eMedicine Specialties > Ophthalmology > Extraocular Muscles

Duane Syndrome: Follow-up

Author: Arun Verma, MD, Senior Consultant, Department of Ophthalmology, Dr Daljit Singh Eye Hospital, India
Contributor Information and Disclosures

Updated: Mar 20, 2008

Follow-up

Complications

  • Undercorrection of the primary position esotropia and face turn probably is the most common adverse outcome after surgical treatment of DS, especially when surgeons use amounts of recession typical of their experience in treating concomitant esotropia.
  • Undercorrection may be obvious in the immediate postoperative period, or the face turn may reappear years after what initially appeared to be a good result. The face turn is presumably due to contracture of the ipsilateral medial rectus muscle.
  • Undercorrection can be managed by means of vertical rectus transposition or by means of repeat recession of the medial rectus muscle, if the initial recession was relatively small (<8 mm). Repeat recession of the medial rectus is necessary if passive duction testing still indicates restriction.
  • With the large recessions necessary to treat DS, overcorrection occasionally occurs. Treatment of this secondary exotropia consists of advancement of the recessed medial rectus muscle or recession of the lateral rectus muscle, particularly if passive ductions indicate tightness of the lateral rectus muscles.
  • New vertical deviations may result from vertical rectus transposition. This condition is treated with (1) dissection (which involves considerable scar tissue) and recession of the appropriate transposed vertical rectus muscle in the ipsilateral eye, particularly if vertical passive ductions are positive, or (2) recession of the appropriate vertical rectus muscle in the contralateral eye.

Prognosis

  • The prognosis of surgery is not good; therefore, it is best to avoid surgery if patients have some form of binocular vision.

Patient Education

  • Patients must be taught to learn some form of head tilt and orthoptic training to have some binocular vision.

Miscellaneous

Medicolegal Pitfalls

  • Surgical results in DS are disappointing, and the outcomes are not predictable.
  • Patients must be well informed about the plan of treatment, about all possible surgical complications, and especially about the unexpected results.
  • Taking preoperative and postoperative pictures is wise.
  • The surgeon must spend a lot of time explaining the problems and consequences of the surgery to the patient because the surgery offers more cosmetic than functional benefits.
  • Obtaining well-informed written consent is mandatory before anything procedure is contemplated in patients with DS.
  • Showing patients photographs of failed procedures rather than images of successful procedures is a good way to help fully prepare patients.

Special Concerns

  • If the patient has amblyopia along with DS, improving the amblyopia with rigorous active vision therapy is of special concern.
  • The author has more than 15 years of experience in treating more than 5000 cases of various types of amblyopia, including the challenging ones associated with DS.  
    • The satisfaction obtained by improving the vision rather than any other result is perhaps the best reward in these cases.
    • If the patient can maintain some binocular vision, even with a head tilt, not disturbing the balance by performing unwanted surgery and jeopardizing the patient's situation is of special concern.
  • Clinical pearls
    • DRS exists in 0.1% of the general population and accounts for 1-5% of all strabismus cases.
    • Because systemic abnormalities exist in many cases, a complete physical examination, including blood work, auditory testing, and electrocardiogram (ECG), is essential.
    • Differential diagnosis includes epicanthal folds, congenital esotropia, convergence excess, accommodative excess, excessive hyperopia with resultant esotropia, Brown syndrome, double elevator palsy, Möbius syndrome, congenital fibrosis syndrome, sixth cranial nerve palsy, Grave disease, and orbital pseudotumor.
    • Children benefit most from surgical intervention. Typically, surgery is appropriate for adults only when the strabismus is cosmetically unacceptable.
 


More on Duane Syndrome

Overview: Duane Syndrome
Differential Diagnoses & Workup: Duane Syndrome
Treatment & Medication: Duane Syndrome
Follow-up: Duane Syndrome
Multimedia: Duane Syndrome
References
[ CLOSE WINDOW ]

References

  1. Sinclair WW. Abnormal associated movements of the eyelids. Ophthalmol Rev. 1895;14:307.

  2. Bahr K. Vorstellung eines Falles von eigenartiger Muskelanomalie eines Auges Ber Dtsch. Ges Opthalmol. 1896;25:334.

  3. Stilling J. Bergmann JF, ed. Untersuchungen uber die Entstehung der Kurzsichtigkeit. Wiesbaden; 1887:13.

  4. Turk S. Bemerkungen zu einem Falle von Retraction des Auges. Cbl Pract Augenheilk. 1899;23:14.

  5. Wolff J. The occurrences of retraction movements of the eyeball together with congenital defects in the external ocular muscles. Arch Ophthalmol. 1900;29:297.

