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Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 108-110

Duane's retraction syndrome with severe upshoot and ipsilateral superior rectus contracture: A rare presentation

1 Department of Ophthalmology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Pediatric Ophthalmology and Strabismus, Dr. Shroff's Charity Eye Hospital, New Delhi, India

Date of Submission30-Jul-2013
Date of Acceptance09-Oct-2013
Date of Web Publication11-Apr-2014

Correspondence Address:
Priyanka Arora
Department of Ophthalmology, Dayanand Medical College and Hospital, Ludhiana - 141 001, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-3897.130544

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Type III Duane's retraction syndrome (DRS) is a rare condition and represents only 1% of all cases of Duane's syndrome. It consists of limited/absent abduction as well as adduction of the affected eye. A characteristic upshoot/downshoot/both, in adduction, and globe retraction may occur in DRS patients. Secondary muscle changes like medial rectus contracture or superior rectus contracture (SRC) may rarely be associated with DRS. SRC has been reported in patients with unilateral superior oblique palsy and dissociated vertical deviation. However, no case report of association of SRC with DRS has been reported. We report a rare case of type III DRS with severe upshoot, globe retraction and ipsilateral SRC. It is hypothesized that a long standing severe upshoot could be the reason for development of secondary SRC. The condition was treated with Y-split of lateral rectus and superior rectus recession.

Keywords: Duane′s retraction syndrome, superior rectus contracture, upshoot

How to cite this article:
Arora P, Ganesh S, Shanker V. Duane's retraction syndrome with severe upshoot and ipsilateral superior rectus contracture: A rare presentation. J Clin Ophthalmol Res 2014;2:108-10

How to cite this URL:
Arora P, Ganesh S, Shanker V. Duane's retraction syndrome with severe upshoot and ipsilateral superior rectus contracture: A rare presentation. J Clin Ophthalmol Res [serial online] 2014 [cited 2023 Mar 24];2:108-10. Available from: https://www.jcor.in/text.asp?2014/2/2/108/130544

Duane's retraction syndrome (DRS) is an unusual congenital form of strabismus characterized by limitation of horizontal eye movements and globe retraction with palpebral fissure narrowing on attempted adduction. [1] Type III DRS is rare; representing only 1% of all cases of DRS and consists of limited/absent abduction with variable limitation of adduction. A characteristic upshoot/downshoot or both may occur in adduction. Secondary muscle changes like medial rectus or superior rectus contracture (SRC) may rarely be associated with DRS. [2]

SRC has been described in patients with unilateral superior oblique palsy. [3] Its association with dissociated vertical deviation (DVD) has been reported, [4] but no study in literature has reported SRC in DRS. We report a rare case of type III DRS with SRC.

  Case Report Top

A 23-year-old male presented with the deviation of left eye since birth. There was history of adopting an abnormal head posture while concentrating over distant objects. There were no preceding illnesses and the patient's birth history, family history and medical history were noncontributory. There was no history suggestive of any surgical intervention.

On examination, his best corrected visual acuity was 20/20 in both the eyes with no significant refractive error, with a near vision of N6 in both eyes. He preferred his right eye to fixate and adopted a face turn to right. Ocular motility testing was notable for limitation of adduction (–2) as well as abduction (–1) in the left eye with severe upshoot of the left eye in adduction, consistent with the clinical picture of type III DRS in left eye. Prism alternate cover test showed incomitant left hypertropia (LHT) in various gaze positions. LHT measured 40 prism dioptre (PD) with 25 PD exotropia (XT) in the primary position, 20 PD LHT with 30 PD XT in right gaze and increased to 45 PD with no horizontal deviation on the left gaze [Figure 1]. The deviation in upgaze was measured as 40 PD LHT with 25 PD XT while in downgaze, the deviation was 45PD LHT and 30 PD XT. His LHT was worse on left head tilt (45 PD) than on right head tilt (30 PD) [Table 1]. He had evidence of fusion with Worth 4 dot test in left gaze position (habitual right face turn). However, in other gaze positions, left eye suppression was noted. Stereopsis was not detected with Titmus fly and Wirt circles in any gaze position.
Table 1: Measurements of binocular alignment (prism dioptre) in various gaze positions and head tilts with right eye fixation

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Figure 1: Preoperative (a-i): Ocular alignment in nine cardinal gaze positions. In primary gaze, left eye showed hypertropia and exotropia (e), which increased in left gaze (f). When the left eye was adducted (d), the left eye abruptly moved upward, crossed the horizontal line, and the cornea disappeared under the upper lid (severe left eye upshoot)

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Forced duction test was performed which revealed a tight superior rectus as well as tightness of the lateral rectus of the left eye but was negative for right eye in all directions. Fundus evaluation was within normal limits. To summarize, the presence of large LHT in primary position which increased on ipsilateral gaze and ipsilateral head tilt, suggested the possibility of SRC, which was further confirmed by a positive forced duction test. The presence of upshoot, palpebral fissure changes, and limited ocular movements confirmed the diagnosis of type III DRS.

