphia: J.B. Lippincott Co 1990 : 419 ­ 435.
5. Rhee DJ, Pyfer MF. Pediatrics; Strabismus Syndromes. In: Rhee DJ, Pyfer MF. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease, 3rd ed. Philadelphia: Lippincott Williams and Wilkins 1999:209-210.
6. Appukuttan B, Gillanders E, Juo SH, et al. Localization of a gene for Duane retraction syndrome to chromosome 2q31. Am J Hum Genet 1999;65(6):1639-1646.
7. Sprunger DT. Recession of both horizontal rectus muscles in Duane syndrome with globe retraction in primary position. J AAPOS 1997;1(1):31­3.