|Year : 2013 | Volume
| Issue : 2 | Page : 85-86
A rare case of duane retraction syndrome type 1 with exotropia and nystagmus
Sandhya R Kruthiventi, Akash P Shah, Abhay A Lune, Renu M Magdum
Department of Ophthalmology, Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||10-Jan-2014|
Akash P Shah
Department of Ophthalmology, Dr. D. Y. Patil Medical College and Research Centre, Sant Tukaram Nagar, Pimpri, Pune - 411 018, Maharashtra
Duane retraction syndrome is a congenital miswiring of the medial and lateral rectus muscle. We reported a case of 12-year-old female child having left eye abduction limitation with normal adduction, widening of interpalpebral fissure of left eye on attempting abduction. She has 5 ∆ exotropia for distance and orthophoria for near in primary gaze. She has pendular nystagmus also.
Keywords: Duane retraction syndrome, exotropia, nystagmus
|How to cite this article:|
Kruthiventi SR, Shah AP, Lune AA, Magdum RM. A rare case of duane retraction syndrome type 1 with exotropia and nystagmus. Sudanese J Ophthalmol 2013;5:85-6
|How to cite this URL:|
Kruthiventi SR, Shah AP, Lune AA, Magdum RM. A rare case of duane retraction syndrome type 1 with exotropia and nystagmus. Sudanese J Ophthalmol [serial online] 2013 [cited 2020 Feb 17];5:85-6. Available from: http://www.sjopthal.net/text.asp?2013/5/2/85/124842
| Introduction|| |
The incidence of Duane retraction syndrome is approximately 1% of the total cases of strabismus.  Nearly 80% of cases are unilateral and characterized by limited abduction (type 1 Duane's syndrome), limited adduction (type 2 Duane's syndrome), or both (type 3 Duane's syndrome). Duane's retraction syndrome type 1 is associated with either straight eyes or slight esotropia. Here, we have a case associated with type 1 Duane's syndrome with exotropia. Nystagmus is a rare association with Duane's syndrome.
| Case Report|| |
This was a case report of a 12-year-old female child having left eye movement restriction since childhood (informed by her mother) came to us. Her vision in both eyes were 6/36 unaided improved to 6/9 with-1.50 DS. In prism bar cover test she has 5 ∆ exotropia for distance and orthophoria for near in primary gaze [Figure 1]. She has limitation in abduction with widening of interpalpebral fissure in left eye [Figure 2]. Adduction is normal in right eye with elevation of left eye [Figure 3]. She has upshot in adduction. She has exotropia of both eyes on attempted elevation [Figure 4]. She has pendular horizontal nystagmus. On the basis of above examination findings diagnosis of Duane retraction syndrome type 1 with horizontal pendular nystagmus was made. Fundus examination was normal. There was no associated systemic problem.
|Figure 2: Limitation of abduction with widening of palpebral fissure of left eye on levoversion|
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| Discussion|| |
Duane's syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outwards. The syndrome was first described by ophthalmologists Jakob Stilling (1887) and Siegmund Türk (1896) and subsequently named after Alexander Duane who discussed the disorder in more detail in 1905.  Duane's syndrome is most probably a miswiring of the eye muscles, causing some eye muscles to contract when they shouldn't and other eye muscles not to contract when they should.  It is found that in most cases the abducen nucleus and nerve are absent or hypoplastic and the lateral rectus muscle is innervated by a branch of the oculomotor nerve.  This misdirection of nerve fibers results in opposing muscles being innervated by the same nerve. Thus, on attempted abduction, stimulation of the lateral rectus through the oculomotor nerve will be accompanied by stimulation of the opposing medial rectus through the same nerve; a muscle which works to adduct the eye. Thus, co-contraction of the muscles takes place, limiting the amount of movement achievable and also resulting in retraction of the eye into the socket. Most of the patients are diagnosed by the age of 10 years and Duane's is more common in girls (60% of the cases) than boys (40% of the cases) and more on left eye then right eye.  The gene sal-like 4 has been implicated as a cause of this condition.
The characteristic features of the syndrome are:
- Limitation of abduction (type 1 Duane's syndrome) or adduction (type 2 Duane's syndrome) or both (type 3 Duane's syndrome) of the affected eye.
- Retraction of the eyeball into the socket on adduction, with associated narrowing of the palpebral fissure
- Widening of the palpebral fissure on attempted abduction.
- Poor convergence
- A head turn to the side of the affected eye to compensate for the movement limitations of the eye(s) and to maintain binocular vision.
- In the primary position, eyes may be straight, esotropia or exotropia.
In Duane syndrome, the majority of patients remain symptom free and able to maintain binocularity with only a slight face turn. Amblyopia is uncommon and where present, mostly due to anisometropia. This can be treated with occlusion. Any refractive error should be corrected.
| References|| |
|1.||Gurwood AS, Terrigno CA. Duane's retraction syndrome: Literature review. Optometry 2000;71:722-6. |
|2.||Duane A. Congenital deficiency of abduction associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. Arch Ophthalmol (Chicago) 1905;34:133-50. |
|3.||Hotchkiss MG, Miller NR, Clark AW, Green WR. Bilateral Duane's retraction syndrome. A clinical-pathologic case report. Arch Ophthalmol 1980;98:870-4. |
|4.||Fells P, Lee JP. Strabismus. In: Spalton DJ, Hitchings RA, Hunter PA, editors. Atlas of Clinical Ophthalmology. London, New York: Gower Medical Publishing; 1984. p. 6.7. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]