  6. 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. Oct 1996;114(10):1255-6; discussion 1257. [Medline].

  7. Brown HW. Congenital structural anomalies of the muscles. In: Allen JH, ed. Strabismus Ophthalmic Symposium 11. St. Louis: CV Mosby Co; 1958:391.

  8. 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.

  9. Malbran J. Estrabismos y paralysis. Clinica y terapeutica, Buenos Aires. Editorial Oftalmologia. 1949;627.

  10. Huber A. Electrophysiology of the retraction syndromes. Br J Ophthalmol. Mar 1974;58(3):293-300. [Medline].

  11. Engle E. The genetics of strabismus: Duane, Moebius, and fibrosis syndromes. In: Traboulis E, ed. Genetic Diseases of the Eye: A Textbook and Atlas. New York: Oxford University Press; 1998:477-512.

  12. Kirkham TH. Anisometropia and amblyopia in Duane's syndrome. Am J Ophthalmol. May 1970;69(5):774-7. [Medline].

  13. Maruo T, Kusota N, Arimoto H, Kikuchi R. Duane's syndrome. Jap J Ophthalmol. 1979;23:453.

  14. Tredici TD, von Noorden GK. Are anisometropia and amblyopia common in Duane's syndrome?. J Pediatr Ophthalmol Strabismus. Jan-Feb 1985;22(1):23-5. [Medline].

  15. 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. Sep 15 1992;114(3):311-3. [Medline].

  16. Duane TD, Schatz NJ, Caputo AR. Pseudo-Duane's retraction syndrome. Trans Am Ophthalmol Soc. 1977;74:122-32. [Medline].

  17. 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. Sep 1965;60(3):443-8. [Medline].

  18. Matteucci P. 1 dufetti congeniti di abduzione (congenital abduction deficiency) con particolare riguardo alla patogenesi. Rass Ital Ottallmol. 1946;15:345.

  19. Miller NR, Kiel SM, Green WR, Clark AW. Unilateral Duane's retraction syndrome (Type 1). Arch Ophthalmol. Sep 1982;100(9):1468-72. [Medline].

  20. Alexandrakis G, Saunders RA. Duane retraction syndrome. Ophthalmol Clin North Am. Sep 2001;14(3):407-17. [Medline].

  21. 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. Dec 1999;65(6):1639-46. [Medline].

  22. Bagheri A, Repka MX. Association of Duane retraction syndrome and Brown syndrome. J Pediatr Ophthalmol Strabismus. Jul-Aug 2005;42(4):235-7. [Medline].

  23. Bremer JL. Recurrent branches of abducens nerve in human embryos. Am J Anat. 1921;28:371.

  24. Britt MT, Velez FG, Velez G, Rosenbaum AL. Vertical rectus muscle transposition for bilateral duane syndrome. J AAPOS. Oct 2005;9(5):416-21. [Medline].

  25. Campos E. Strabismus and ocular motility disorders. In: Proceedings of the Sixth Meeting of the International Strabismological Association. Surfer's Paradise, Australia: MacMillan Press; 1990:221-235.

  26. Carlson MR, Jampolsky A. An adjustable transposition procedure for abduction deficiencies. Am J Ophthalmol. Mar 1979;87(3):382-7. [Medline].

  27. Chew CK, Foster P, Hurst JA, Salmon JF. Duane's retraction syndrome associated with chromosome 4q27-31 segment deletion. Am J Ophthalmol. Jun 1995;119(6):807-9. [Medline].

  28. Chua B, Johnson K, Donaldson C, Martin F. Management of Duane retraction syndrome. J Pediatr Ophthalmol Strabismus. Jan-Feb 2005;42(1):13-7; quiz 45-6. [Medline].

  29. Cross HE, Pfaffenbach DD. Duane's retraction syndrome and associated congenital malformations. Am J Ophthalmol. Mar 1972;73(3):442-50. [Medline].

  30. 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. Nov 1 1993;47(6):925-30. [Medline].

  31. de Decker W. Kestenbaum transposition operation for treatment of the Duane I retraction syndrome. Trans Ophthalmol Soc U K. 1980;100(4):479-82. [Medline].

