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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 948-951

Superior rectus recession combined with lateral rectus recession and Y-split surgery for exotropia with large comitant innervational hypertropia in a case of type III Duane retraction syndrome


1 Department of Pediatric Ophthalmology, Strabismus and Neuroophthalmology, MS Ophthalmology, Dr. Om Parkash Eye Institute, Amritsar, Punjab, India
2 Department of Pediataric Ophthalmology, Strabismus Abd Neuro-Ophthalmology, MS Ophthalmology DNBE, Dr. Shroff's Charity Eye Hospital, Daryaganj, Delhi, India

Date of Submission11-Feb-2022
Date of Acceptance20-Jul-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Suma Ganesh
Dr Shroff Charity Eye Hospital, 5027, Kedar Nath Road, Daryaganj, New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_421_22

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  Abstract 


The aim of this study is to report a case of hypertropia with severe globe retraction and innervational upshoot present in type III Duane retraction syndrome (DRS) and its management. We report a case of a 24-year-old male with type III DRS with large hypertropia and exotropia, 90° upshoot (grade 4) on attempted adduction, globe retraction on adduction and depression and significant face turn. MRI brain and orbit revealed absence of right abducens nerve with relative low position of horizontal rectus muscles, representing the bridle effect, with normal inferior rectus and superior rectus. The case was managed by performing right eye lateral rectus recession with Y-split for the severe upshoot and superior rectus recession for the hypertropia. Recession of the superior rectus muscle along with lateral rectus recession with Y-split is a safe and effective treatment of type III DRS cases with hypertropia and exotropia.

Keywords: Hypertropia, lateral rectus recession with Y-split, superior rectus recession, type III DRS


How to cite this article:
Baldev V, Ganesh S. Superior rectus recession combined with lateral rectus recession and Y-split surgery for exotropia with large comitant innervational hypertropia in a case of type III Duane retraction syndrome. Indian J Ophthalmol Case Rep 2022;2:948-51

How to cite this URL:
Baldev V, Ganesh S. Superior rectus recession combined with lateral rectus recession and Y-split surgery for exotropia with large comitant innervational hypertropia in a case of type III Duane retraction syndrome. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Nov 27];2:948-51. Available from: https://www.ijoreports.in/text.asp?2022/2/4/948/358171



Type III Duane retraction syndrome (DRS) is a form of congenital cranial disinnervational syndrome presenting with absence or presence of abducens nerve on the affected side with limited abduction and adduction, palpebral fissure changes, globe retraction, and upshoots or downshoots of the affected eye on adduction.[1] Vertical strabismus in the primary position is more often observed in patients with type III DRS, but few cases have been reported in the literature as it is less commonly observed.[2],[3]

We report a case of DRS type III with MRI finding suggestive of absent abducens nerve nucleus, relative low position of horizontal recti, presenting with large hypertropia and exotropia, grade 4 (90°) upshoot on attempted adduction, globe retraction on adduction and depression and a significant face turn.


  Case Report Top


A 24-year-old male presented with history of inability to move the right eye temporally and nasally since birth and was not happy with a very conspicuous, small-looking right eye, especially when looking nasally. There was a history of adopting an abnormal head posture while concentrating on distant objects. 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 (BCVA), measured using Snellen's chart, was 6/6 in both eyes with refraction of −4.0/−0.75 × 10° and −4.5/2.0 × 170°in the right and left eye, respectively. There was compensatory head posture (CHP) with 15° face turn to the left side, as measured with a goniometer [Figure 1]. Extraocular movements showed abduction limitation of −3 and adduction limitation of −3 in the right eye with decrease in palpebral fissure height on adduction [Figure 2]. There was grade 4 (pumpkin-seed sign) upshoot of the right eye on adduction with grade 3 (72%) globe retraction on adduction and mild globe retraction on depression.[4]
Figure 1: Clinical photograph showing 15° face turn to the left side, as measured with a goniometer

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Figure 2: Nine-gaze clinical photographs showing abduction and adduction limitation (red arrows) with narrowing of palpebral fissure on attempted adduction (red circle). The measurement of deviation for distance fixation in prism diopters is mentioned alongside the photographs. (XT – Manifest exotropia, RHT – Right hypertropia)

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Alternate prism cover test showed manifest right exotropia of 20 prism diopters (PD) with right hypertropia of 20 PD for distance and 30 PD exotropia with 18 PD hypertropia at near. Vertical deviation was comitant in all gazes. Nine-gaze ocular motility pattern and measurement of deviation are shown in [Figure 2]. He had binocular single vision with CHP and right-eye suppression without CHP (Worth four dot test) for distance and near and stereopsis of 80 seconds of arc (Randot test). Fundus examination was unremarkable.