The patient underwent surgery in the form of both eyes lateral rectus recession of 6 mm with Y-split of left lateral rectus and left superior rectus recession of 6 mm. Postoperatively, the face turn improved significantly, horizontal realignment was complete with marked reduction of LHT in primary position and with similar comitant deviation in all other gazes. The upshoot in adduction was markedly reduced [Table 1], [Figure 2]. Fusion was noted in primary position. In his latest follow-up 1 year postoperatively, there was no evidence of abnormal head posturing and the alignment was stable and cosmetically acceptable.
Figure 2: Postoperative (3months) (a-i): Ocular alignment in nine cardinal gaze postions. Horizontal realignment was complete with marked reduction of left hypertropia in primary position (e), and similar comitant deviation in other gazes (d, f). The upshoot in adduction was markedly reduced (d)

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  Discussion Top

SRC syndrome was first reported in 1964 in patients with unilateral superior oblique palsy. [3] SRC should be suspected when there is hypertropia of >15 PD in primary position which increases in ipsilateral version (difference >5 PD) and on head tilt to the affected side. [5] Tight superior rectus muscle on forced duction test is another clue toward the diagnosis. [5],[6] Jampolsky [7] however suggested that this syndrome is not seen exclusively in superior oblique palsy but also in several other entities such as DVD, thyroid myopathy, orbital floor fracture, sensory exotropia, and monocular elevation deficit.

An upshoot/downshoot is reported to be more frequent in type III DRS. [8] An upshoot may occur due to coinnervation of superior rectus muscle with the lateral rectus or could be because of the mechanical factors as the bridle or leash effect due to tight lateral rectus. [9] The innervational type of upshoot is characterized by the presence of hypertropia in primary position. [10] Another characteristic feature is that in innervational type, there is a gradually increasing upshoot of the eye as it moves in adduction. Some patients may exhibit a combination of both mechanical as well as innervational type. [8] In our patient, the presence of hypertropia and positive Force Duction Test (FDT) for lateral rectus indicate that the upshoot was due to both innervational as well as mechanical factors. So, it seems plausible that the long standing upshoot with large hypertropia in primary position could have resulted in development of SRC in our patient. Khawam et al., [4] explained Jampolsky's findings, that because the fixing (non-Duane's) eye constantly works against the imbalance of innervational forces in the Duane's eye, contracture of the fellow eye (Duane's eye) yoke muscle may result. So, in our patient the SRC could have been the result of long-standing upshoot caused by chronically innervated superior rectus muscle.

Various surgical approaches have been described - including recession of lateral and medial rectus muscles, [11] posterior fixation suture of horizontal recti, [12] lowering of insertion of lateral rectus muscle, vertical rectus recession, [8] and Y-splitting of lateral rectus muscle at the insertion [13] for the treatment of upshoot and downshoot in DRS. The management of SRC includes large recession of the superior rectus muscle. Our patient had type III DRS with severe upshoot, large hypertropia in primary position, and SRC. The condition was successfully managed by superior rectus recession along with lateral rectus recession and Y-split surgery. The lateral rectus of left eye was recessed to counter the resection effect of Y-split.

In conclusion, SRC can occur in association with DRS, due to long-standing upshoot. The clinicians should be aware of the possible combination and plan their management accordingly.

  References Top

1.DeRespinis PA, Caputo AR, Wagner RS, Guo S. Duane's retraction syndrome. Surv Ophthalmol 1993;38:257-88.  Back to cited text no. 1
2.Jampolsky A. Duane syndrome. In: Rosenbaum AL, Santiago AP, editors. Clinical Strabismus Management: Principles and Surgical Technique. Philadelphia: WB Saunders; 1999.  Back to cited text no. 2
3.Scott AB, Jampolsky A. Ocular deviations. Intl Ophthalmol Clin 1964;700.  Back to cited text no. 3
4.Khawam E, Ghazi N, Salti H. "Jampolsky Syndrome": Superior rectus overaction-contracture syndrome: Prevalence, characteristics, etiology and management. Binocul Vis Strabismus Q 2000;15:331-42.  Back to cited text no. 4
5.Molinari A, Ugrin MC. Frequency of the superior rectus muscle overaction/contracture syndrome in unilateral fourth nerve palsy. J AAPOS 2009;13:571-4.  Back to cited text no. 5
6.Jampolsky A. A new look at the head tilt test. In: Fuchs AF, Brandt TH, Buttner U, Zee DS, editors. Contemporary Ocular Motor and Vestibular Research: A Tribute To David A. Robinson. Stuttgart: Thieme Verlag; 1994.  Back to cited text no. 6
7.Jampolsky A. Management of vertical strabismus. Trans New Orleans Acad Ophthalmol 1986;34:141-71.  Back to cited text no. 7
8.Mohan K, Saroha V. Vertical rectus recession for the innervational upshoot and downshoot in Duane's retraction syndrome. J Pediatr Ophthalmol Strabismus 2002;39:94-9.  Back to cited text no. 8
9.Scott AB, Wong GY. Duane's syndrome. An electromyographic study. Arch Ophthalmol 1972;87:140-7.  Back to cited text no. 9
10.Kraft SP. Surgical approach to Duane syndrome. J Pediatr Ophthalmol Strabismus 1988;25:119-30.  Back to cited text no. 10
11.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;114: 311-3.  Back to cited text no. 11
12.Von Noorden GK, Murray E. Up and down shoots in Duane's retraction syndrome. J Pediatr Ophthalmol Strabismus 1986;23:212-5.  Back to cited text no. 12
13.Rao VB, Helveston EM, Sahare P. Treatment of upshoot and downshoot in Duane syndrome by recession and Y-splitting of the lateral rectus muscle. J AAPOS 2003;7:389-95.  Back to cited text no. 13


  [Figure 1], [Figure 2]

  [Table 1]

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