  32. DeRespinis PA, Caputo AR, Wagner RS, Guo S. Duane's retraction syndrome. Surv Ophthalmol. Nov-Dec 1993;38(3):257-88. [Medline].

  33. Diamond G. Esotropia. In: Yanoff M, Duker JS. Ophthalmology. Philadelphia: Mosby; 1999:6.6.1-6.6.8.

  34. Ferrell RL, Jones B, Lucas RV Jr. Simultaneous occurrence of the Holt-Oram and the Duane syndromes. J Pediatr. Oct 1966;69(4):630-4. [Medline].

  35. Glaser JS, Bachynski B. Congenital motor and sensory anomalies. In: Glaser JS. Neuroophthalmology. 2nd ed. Philadelphia: JB Lippincott Co; 1990:419-435.

  36. Gobin MH. Surgical management of Duane's syndrome. Br J Ophthalmol. Mar 1974;58(3):301-6. [Medline].

  37. Goldstein JH, Sacks DB. Bilateral Duane's syndrome. J Pediatr Ophthalmol. Jan-Feb 1977;14(1):12-7. [Medline].

  38. Gourdeau A, Miller N, Zee D, Morris J. Central ocular motor abnormalities in Duane's retraction syndrome. Arch Ophthalmol. Oct 1981;99(10):1809-10. [Medline].

  39. Hotchkiss MG, Miller NR, Clark AW, Green WR. Bilateral Duane's retraction syndrome. A clinical-pathologic case report. Arch Ophthalmol. May 1980;98(5):870-4. [Medline].

  40. Isenberg S, Urist MJ. Clinical observations in 101 consecutive patients with Duane's retraction syndrome. Am J Ophthalmol. Sep 1977;84(3):419-25. [Medline].

  41. Jay WM, Hoyt CS. Abnormal brain stem auditory-evoked potentials in Stilling-Turk-Duane retraction syndrome. Am J Ophthalmol. Jun 1980;89(6):814-8. [Medline].

  42. Johnson LV. Adherence syndrome: Pseudoparalysis of the lateral or superior rectus muscles. Arch Ophthalmol. 1950;44:870.

  43. Kawano K, Fujita S. Duane's retraction syndrome associated with morning glory syndrome. J Pediatr Ophthalmol Strabismus. Jan-Feb 1981;18(1):51-4. [Medline].

  44. Kirkham TH. Duane's syndrome and familial perceptive deafness. Br J Ophthalmol. May 1969;53(5):335-9. [Medline].

  45. 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. Sep 2005;26(3):176-83. [Medline].

  46. Kowal VO, McKeown CA. Duane's syndrome. Int Ophthalmol Clin. 1992;32(1):51-62. [Medline].

  47. Kraft SP. A surgical approach for Duane syndrome. J Pediatr Ophthalmol Strabismus. May-Jun 1988;25(3):119-30. [Medline].

  48. Kraft SP, O'Donoghue EP, Roarty JD. Improvement of compensatory head postures after strabismus surgery. Ophthalmology. Aug 1992;99(8):1301-8. [Medline].

  49. Kruger KE. [Contribution on the etiology of the Stilling-Turk-Duane syndrome]. Acta Ophthalmol (Copenh). 1969;47(2):415-23. [Medline].

  50. Laughlin R. Hereditary paralysis of the abducens nerve. Am J Ophthalmol. 1937;20:396.

  51. MacDonald AL, Crawford JS, Smith DR. Duane's Retraction Syndrome: an evaluation of the sensory status. Can J Ophthalmol. Oct 1974;9(4):458-62. [Medline].

  52. 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. [Medline].

  53. Mein J, Trimble R. Diagnosis and Management of Ocular Motility Disorders. 2nd ed. Blackwells; 1991.

  54. Molarte AB, Rosenbaum AL. Vertical rectus muscle transposition surgery for Duane's syndrome. J Pediatr Ophthalmol Strabismus. Jul-Aug 1990;27(4):171-7. [Medline].

  55. Morad Y, Kowal L, Scott AB. Lateral rectus muscle disinsertion and reattachment to the lateral orbital wall. Br J Ophthalmol. Aug 2005;89(8):983-5. [Medline].

  56. 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.

  57. Nelson LB. Severe adduction deficiency following a large medial rectus recession in Duane's retraction syndrome. Arch Ophthalmol. Jun 1986;104(6):859-62. [Medline].

