|Year : 2019 | Volume
| Issue : 3 | Page : 101-104
Face turns in Type 1 Duane's retraction syndrome – Right? Left? or none?
Snehal Rhishikesh Thakre, Jyotika Prasanna Mishrikotkar, Pradnya Ashish Deshmukh
Department of Ophthalmology, MGM Medical College, Aurangabad, Maharashtra, India
|Date of Submission||16-May-2018|
|Date of Acceptance||29-Mar-2019|
|Date of Web Publication||11-Dec-2019|
Snehal Rhishikesh Thakre
Department of Ophthalmology, MGM Medical College, N-6 CIDCO, Aurangabad - 431 006, Maharashtra
Source of Support: None, Conflict of Interest: None
Purpose: The purpose of this study is to create awareness about the face turns in Type 1 Duane's retraction syndrome (DRS). Subjects and Methods: This was a retrospective analysis of 27 cases of Type 1 DRS seen in a teaching hospital. A complete strabismus workup with emphasis on deviation in primary gaze and presence and direction of anomalous head posture was done. Results: Of 27 patients, 9 patients (33.33%) had esotropia, 8 (29.63%) had exotropia, whereas 10 (37.03%) were orthotropic in primary gaze; 17 (62.96%) had a face turn, 9 (33.33%) patients had a face turn toward the affected side, 8 (29.63%) toward the sound side, while 10 (37.03%) did not have a face turn. Conclusions: It is important to be aware that patients with Type 1 DRS may have an associated face turn; the direction of which is variable depending on the deviation present in the primary position. Awareness of this interesting observation of the variations in face turn prevents a misdiagnosis of sixth nerve palsy which has more serious clinical implications.
Keywords: Anomalous head posture, Duane's retraction syndrome, face turn, primary gaze, strabismus
|How to cite this article:|
Thakre SR, Mishrikotkar JP, Deshmukh PA. Face turns in Type 1 Duane's retraction syndrome – Right? Left? or none?. J Clin Ophthalmol Res 2019;7:101-4
|How to cite this URL:|
Thakre SR, Mishrikotkar JP, Deshmukh PA. Face turns in Type 1 Duane's retraction syndrome – Right? Left? or none?. J Clin Ophthalmol Res [serial online] 2019 [cited 2020 Feb 26];7:101-4. Available from: http://www.jcor.in/text.asp?2019/7/3/101/272708
Incomitant strabismus (ocular muscle palsy and restrictive strabismus syndromes such as Duane's retraction syndrome (DRS) and Brown's syndrome) and nystagmus are the most common causes of abnormal head posture. Nystagmus is easily ruled out by the presence of the oscillatory movements of the eye, but DRS is often mistaken for sixth nerve palsy. DRS is a clinical ocular disorder consisting of retraction of the globe with narrowing of the lid fissure in attempted adduction, frequent abduction deficiency with variable limitation to adduction, and upshoot and/or downshoot of the affected eye on adduction. Now, it is a part of congenital cranial dysinnervation disorders along with Moebius syndrome and congenital fibrosis of extraocular muscles and is referred to as co–contractive retraction syndromes 1–3.
In 1974, Huber classified DRS into the three types.,
DRS Type I was characterized by marked limitation or absence of abduction, normal or only slightly defective adduction, narrowing of the palpebral fissure and retraction on adduction, and widening of the palpebral fissure on attempted abduction. DRS Type II was described with limitation or absence of adduction with exotropia of the affected eye, normal or slightly limited abduction, narrowing of the palpebral fissure, and retraction of the globe on attempted adduction. DRS Type III was a combination of limitation of both abduction and adduction; retraction of the globe, and narrowing of the palpebral fissure on attempted adduction.
A modification of Huber's classification was proposed by Ahluwalia et al. It included the deviation noted in the primary position of gaze in each of the three groups. It is relevant only for Type 1 DRS as Types 2 and 3 have exotropia in primary gaze. Therefore, Type 1A, 1B, and 1C DRS would be an abduction deficiency with the deviation in primary gaze being an esotropia, exotropia, and orthophoria, respectively.
Yüksel et al. described that face turns were the preferred with the adopted anomalous head posture in patients with DRS. These face turns were variable in direction in Type 1 DRS [Table 1].
Patients with DRS often present with a face turn with or without an obvious deviation. The aim of this face turn is to achieve binocular single vision and fusion. The direction of the face turn depends on the deviation in the primary position.
The purpose of this study is to raise awareness among the general ophthalmologist about the well described but often forgotten fact – the various directions of face turns (as described by Yüksel et al.) in Type 1 DRS, which is not only the most common type of DRS to be seen in clinical practice but also the one which is usually confused with lateral rectus palsy which has more serious clinical implications. Although this fact has been well described and documented in literature, there is a need to emphasize on it. This study is written from the perspective of the general ophthalmologist who is often confounded when seeing a child with a face turn, who is not fitting in a quick diagnosis.
| Subjects and Methods|| |
This was a retrospective analysis of the 27 cases of Type 1 DRS seen in the ophthalmology outpatient department of a medical college and hospital from September 2008 to January 2017. The data included a complete strabismus workup with emphasis on deviation in primary gaze, presence of a face turn, and direction of the face turn if present.
The study protocol was approved by the Institutional Ethical Committee and adhered to the tenets of the Declaration of Helsinki.
| Results|| |
Of the 27 patients of Type 1 DRS, 21 (77.77%) were female and 6 (22.22%) were male. Laterality and side affected are described in [Table 2].
