Diagnostic Considerations

Disorders similar in presentation to Duane syndrome (DS) can be acquired as a result of trauma or following localized infection of the orbit leading to inflammation and consequent mechanical restrictions of eye movement. In such cases, a thorough history usually will help in distinguishing between these conditions. In the clinical setting, the principal difficulties in differential diagnosis arise as a consequence of the very early age at which patients with this condition first present. The clinician must be persistent in examining abduction and adduction, as well as in looking for any associated palpebral fissure changes or head postures, when attempting to determine whether what often presents as a common childhood squint is, in fact, Duane syndrome. Fissure changes and the other associated characteristics of Duane syndrome, such as upshoots or downshoots and globe retraction, are vital when deciding whether any abduction limitation is the result of Duane syndrome and not a consequence of cranial nerve VI or abducens cranial nerve palsy.

Duane syndrome with radial-ray anomalies (see also Differential Diagnosis and other problems to be considered in the Physical section)

Moebius syndrome

Congenital or familiar esotropia

Congenital oculomotor apraxia

Epicanthal folds

Convergence or accommodative excess

Orbital pseudotumor

Grave disease

Ocular anomalies commonly associated with Duane syndrome

Dysplasia of the iris stroma

Papillary anomalies

Cataracts

Heterochromia

Coloboma

Crocodile tears

Microphthalmos

Systemic anomalies

Goldenhar syndrome

Klippel-Feil anomalad

Congenital labyrinthine deafness

Differential Diagnoses

Workup

Sinclair WW. Abnormal associated movements of the eyelids. Ophthalmol Rev. 1895. 14:307.
Bahr K. Vorstellung eines Falles von eigenartiger Muskelanomalie eines Auges Ber Dtsch. Ges Opthalmol. 1896. 25:334.
Stilling J. Bergmann JF, ed. Untersuchungen uber die Entstehung der Kurzsichtigkeit. Wiesbaden; 1887. 13.
Turk S. Bemerkungen zu einem Falle von Retraction des Auges. Cbl Pract Augenheilk. 1899. 23:14.
Wolff J. The occurrences of retraction movements of the eyeball together with congenital defects in the external ocular muscles. Arch Ophthalmol. 1900. 29:297.
Duane A. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. 1905. Arch Ophthalmol. 1996 Oct. 114(10):1255-6; discussion 1257. [QxMD MEDLINE Link].
Brown HW. Congenital structural anomalies of the muscles. Allen JH, ed. Strabismus Ophthalmic Symposium 11. St. Louis: CV Mosby Co; 1958. 391.
Lyle TK, Bridgeman GJO. Worth and Chavasse’s Squint. The Binocular Reflexes and the Treatment of Strabismus. 9th ed. London: Bailliere Tindall and Cox; 1959. 251-5.
Malbran J. Estrabismos y paralysis. Clinica y terapeutica, Buenos Aires. Editorial Oftalmologia. 1949. 627.
Huber A. Electrophysiology of the retraction syndromes. Br J Ophthalmol. 1974 Mar. 58(3):293-300. [QxMD MEDLINE Link].
Engle E. The genetics of strabismus: Duane, Moebius, and fibrosis syndromes. Traboulis E, ed. Genetic Diseases of the Eye: A Textbook and Atlas. New York: Oxford University Press; 1998. 477-512.
Kekunnaya R, Gupta A, Sachdeva V, Krishnaiah S, Rao BV, Vashist U, et al. Duane Retraction Syndrome: Series of 441 Cases. J Pediatr Ophthalmol Strabismus. 2011 Nov 8. 1-6. [QxMD MEDLINE Link].
Kirkham TH. Anisometropia and amblyopia in Duane's syndrome. Am J Ophthalmol. 1970 May. 69(5):774-7. [QxMD MEDLINE Link].
Maruo T, Kusota N, Arimoto H, Kikuchi R. Duane’s syndrome. Jap J Ophthalmol. 1979. 23:453.
Tredici TD, von Noorden GK. Are anisometropia and amblyopia common in Duane's syndrome?. J Pediatr Ophthalmol Strabismus. 1985 Jan-Feb. 22(1):23-5. [QxMD MEDLINE Link].