  58. Noel LP, Clarke WN. Adduction deficiency following medial recti recession in Duane's retraction syndrome. Case report. Arch Ophthalmol. Apr 1987;105(4):465. [Medline].

  59. O'Malley ER, Helveston EM, Ellis FD. Duane's retraction syndrome -- plus. J Pediatr Ophthalmol Strabismus. May-Jun 1982;19(3):161-5. [Medline].

  60. Okihiro MM, Tasaki T, Nakano KK, Bennett BK. Duane syndrome and congenital upper-limb anomalies. A familial occurrence. Arch Neurol. Mar 1977;34(3):174-9. [Medline].

  61. Otradovec J. [A bilateral Duane's syndrome in a brain stem tumor]. Klin Monatsbl Augenheilkd. 1968;153(5):686-91. [Medline].

  62. 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. Mar 1998;125(3):399-401. [Medline].

  63. Pfaffenbach DD, Cross HE, Kearns TP. Congenital anomalies in Duane's retraction syndrome. Arch Ophthalmol. Dec 1972;88(6):635-9. [Medline].

  64. Pieroni D. Goldenhar's syndrome associated with bilateral Duane's retraction syndrome. J Pediatr Ophthalmol. 1969;6:16.

  65. Pressman SH, Scott WE. Surgical treatment of Duane's syndrome. Ophthalmology. Jan 1986;93(1):29-38. [Medline].

  66. Raab EL. Clinical features of Duane's syndrome. J Pediatr Ophthalmol Strabismus. 1966;23:64.

  67. Ramsay J, Taylor D. Congenital crocodile tears: a key to the aetiology of Duane's syndrome. Br J Ophthalmol. Jul 1980;64(7):518-22. [Medline].

  68. 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.

  69. Ro A, Chernoff G, MacRae D, Orton RB, Cadera W. Auditory function in Duane's retraction syndrome. Am J Ophthalmol. Jan 15 1990;109(1):75-8. [Medline].

  70. Rogers GL, Bremer DL. Surgical treatment of the upshoot and downshoot in Duanes' retraction syndrome. Ophthalmology. Nov 1984;91(11):1380-3. [Medline].

  71. Rosenbaum AL, Weiss SJ. Monozygotic twins discordant for Duane's retraction syndrome. J Pediatr Ophthalmol Strabismus. Nov-Dec 1978;15(6):359-61. [Medline].

  72. 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.

  73. Saunders RA, Phillips MS. Anterior segment ischemia after three rectus muscle surgery. Ophthalmology. Apr 1988;95(4):533-7. [Medline].

  74. Scott AB, Wong GY. Duane's syndrome. An electromyographic study. Arch Ophthalmol. Feb 1972;87(2):140-7. [Medline].

  75. Shainberg M. Duane syndrome. Am Orthopt J. 2000.

  76. Sprunger DT. Recession of both horizontal rectus muscles in Duane syndrome with globe retraction in primary position. J AAPOS. Mar 1997;1(1):31-3. [Medline].

  77. Strachan IM, Brown BH. Electromyography of extraocular muscles in Duane's syndrome. Br J Ophthalmol. Aug 1972;56(8):594-9. [Medline].

  78. 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. Oct 1994;3(10):1859-66. [Medline].

  79. von Noorden GK, Murray E. Up- and downshoot in Duane's retraction syndrome. J Pediatr Ophthalmol Strabismus. Sep-Oct 1986;23(5):212-5. [Medline].

  80. Yuksel D, Optican LM, Lefevre P. Properties of saccades in duane retraction syndrome. Invest Ophthalmol Vis Sci. Sep 2005;46(9):3144-51. [Medline].

  81. Zhang F. Clinical features of 201 cases with Duane's retraction syndrome. Chin Med J (Engl). Oct 1997;110(10):789-91. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

DS, Duane's syndrome, Duane retraction syndrome, DRS, congenital retraction syndrome, DRS, Stilling-Turk-Duane retraction syndrome, Duane radial dysplasia syndrome, Duane-Stilling-Turk syndrome, Stilling's syndrome, Stilling syndrome, Turk's syndrome, Turk syndrome, Turk-Stilling syndrome, Duane 1, Duane 2, Duane 3

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Arun Verma, MD, Senior Consultant, Department of Ophthalmology, Dr Daljit Singh Eye Hospital, India
Disclosure: Nothing to disclose.

Medical Editor

Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

J James Rowsey, MD, Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida
J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

RELATED EMEDICINE ARTICLES
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.