The average age at presentation was 11.22 years (range: 9 months–34 years).
Seventeen (62.96%) of the 27 patients presented with a face turn [Table 3]. Sixteen (37.03%) had a face turn to one side only as expected, 9 (33.33%) patients had a face turn toward the affected side [Figure 1], 8 (29.63%) toward the sound side [Figure 2], while 10 (37.03%) did not have a face turn [Figure 3]. A 10-year-old girl with bilateral Type 1 DRS and esotropia had an alternating face turn toward the affected side depending on which eye she used for fixing.
|Figure 1: Left-sided esotropic Duane's retraction syndrome Type 1 with regular face turn toward the affected side for near and distance|
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|Figure 2: Left-sided exotropic Duane's retraction syndrome Type 1 paradoxical face turn toward the normal side for near and distance|
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|Figure 3: Right-sided orthophoric Duane's retraction syndrome Type 1 with no face turn for near and distance. Upshoot on adduction of RE is noted|
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It was seen that the patients having esotropia had a face turn toward the affected side, those having exotropia had a face turn toward the normal side, whereas those patients who were orthotropic did not have a face turn [Table 4].
|Table 4: Associated Strabismus in Primary Gaze and Type of Face Turn (Regular/Paradoxical)|
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| Discussion|| |
DRS is an incomitant, restrictive strabismus, which may present with a face turn. The direction of the face turn is variable depending on the type of deviation present in primary gaze; which is hypothesized to be either due to flaccidity of the lateral rectus (resulting in esotropia) or a tight lateral rectus (resulting in exotropia). Yüksel et al. explained that the deviation in the primary position in Type 1 DRS is dependent on the amount of fibers that abandon the medial rectus nerve for supply to the lateral rectus. In Type 1A, more fibers supply the medial rectus, and hence, the eye remains in adduction; in Type 1B, more nerve fibres supply the lateral rectus, hence the is abduction, and in Type 1C, there is almost equal distribution of the nerve fibers maintaining the eye in the orthophoric position. This phenomenon leads to progressive change in the compensatory abnormal head posture; face turn toward the affected side, no torticollis, face turn toward the sound side. Thus, the deviation varies in the subtypes of Type 1 DRS. The anomalous head posture is adopted to achieve fusion and binocularity. Face turn associated with limitation of abduction is also seen in sixth nerve palsy, and this often confounds the general ophthalmologist about the diagnosis.
This study attempts to describe the directions of face turn seen solely in Type 1 DRS.
Our 27 patients were almost equally distributed in the types of deviation in primary gaze – 9 (33.33%) had esotropia (one had alternating Esotropia), 8 (29.63%) had exotropia, whereas 10 (37.03%) were orthotropic in primary gaze. In this study, the difference in the type of deviation in primary gaze was not significant, similar to observations made by Kekunnaya et al. and Shrestha and Sharma who in separate studies found no difference in the type of deviation in patients with Type 1 DRS (P > 0.05%). If the subclassification of Type 1 DRS as suggested by Yüksel et al. Ahluwalia et al. were to be applied to these patients, there would be three subcategories of Type 1 DRS – Type 1A Eso-DRS (9, 33.33%), Type 1B Exo-DRS (8, 29.63%), and Type 1C Ortho-DRS (10, 37.03%).
Seventeen (62.96%) of our patients presented with a face turn. Kushner in 1979 found that DRS was the most common cause of abnormal head posture in patients with horizontal incomitance (31/42 cases). Boricean and Bărar noted that DRS was the most common form of face turn in children.
Anomalous head posture in the forms of a face turn is adopted to achieve fusion and binocularity. Hence, if a patient is having esotropia, face turn is toward the affected side, and if there is exotropia, then face turn is toward the opposite side to achieve parallel visual axes. A face turn with a limitation of abduction with an associated exotropia should immediately raise suspicion of DRS Type 1 and not lateral rectus palsy (which should have a history of recent onset, associated with an esotropia and a face turn toward the same side. Furthermore, a patient will complain of troublesome diplopia).
Almost the same number of patients presented with face turn toward affected side (9%–33%), toward the sound side (8%–29.63%) or without face turns. One patient with bilateral DRS and alternating esotropia had an alternating face turn toward the sound side depending on which eye she was using for fixation. In the study by Shrestha and Sharma, 26 (86.7%) patients of Type 1 DRS did not have any anomalous face turn, 3 (10%) had a face turn, and 1 (3.3%) had a head tilt, whereas Meenakshi et al. found a highly significant association between type of DRS and the direction of face turn with 35.29% patients of Type 1 DRS having a face turn to the same side as DRS. Bhate et al. noted that there is a propensity for the face turn to be toward the normal side in exotropic DRS.
| Conclusions|| |
Thus, this study of 27 patients of only Type 1 DRS emphasizes the findings of Yüksel et al. that the patients could present with or without an anomalous head posture in the form of a face turn. The direction of the face turn depended on the type of deviation; the patient had in primary gaze as the purpose of adopting this head posture was to achieve fusion and binocular vision. The fallacies of this study were a small study population and that the stereopsis was not measured in the primary and adopted gaze which would have given an idea about the functional importance of the face turn. Awareness of this well documented but interesting observation of the variations in face turn is important to prevent a misdiagnosis of sixth nerve palsy and also helps to plan for the correction of this anomalous posture.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for 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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2], [Table 3], [Table 4]