von Noorden GK. Recession of both horizontal recti muscles in Duane's retraction syndrome with elevation and depression of the adducted eye. Am J Ophthalmol. 1992 Sep 15. 114(3):311-3. [QxMD MEDLINE Link].
Duane TD, Schatz NJ, Caputo AR. Pseudo-Duane's retraction syndrome. Trans Am Ophthalmol Soc. 1977. 74:122-32. [QxMD MEDLINE Link].
Hoyt WF, Nachtigaller H. Anomalies of ocular motor nerves. Neuroanatomic correlates of paradoxical innervation in Duane's syndrome and related congenital ocular motor disorders. Am J Ophthalmol. 1965 Sep. 60(3):443-8. [QxMD MEDLINE Link].
Matteucci P. 1 dufetti congeniti di abduzione (congenital abduction deficiency) con particolare riguardo alla patogenesi. Rass Ital Ottallmol. 1946. 15:345.
Miller NR, Kiel SM, Green WR, Clark AW. Unilateral Duane's retraction syndrome (Type 1). Arch Ophthalmol. 1982 Sep. 100(9):1468-72. [QxMD MEDLINE Link].
Merino P, Merino M, Gómez De Liaño P, Blanco N. Horizontal rectus surgery in Duane syndrome. Eur J Ophthalmol. 2011 Jun 23. [QxMD MEDLINE Link].
Pineles SL, Rosenbaum AL, Kekunnaya R, Velez FG. Medial rectus recession after vertical rectus transposition in patients with esotropic Duane syndrome. Arch Ophthalmol. 2011 Sep. 129(9):1195-8. [QxMD MEDLINE Link].
Alexandrakis G, Saunders RA. Duane retraction syndrome. Ophthalmol Clin North Am. 2001 Sep. 14(3):407-17. [QxMD MEDLINE Link].
Appukuttan B, Gillanders E, Juo SH, Freas-Lutz D, Ott S, Sood R, et al. Localization of a gene for Duane retraction syndrome to chromosome 2q31. Am J Hum Genet. 1999 Dec. 65(6):1639-46. [QxMD MEDLINE Link].
Bagheri A, Repka MX. Association of Duane retraction syndrome and Brown syndrome. J Pediatr Ophthalmol Strabismus. 2005 Jul-Aug. 42(4):235-7. [QxMD MEDLINE Link].
Bremer JL. Recurrent branches of abducens nerve in human embryos. Am J Anat. 1921. 28:371.
Britt MT, Velez FG, Velez G, Rosenbaum AL. Vertical rectus muscle transposition for bilateral duane syndrome. J AAPOS. 2005 Oct. 9(5):416-21. [QxMD MEDLINE Link].
Campos E. Strabismus and ocular motility disorders. Proceedings of the Sixth Meeting of the International Strabismological Association. Surfer's Paradise, Australia: MacMillan Press; 1990. 221-235.
Carlson MR, Jampolsky A. An adjustable transposition procedure for abduction deficiencies. Am J Ophthalmol. 1979 Mar. 87(3):382-7. [QxMD MEDLINE Link].
Chew CK, Foster P, Hurst JA, Salmon JF. Duane's retraction syndrome associated with chromosome 4q27-31 segment deletion. Am J Ophthalmol. 1995 Jun. 119(6):807-9. [QxMD MEDLINE Link].
Chua B, Johnson K, Donaldson C, Martin F. Management of Duane retraction syndrome. J Pediatr Ophthalmol Strabismus. 2005 Jan-Feb. 42(1):13-7; quiz 45-6. [QxMD MEDLINE Link].
Cross HE, Pfaffenbach DD. Duane's retraction syndrome and associated congenital malformations. Am J Ophthalmol. 1972 Mar. 73(3):442-50. [QxMD MEDLINE Link].
Cullen P, Rodgers CS, Callen DF, Connolly VM, Eyre H, Fells P, et al. Association of familial Duane anomaly and urogenital abnormalities with a bisatellited marker derived from chromosome 22. Am J Med Genet. 1993 Nov 1. 47(6):925-30. [QxMD MEDLINE Link].
de Decker W. Kestenbaum transposition operation for treatment of the Duane I retraction syndrome. Trans Ophthalmol Soc U K. 1980. 100(4):479-82. [QxMD MEDLINE Link].
DeRespinis PA, Caputo AR, Wagner RS, Guo S. Duane's retraction syndrome. Surv Ophthalmol. 1993 Nov-Dec. 38(3):257-88. [QxMD MEDLINE Link].
Diamond G. Esotropia. Yanoff M, Duker JS. Ophthalmology. Philadelphia: Mosby; 1999. 6.6.1-6.6.8.
Ferrell RL, Jones B, Lucas RV Jr. Simultaneous occurrence of the Holt-Oram and the Duane syndromes. J Pediatr. 1966 Oct. 69(4):630-4. [QxMD MEDLINE Link].