MRI brain and orbit revealed absence of cisternal segment of right abducens nerve with relative low position of right eye horizontal rectus muscles, representing the bridle effect [Figure 3]. Based on clinical findings and imaging, a diagnosis of type III exotropic DRS, grade 4 upshoot, grade 3 globe retraction, and significant face turn was made.
Figure 3: Axial MR 3D FIESTA image (a) showing expected location of absent cisternal segment of the right abducens nerve (white arrows), and Coronal T1W MR image (b) showing relatively low position of horizontal rectus muscles (white arrowheads), representing bridle effect

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Intraoperative forced duction test revealed +2 tightness of medial rectus (MR) and lateral rectus (LR) in right eye with +1 tightness of superior rectus (SR), but it was negative for left eye in all directions. The patient underwent right eye lateral rectus recession of 12 mm depending on tightness intraoperatively, and further Y-split of LR was done. The muscle was split into upper and lower equal halves as far back as possible and then the two arms were spread apart by a total of 20 mm and reattached after recession at upper and lower border so that the muscle was spread out 20 mm apart. Also, for a tight SR, recession of 7.5 mm was performed. A muscle slip was noticed under the LR muscle originating from the muscle belly, inserting 3 mm behind the insertion of LR muscle [Figure 4], which was dissected. One month postoperatively, the patient's face turn improved completely with orthophoria in the primary position. The pumpkin-seed sign grade 4 upshoot on adduction was eliminated [Figure 5]. Globe retraction did not worsen but on adduction improved from 74% to 54% and was cosmetically acceptable to the patient. The patient developed binocular single vision in primary position.
Figure 4: Intraoperative photograph showing muscle slip (red arrow) present, originating from the belly of the lateral rectus (LR) (yellow arrow) and inserting 3 mm behind insertion of LR

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Figure 5: (a) Nine-gaze clinical photographs showing postoperative orthophoria in primary gaze with reduced elevation on attempted adduction. (b) Clinical photographs showing improved face turn, postoperatively

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  Discussion Top


In unilateral DRS, type I is the most common presentation followed by type III and type II. In type III DRS, it is observed that horizontal strabismus and orthophoria are all equally common.[4] Along with horizontal strabismus, vertical strabismus is also seen in type III DRS.[3],[4] The vertical deviations may be due to fibrosis of the vertical muscles, innervational anomalies, or tethering effect of the horizontal muscles.[5],[6]

It has been reported that the innervational type of upshoot can be characterized by the presence of hypertropia in primary position with gradual elevation on adduction.[7] Many electromyographic studies have shown that there is co-contraction of the superior rectus with lateral rectus with or without inferior oblique in cases of upshoots.[8],[9] In our case, there was presence of hypertropia in the primary position along with gradual elevation of the eye on adduction, and tight forced duction test on lateral rectus, and superior rectus MRI brain and orbit revealed absence of cisternal segment of the right abducens nerve with normal inferior rectus and superior rectus. The horizontal recti were at a relatively low position, representing the bridle effect, which could be responsible for the pumpkin-seed sign.

Hence, based on the above findings, we suggest that the upshoot in this case was of innervational type with large hypertropia.

Additionally, there was severe globe retraction (74%) in our patient, which we thought could worsen with Y-split. Thus, choosing the appropriate management was important to alleviate both globe retraction and upshoot. Various treatment modalities for upshoot and downshoot are recession of lateral and medial rectus muscles,[10] posterior fixation suture of horizontal recti or the lateral rectus muscle alone, vertical rectus recession,[5] and Y-splitting of lateral rectus muscle at insertion.[11]

In our case, right eye lateral rectus recession of 12 mm for severe globe retraction, Y-split for the upshoot, and superior rectus recession of 7.5 mm for the hypertropia were performed, thereby eliminating the upshoot and improving the globe retraction.

Therefore, we report this case, discussing the management and outcome of large vertical deviations associated with exotropia in type III DRS with severe innervational upshoot.


  Conclusion Top


In conclusion, hypertropia with severe globe retraction and innervational upshoot can present in type III DRS cases, and recession of the superior rectus muscle along with lateral rectus recession with Y-split is a safe and effective treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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.  Back to cited text no. 1
    
2.
Yüksel D, Orban de Xivry JJ, Lefèvre P. Review of the major findings about Duane retraction syndrome (DRS) leading to an updated form of classification. Vision Res 2010;23:2334-47.  Back to cited text no. 2
    
3.
She HC, Man FY, Wang ZC, Wang JH, Jiao YH, Zhao KX. Vertical deviation and A pattern of type III Duane retraction syndrome. Chin Med J 2012;125:2929-32.  Back to cited text no. 3
    
4.
Kekunnaya R, Negalur M. Duane retraction syndrome: Causes, effects and management strategies. Clin Ophthal (Auckland, NZ) 2017;11:1917-30.  Back to cited text no. 4
    
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.  Back to cited text no. 5
    
6.
Gurwoods AS, Terrigno CA. Duane's retraction syndrome: Literature review. Optometry 2000;71:722-76.  Back to cited text no. 6
    
7.
Kraft SP. A surgical approach for Duane syndrome. J Pediatr Ophthalmol Strabismus 1988;25:119-30.  Back to cited text no. 7
    
8.
Scott AB, Wong GY. Duane's syndrome: An electromyographic study. Arch Ophthalmol 1972;87:140-7.  Back to cited text no. 8
    
9.
Metz HS, Scott AB, Scott WE. Horizontal saccadic velocities in Duane's Syndrome. Am J Ophthalmol 1975;80:901-6.  Back to cited text no. 9
    
10.
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. 10
    
11.
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. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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