|Year : 2017 | Volume
| Issue : 1 | Page : 28-30
A rare case of Duane's retraction syndrome: Type 3 with exotropia and nystagmus
Department of Ophthalmology, Dr. Yashwant Singh Parmar Govt. Medical College, Nahan, District Sirmour, Himachal Pradesh, India
|Date of Web Publication||19-Sep-2017|
Pine Castle, Near Mist Chamber, Khalini, Shimla - 171 002, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Duane's retraction syndrome (DRS) is a well-known congenital ocular motility disorder. It has been described as the consequence of a congenital anomaly of the 6th cranial nerve nuclei with aberrant innervations by supply from the 3rd cranial nerve. The syndrome includes abduction deficiency, adduction deficiency, or a combination of the two. Compensatory head adaptation helps maintain binocular vision. The purpose of this study is to report a rare case of DRS with a probable inverse DRS (occurrence of both the forms together is a rare phenomenon) presenting to a secondary care institute in this hilly terrain and highlight the ocular findings.
Keywords: Abduction, adduction, Duane's
|How to cite this article:|
Chauhan A. A rare case of Duane's retraction syndrome: Type 3 with exotropia and nystagmus. Sudanese J Ophthalmol 2017;9:28-30
| Introduction|| |
Duane's retraction syndrome (DRS) is a congenital ocular motility disorder. In the most common type of DRS, there is marked restriction of abduction, with eyeball retraction and narrowing of the palpebral aperture on adduction. DRS is rare in the general population with an incidence of about 0.1%. Duane was the first to recognize the retraction syndrome. Herein, we report an undiagnosed patient of DRS reporting to a secondary care institute.
| Case Report|| |
A 12-year-old girl along with her parents presented to us for routine ocular examination. There were no ocular complaints and the parents had come only to get the child's eyesight tested. The girl was wearing glasses for the past 5 years which was prescribed to her by some local spectacle makers for her decrease in vision. Her medical history, developmental history, prenatal, perinatal, postnatal, and family histories were all unremarkable. On examination, her general physical and systemic examination was normal. An occasional head tilt was seen to the right and left. Her uncorrected visual acuities were 6/6P (P implies partial, i.e., the patient reads some but not all letters in the line) in the right eye (O.D.) and 6/9P in the left eye (O.S.). Cycloplegic refraction was carried out and the improvement noted were 6/6 in the right eye (with plus 0.25 Diopter spherical lens) and 6/9 in the left eye (with plus 0.50 Diopter cylindrical lens at 100°). Pupillary reactions, color vision, slit lamp examination, fundus examination, and intraocular pressure were normal. Nystagmus was elicited on extreme lateral gaze to the left. Confrontation visual fields were full in both eyes. Both global and contour stereopsis were present.
Hirschberg corneal reflex test revealed 15° exotropia (left eye). Direct cover test confirmed the manifest squint and alternate cover test confirmed the alternating pattern (left > right). Forced duction test did not reveal any restrictive pathology. Eyelid measurements in primary gaze showed palpebral apertures of 10 mm O.D. and 10 mm O.S. Necessary requirements for performing other tests for sensory anomalies were not available at the secondary care institute.
- There was marked narrowing of palpebral aperture of the left eye (4 mm) along with an upshoot phenomenon of the left eye on adduction [Figure 1]a and [Figure 1]c plus limited adduction in the left eye [Figure 1]f plus mild narrowing of palpebral aperture (8 mm) of the right eye on abduction [Figure 1]c (this is contrary to cases where the palpebral aperture of the abducting eye remains same of increases). This could be inverse DRS in the other eye, but the occurrence of both the forms together has rarely been reported after we undertook an extensive literature search on the internet
- On the contrary, widening of the palpebral aperture of the abducting eye (right eye) was seen on dextroelevation and dextrodepression [Figure 1]a and [Figure 1]f with upshoot phenomenon again prominently seen in [Figure 1]a
- Limited abduction of the left eye was seen [Figure 1]c]. Mild weakness in adduction of the right eye was also noted
- An occasional head tilt to both the right [Figure 1]b and [Figure 1]g and left sides was seen which could be a compensation for DRS in the left eye and probably its inverse form in the other eye
- Limited convergence [Figure 1]d.
Based on the above findings, a diagnosis of DRS-3 was made. No congenital malformations or systemic associations were noted in our patient. The complete hematological investigation and computed tomography head of the patient were carried out and were within normal limits. Magnetic resonance imaging (MRI) was advised to the patient as its facility was not available in our institute. The patient was prescribed glasses, surgical prognosis explained and we are awaiting a follow-up from her.
| Discussion|| |
Stilling, Türk, and Alexander Duane were the first to describe this particular form of strabismus, and hence, it is also referred to as the Stilling–Turk–Duane syndrome. It is usually unilateral and sporadic; however, cases of familial transmission, mostly bilateral with an autosomal dominant inheritance pattern, have been reported. Majority of studies pointed out up to 60% predominance of the syndrome among females, with left side being affected predominantly. DRS is unique in causing narrowing of the palpebral fissure on adduction with globe retraction secondary to cocontraction of both medial and lateral recti muscles on the affected side. Indeed, there is evidence that the lateral rectus muscle of the affected eye is partially innervated by branches from the inferior oculomotor nerve as supported by early electromyographic studies. The upshoot or downshoot that occurs when an eye is adducted in DRS is believed to be related to a taut lateral rectus muscle. When an eye is adducted, the lateral rectus muscle is slipped over the globe, producing this abnormal eye movement.
Huber's classification for DRS is as follows: Type I - palsy of abduction with retraction on adduction, Type II - palsy of adduction with retraction and intact abduction, Type III - palsy of abduction and adduction with retraction on attempted adduction. Other features being shooting of the eye up or down on attempting adduction, widening of the palpebral fissure on attempting abduction, and deficient convergence. According to several studies, DRS Type I is more common than Types II and III. Upshoots and downshoots are statistically significantly more common in Type III DRS, with exotropia as in our case.
Upshoot and downshoot were present in 25%–39% of patients with DRS, being significantly higher in adults than in children while in our case, this phenomenon was seen in a young child. Systemic associations are present in up to 30% of cases and include limb abnormalities, cardiac abnormalities, neurosensory deafness, Goldenhar syndrome, and congenital gustolacrimal reflex (“crocodile tearing”).
The role of electromyography and MRI as diagnostic tool in DRS has been advocated. The major indications for surgical correction in patients with Duane's syndrome are an abnormal head position >15° and/or a significant deviation in primary position. Several surgical options have been described in literature. These include ipsilateral rectus muscle recession, vertical rectus muscle transposition, lateral posterior fixation sutures, simultaneous medial and lateral rectus recessions, and surgery on the normal eye. Y-splitting and recession of the lateral rectus muscle are safe and simple procedure.
| Conclusion|| |
Most patients with DRS are asymptomatic and maintain binocularity with head tilt while amblyopia is seen in some. Predominant female involvement, head tilt, and characteristic ocular movements which are associated with this syndrome were seen while systemic associations were not seen in our case. A high degree of suspicion on part of the ophthalmologist is required in clinching the diagnosis.
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.
We would like to thank our patient.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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