Glaser JS, Bachynski B. Congenital motor and sensory anomalies. Glaser JS. Neuroophthalmology. 2nd ed. Philadelphia: JB Lippincott Co; 1990. 419-435.
Gobin MH. Surgical management of Duane's syndrome. Br J Ophthalmol. 1974 Mar. 58(3):301-6. [QxMD MEDLINE Link].
Goldstein JH, Sacks DB. Bilateral Duane's syndrome. J Pediatr Ophthalmol. 1977 Jan-Feb. 14(1):12-7. [QxMD MEDLINE Link].
Gourdeau A, Miller N, Zee D, Morris J. Central ocular motor abnormalities in Duane's retraction syndrome. Arch Ophthalmol. 1981 Oct. 99(10):1809-10. [QxMD MEDLINE Link].
Hotchkiss MG, Miller NR, Clark AW, Green WR. Bilateral Duane's retraction syndrome. A clinical-pathologic case report. Arch Ophthalmol. 1980 May. 98(5):870-4. [QxMD MEDLINE Link].
Isenberg S, Urist MJ. Clinical observations in 101 consecutive patients with Duane's retraction syndrome. Am J Ophthalmol. 1977 Sep. 84(3):419-25. [QxMD MEDLINE Link].
Jay WM, Hoyt CS. Abnormal brain stem auditory-evoked potentials in Stilling-Turk-Duane retraction syndrome. Am J Ophthalmol. 1980 Jun. 89(6):814-8. [QxMD MEDLINE Link].
Johnson LV. Adherence syndrome: Pseudoparalysis of the lateral or superior rectus muscles. Arch Ophthalmol. 1950. 44:870.
Kawano K, Fujita S. Duane's retraction syndrome associated with morning glory syndrome. J Pediatr Ophthalmol Strabismus. 1981 Jan-Feb. 18(1):51-4. [QxMD MEDLINE Link].
Kirkham TH. Duane's syndrome and familial perceptive deafness. Br J Ophthalmol. 1969 May. 53(5):335-9. [QxMD MEDLINE Link].
Kohlhase J, Chitayat D, Kotzot D, Ceylaner S, Froster UG, Fuchs , et al. SALL4 mutations in Okihiro syndrome (Duane-radial ray syndrome), acro-renal-ocular syndrome, and related disorders. Hum Mutat. 2005 Sep. 26(3):176-83. [QxMD MEDLINE Link].
Kowal VO, McKeown CA. Duane's syndrome. Int Ophthalmol Clin. 1992. 32(1):51-62. [QxMD MEDLINE Link].
Kraft SP. A surgical approach for Duane syndrome. J Pediatr Ophthalmol Strabismus. 1988 May-Jun. 25(3):119-30. [QxMD MEDLINE Link].
Kraft SP, O'Donoghue EP, Roarty JD. Improvement of compensatory head postures after strabismus surgery. Ophthalmology. 1992 Aug. 99(8):1301-8. [QxMD MEDLINE Link].
Kruger KE. [Contribution on the etiology of the Stilling-Turk-Duane syndrome]. Acta Ophthalmol (Copenh). 1969. 47(2):415-23. [QxMD MEDLINE Link].
Laughlin R. Hereditary paralysis of the abducens nerve. Am J Ophthalmol. 1937. 20:396.
MacDonald AL, Crawford JS, Smith DR. Duane's Retraction Syndrome: an evaluation of the sensory status. Can J Ophthalmol. 1974 Oct. 9(4):458-62. [QxMD MEDLINE Link].
Mehel E, Quere MA, Lavenant F, Pechereau A. [Epidemiological and clinical aspects of Stilling-Turk-Duane syndrome]. J Fr Ophtalmol. 1996. 19(8-9):533-42. [QxMD MEDLINE Link].
Mein J, Trimble R. Diagnosis and Management of Ocular Motility Disorders. 2nd ed. Blackwells; 1991.
Molarte AB, Rosenbaum AL. Vertical rectus muscle transposition surgery for Duane's syndrome. J Pediatr Ophthalmol Strabismus. 1990 Jul-Aug. 27(4):171-7. [QxMD MEDLINE Link].
Morad Y, Kowal L, Scott AB. Lateral rectus muscle disinsertion and reattachment to the lateral orbital wall. Br J Ophthalmol. 2005 Aug. 89(8):983-5. [QxMD MEDLINE Link].
Moster M. Paresis of isolated and multiple cranial nerves and painful ophthalmoplegia. Yanoff M, Duker JS. Ophthalmology. Philadelphia: Mosby; 1999. 11.16.1-11.16.12.
Nelson LB. Severe adduction deficiency following a large medial rectus recession in Duane's retraction syndrome. Arch Ophthalmol. 1986 Jun. 104(6):859-62. [QxMD MEDLINE Link].
Noel LP, Clarke WN. Adduction deficiency following medial recti recession in Duane's retraction syndrome. Case report. Arch Ophthalmol. 1987 Apr. 105(4):465. [QxMD MEDLINE Link].
