DUANE’S SYNDROME: SURGICAL OUTCOME AND NON OPHTHALMOLOGIC ASSOCIATIONS

Sarah Sarfraz, Saemah Nuzhat Zafar, Ayesha Khan

Abstract


Background: Duane retraction syndrome (DRS) is the most common of the ocular congenital cranial dysinnervation disorders .This study evaluates the types of Duane syndrome and its management in patients presenting to the paediatric and strabismus unit of a tertiary care eye hospital. Methods: This case series study involved 41 patients diagnosed with Duane syndrome between January 2007 and December 2009. History of presenting complaints, past treatment and family history were recorded. Ocular examination and orthoptic assessment was carried out Results: Forty one patients were included in this case series study. It involved 10 right eyes, 27 left eyes and both eyes of 4 patients. There were 26 females and 15 males. Type-1 Duane syndrome was present in 28 (68.3%), type 2 in 8 (19.5%), Type-3 in 4 (9.8%) and type-4 with synergistic divergence was present in 1 (2.4%) patient. Comorbidity was present in 6(14.6%) patients. Surgery was carried out in 26 (63.4%) patients either for abnormal head posturing or significant upshoots or down shoots. Upshoots noted in 21 eyes, were completely or partially resolved in 15 cases. Among 4 patients with down shoots on adduction, complete resolution was seen in 1. The pre and post-operative measurements of horizontal deviation showed statistically significant difference in Duane type-1 and 2, where as in Duane type-3 it was not significant. One patient with type-4 Duane did not undergo surgery. Conclusions: Recession of the horizontal recti is more effective in treating the upshoot or down shoot associated with DRS as compared to recession and y-split of the horizontal muscle.

Keywords: Duane retraction syndrome, upshoots and downshoots, congenital cranial dysinnervation disorder

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References


Gutowski NJ, Bosley TM, Engle EC. 110th ENMC International Workshop: the congenital cranial dysinnervation disorders (CCDDs). Naarden, The Netherlands, 25-27 October, 2002. Neuromuscul Disord. 2003;13:573–8

Demer JL, Clark RA, Lim KH, Engle EC. Magnetic resonance imaging evidence for widespread orbital dysinnervation in dominant Duane's retraction syndrome linked to the DURS2 locus. Invest Ophthalmol Vis Sci. 2007;48:194–202

Hertle RW. National Eye Institute Sponsored Classification of Eye Movement Abnormalities and Strabismus Working Group. A next step in naming and classification of eye movement disorders and strabismus. J AAPOS 2002;6:201–2

Mohan K, Sharma A, Pandav SS. Differences in epidemiological and clinical characteristics between various types of Duane retraction syndrome in 331 patients. J AAPOS 2008;12:576–80

DeRespinis PA, Caputo AR, Wagner RS, Guo S. Duane’s retraction syndrome. Surv Ophthalmol 1993;38:257–88.

Kubota N, Takahashi T, Hayashi T, Sakaue T, Maruo T. Outcome of surgery in 124 cases of Duane's Retraction Syndrome (DRS) treated by intraoperatively graduated recession of the medial rectus for esotropic DRS, and of the lateral rectus for exotropic DRS. Binocul Vis Strabismus Q 2001;16:15–22.

Merino P, Merino M, Gomez De Liano P, Blanco N. Horizontal rectus surgery in Duane syndrome. Eur J Ophthalmol 2012;22:125–30.

Andrews CV, Hunter DG, Engle EC. Duane Syndrome. In: Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, editors. GeneReviewsTM [Internet]. Seattle(WA): University of Washington, Seattle; 1993-2007 May 25 [updated 2010 Feb 18]. Available at: http://www.ncbi.nlm.nih.gov/books/NBK1190

Oystreck DT, Engle EC, Bosley TM. Recent progress in understanding congenital cranial dysinnervation disorders. J Neuroophthalmol. 2011;31:69–77.

Hughes PJ, Davies PT, Roche SW, Matthews TD, Lane RJ. Wildervanck or cervico-oculo-acoustic syndrome and MRI findings. J Neurol Neurosurg Psychiatry 1991;54:503–4

Chen X, Zhao KX, Song KY, Guo X, Ma HZ, Du CQ, Zhu LN. A clinical analysis of sixty-seven cases with Duane's retraction syndrome. Zhonghua Yan Ke Za Zhi 2005;41:812–6.

Park WH, Son DH, Yoon SW, Baek SH, Kong SM. The clinical features of Korean patients with Duane's retraction syndrome. Korean J Ophthalmol. 2005;19:132–5.

Mohan K, Saroha V, Sharma A. Factors predicting upshoots and downshoots in Duane’s retraction syndrome. J Pediatr Ophthalmol Strabismus. 2003;40:147–51.

Kekunnaya R, Gupta A, Sachdeva V, Krishnaiah S, Venkateshwar Rao B, Vashist U, et al. Duane Retraction Syndrome: Series of 441 Cases. J pediatr Ophthalmol Strabismus 2012;49:164–9


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