|Year : 1991 | Volume
| Issue : 4 | Page : 183-185
Bilateral inverse Duane's retraction syndrome-A case report
Pranab Kumar Chatterjee, Jagneswar Bhunia, Indranil Bhattacharyya
NRS Medical College, Calcutta, India
Pranab Kumar Chatterjee
NRS Medical College, Calcutta-700 037
Source of Support: None, Conflict of Interest: None
Duane's retraction syndrome is a well known congenital musculo-facial anomaly. Various explanations have been given for the aetiology of this syndrome. Inverse Duane's retraction syndrome is a condition with reverse clinical features. Abduction of the affected eye is possible to some extent and is accompanied by retraction of the eyeball, narrowing of the palpebral fissure and pseudoptosis. There may be some restriction of movement on adduction. The primary lesion is suspected to be in the medial rectus muscle. Frequently the muscle is found to be entrapped following trauma to the medial wall of the orbit. A case of bilateral inverse Duane's retraction syndrome and convergent squint along with left-sided perceptive deafness is reported. As is usually the case there was no structural abnormality or entrapment of the muscle from trauma.
|How to cite this article:|
Chatterjee PK, Bhunia J, Bhattacharyya I. Bilateral inverse Duane's retraction syndrome-A case report. Indian J Ophthalmol 1991;39:183-5
| Introduction|| |
This article reports on a female patient with bilateral inverse Duane's retraction syndrome.
Duane's retraction syndrome  is a well known congenital musculo-fascial anomaly. Three types of deformity may occur .
Type 1. This is the most common variety. Here the following features are present.
(a) A marked restriction or complete abolition of abduction of one, or rarely of both eyes.
(b) A slight restriction of adduction of the affected eye.
(c) A marked retraction frequently with an upshoot or a downshoot of the affected eye on attempted adduction.
(d) A narrowing of the palpebral aperture with some degree of ptosis on adduction (due to retraction of the eye).
(e) A slight widening of the palpebral aperture with some retraction of the upper eyelid on attempted abduction (due to a minimal protraction of the eye).
(f) A deficiency of convergence wherein the affected eye fails to converge fully while the sound eye converges normally.
Type 2. This is rarer. Here there is defective abduction with normal adduction and convergence.
Type 3. In this variety both adduction and abduction are defective but the former considerably more than the latter.
The aetiology of the syndrome is complex. Recent electromyographic data suggest that there is a paradoxic innervation in Duane's syndrome . Co-contraction of the medial and lateral rectus muscles in adduction was thought to be responsible for the retraction of the globe by Breinin . Another explanation is that the oculomotor nerve may send fibres to the lateral rectus, causing ocular retraction durinq adduction . There has been yet another suggestion that most of the innervation to the extraocular muscles reaches them via the gamma efferent fibres. These carry impulses to the muscle spindles. The spindles function as "length setters" or automatic load compensators. They act via the servo-loop through the spindle afferents and the main alpha motorneurones. There exists a direct anatomical link within the muscle by which the alpha motorneurones influence the discharge of the spindle afferents. Should this link be disturbed by the muscle being abnormally stiff, then this servo-loop would be broken or drastically modified. As a result much of the innervation destined for the alpha motorneurones and the main muscle fibres would never reach them.
Inverse Duane's retraction syndrome is a condition with reverse clinical features ,. Abduction of the affected eye is possible to some extent and is accompanied by retraction of the eyeball, narrowing of the palpebral fissure and pseudoptosis. There may be some restriction of movement on adduction. The primary lesion is suspected to be in the medial rectus muscle. Frequently the muscle is found to be entrapped following trauma to the medial wall of the orbit.
In both the conditions, with the limitation of abduction, the patient generally shows an esotropia in the primary position.
| Case report|| |
A twenty-year-old female complained of her unsightly squint (present since birth). No living member on either the paternal or maternal side had squint. There was no history of forceps delivery or trauma.
Visual Acuity : RE 6/9 and N5 unaided. LE 6/6 and N5 unaided.
Cover test : Alternating convergent squint.
Ocular movements: Abduction restricted on both sides. Retraction of both eyeballs and narrowing of the palpebral fissures on attempted abduction and widening of the palpebral fissures on adduction. All synkinetic movements were prominent on the right and less conspicuous on the left side [Figure - 1][Figure - 2].
Fixation : Central in both eyes.
Major amblyoscope : Angle of deviation - RE fixing 23 o
LE fixing 24 o. Binocularity was absent. Thee was alternate suppression and no simultaneous macular perception.
Forced duction test: Marked restriction of abduction. The right eye deviated only 3 o and the left eye 7 o from the primary position. Adduction was full on both sides.
In addition to the ocular findings there was an associated left-sided perceptive deafness.
Radiological examination did not reveal any abnormality in the spine, Cranium or orbital structures. No biochemical investigations were of value.
A 5.5 mm recession of the medical rectus was carried out on either side. At operation extensive fibrous bands from the medial recti muscles to the medial orbital walls were encountered. These were more numerous on the right side. They had to be dissected in order to free the muscles.
The result was cosmetically acceptable. There was a residual esotropia of about 7 o in both the eyes [Figure - 3].
| Discussion|| |
Duane's retraction syndrome is commoner in females. It is usually thought to occur as an isolated sporadic entity although familial patterns have been observed in 5 to 10 % cases . Even though involvement is most frequent on the left side  it is bilateral in 18% of cases. A number of associated findings have been reported with the retraction syndrome .This has led some authors to propose that a teratogenic insult at about eight weeks' gestation may be the cause. The associated anomalies include spinal deformities, ear malformations and hearing defects, palatal changes, epibulbar dermoids or lipodermoids, naevus of Ota, Brown tendon sheath syndrome, microcornea, Smith-Lemli-Opitz syndrome and Wildervanck syndrome. Perceptive deafness is associated in 18% of cases .
Inverse Duane's retraction syndrome is very uncommon. Bilateral cases are even more rare. The fact that other congenital anomalies can be associated with this condition also is justified by the finding of perceptive deafness. One feature of this particular case is noteworthy. Most cases of the inverse retraction syndrome result from entrapment of the medial rectus muscle in the medial orbital wall because of trauma. However, here there was neither a history of injury nor was there any structural abnormality in the orbit.
| Summary|| |
A twenty-year-old female was found to have bilateral inverse Duane's retraction syndrome and convergent squint along with left-sided perceptive deafness. The cause of the condition could not be established. Operation for the squint resulted in almost straight eyes in the primary position.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3]