O'Malley ER, Helveston EM, Ellis FD. Duane's retraction syndrome -- plus. J Pediatr Ophthalmol Strabismus. 1982 May-Jun. 19(3):161-5. [QxMD MEDLINE Link].
Okihiro MM, Tasaki T, Nakano KK, Bennett BK. Duane syndrome and congenital upper-limb anomalies. A familial occurrence. Arch Neurol. 1977 Mar. 34(3):174-9. [QxMD MEDLINE Link].
Otradovec J. [A bilateral Duane's syndrome in a brain stem tumor]. Klin Monatsbl Augenheilkd. 1968. 153(5):686-91. [QxMD MEDLINE Link].
Parsa CF, Grant E, Dillon WP Jr, du Lac S, Hoyt WF. Absence of the abducens nerve in Duane syndrome verified by magnetic resonance imaging. Am J Ophthalmol. 1998 Mar. 125(3):399-401. [QxMD MEDLINE Link].
Pfaffenbach DD, Cross HE, Kearns TP. Congenital anomalies in Duane's retraction syndrome. Arch Ophthalmol. 1972 Dec. 88(6):635-9. [QxMD MEDLINE Link].
Pieroni D. Goldenhar's syndrome associated with bilateral Duane's retraction syndrome. J Pediatr Ophthalmol. 1969. 6:16.
Pressman SH, Scott WE. Surgical treatment of Duane's syndrome. Ophthalmology. 1986 Jan. 93(1):29-38. [QxMD MEDLINE Link].
Raab EL. Clinical features of Duane's syndrome. J Pediatr Ophthalmol Strabismus. 1966. 23:64.
Ramsay J, Taylor D. Congenital crocodile tears: a key to the aetiology of Duane's syndrome. Br J Ophthalmol. 1980 Jul. 64(7):518-22. [QxMD MEDLINE Link].
Rhee DJ, Pyfer MF. Pediatrics: strabismus syndromes. 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.
Ro A, Chernoff G, MacRae D, Orton RB, Cadera W. Auditory function in Duane's retraction syndrome. Am J Ophthalmol. 1990 Jan 15. 109(1):75-8. [QxMD MEDLINE Link].
Rogers GL, Bremer DL. Surgical treatment of the upshoot and downshoot in Duanes' retraction syndrome. Ophthalmology. 1984 Nov. 91(11):1380-3. [QxMD MEDLINE Link].
Rosenbaum AL, Weiss SJ. Monozygotic twins discordant for Duane's retraction syndrome. J Pediatr Ophthalmol Strabismus. 1978 Nov-Dec. 15(6):359-61. [QxMD MEDLINE Link].
Sato S. Electromyographic studies on Duane’s retraction syndrome. Report 1. Qualitative changes of action currents of extraocular muscles in ocular movements. Acta Soc Ophthalmol Jap. 1959. 63:228.
Saunders RA, Phillips MS. Anterior segment ischemia after three rectus muscle surgery. Ophthalmology. 1988 Apr. 95(4):533-7. [QxMD MEDLINE Link].
Scott AB, Wong GY. Duane's syndrome. An electromyographic study. Arch Ophthalmol. 1972 Feb. 87(2):140-7. [QxMD MEDLINE Link].
Shainberg M. Duane syndrome. Am Orthopt J. 2000.
Sprunger DT. Recession of both horizontal rectus muscles in Duane syndrome with globe retraction in primary position. J AAPOS. 1997 Mar. 1(1):31-3. [QxMD MEDLINE Link].
Strachan IM, Brown BH. Electromyography of extraocular muscles in Duane's syndrome. Br J Ophthalmol. 1972 Aug. 56(8):594-9. [QxMD MEDLINE Link].
Vincent C, Kalatzis V, Compain S, Levilliers J, Slim R, Graia F, et al. A proposed new contiguous gene syndrome on 8q consists of Branchio-Oto-Renal (BOR) syndrome, Duane syndrome, a dominant form of hydrocephalus and trapeze aplasia; implications for the mapping of the BOR gene. Hum Mol Genet. 1994 Oct. 3(10):1859-66. [QxMD MEDLINE Link].
von Noorden GK, Murray E. Up- and downshoot in Duane's retraction syndrome. J Pediatr Ophthalmol Strabismus. 1986 Sep-Oct. 23(5):212-5. [QxMD MEDLINE Link].
Yuksel D, Optican LM, Lefevre P. Properties of saccades in duane retraction syndrome. Invest Ophthalmol Vis Sci. 2005 Sep. 46(9):3144-51. [QxMD MEDLINE Link].
Zhang F. Clinical features of 201 cases with Duane's retraction syndrome. Chin Med J (Engl). 1997 Oct. 110(10):789-91. [QxMD MEDLINE Link].
Gaur N, Sharma P. Management of Duane retraction syndrome: A simplified approach. Indian J Ophthalmol. 2019 Jan. 67 (1):16-22. [QxMD MEDLINE Link].

Media Gallery

Duane syndrome.
Duane syndrome.
An individual diagnosed with Duane syndrome in the left eye. In this image, the affected person is attempting to look far right. Notice the afflicted left eye faces straight and up (an "upshoot"), rather than following the right eye to the right.

of 3

Sections Duane Syndrome
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Media Gallery
Tables
References

Category	Description
Type
1 (70-80%)	Inability to abduct Normal or minimal defect in adduction Esotropia with head straight A or V pattern Usually updrift or a downdrift of affected eye on adduction or attempted abduction Globe retraction and palpebral-fissure narrowing on adduction Usual face turn to affected side Normal stereo possible
2 (about 7%)	Inability to adduct Normal or minimal defect in abduction Exotropia of the affected eye Marked upshoot Globe retraction and palpebral-fissure narrowing on adduction Stereo normal or suppressed Face turn to normal side
3 (about 15%)	Inability ability to abduct and adduct Globe retraction and palpebral-fissure narrowing on attempted adduction Possible upshoot and downshoot on adduction Straight or nearly straight head position Usually, normal stereo
4	Not usual Duane syndrome or Duane syndrome-type appearance Large-angle exotropia Face turn to uninvolved side Limited adduction Simultaneous abduction when looking toward uninvolved side Usually suppresses 1 eye
Subtype
A	Affected eye turned inward toward the nose (esotropia)
B	Affected eye turned outward toward the ear (exotropia)
C	Eyes in a straight primary position

Duane Syndrome Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

Tables

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