|Year : 1957 | Volume
| Issue : 3 | Page : 64-68
Duane's retraction syndrome
Department of Ophthalmology, Irwin Hospital, New Delhi, India
N S Jain
Department of Ophthalmology, Irwin Hospital, New Delhi
|How to cite this article:|
Jain N S. Duane's retraction syndrome. Indian J Ophthalmol 1957;5:64-8
In a typical case of this syndrome the manifestations are: (1) restricted adduction, (2) shooting of the eye up and in or down and in on attempting adduction, (3) retraction of the eyeball on adduction with pseudoptosis, (4) limited or abolished abduction, (5) widening of the palpebral fissure on attempting abduction and (6) deficient conevergence.
It seems that variations in the clinical appearances from the typical book picture of the retraction syndrome are common. These and the observations made from surgical exploration carried out in four cases to find an anatomical reason for its occurrence are recorded in this paper.
Case I:- A boy aged 3½ years, was a left sided case. The appearance in primary position was excellent [Figure - 1]A with parallel visual axes and equally wide palpebral apertures on the two sides. On dextroversion [Figure - 1]B there was a typical retraction of the left globe causing pseudoptosis and narrowing of the left palpebral aperture but adduction of the eye was normal and elevation of the globe was absent. On laevoversion [Figure - 1]C abduction of the left eye was completely absent hut there was no widening of the palpebral fissure.
Surgically explored under a general anaesthetic, the external rectus was discovered to be possessed of a normal morphology and elasticity under passive traction. No abnormal muscular, fibrous or fascial adhesions were present.
Exploring the medial rectus no abnormal connections between the muscle and the neighbouring structures were likewise seen. Though its morphology seemed normal without any evidence of fibrosis, abduction of the eye could not he brought about on passive traction.
Case 2:- A girl aged 4 years, also left sided, showed a normal adduction and absence of elevation, like Case 1. Abduction was not so completely absent hut was restricted. In attempting abduction the left palpebral aperture, unlike Case 1, became wider. Surgical exploration under anaesthesia only showed absence of abduction of the left eye on passive traction. Other features being normal in both the internal as well as the external recti.
Case 3:- A female aged 8 years, was also a left sided case showing an appearance exactly identical to Case 2. Appearance of the patient in primary position was excellent [Figure - 2]A. On dextroversion there was a normal adduction and absence of elevation of the affected eye [Figure - 2]B, but ocular retraction causing pseudoptosis and narrowing of the palpebral aperture was present [Figure - 2]C. On laevoversion abduction was destricted and in attempting it there was a tendency to widening of the left palpebral aperture. This case was also explored surgically and observations similar to those made in the first two cases were confirmed. Based on these experiences a marginal myotomy of the left internal rectus was performed. Following this the appearance of the eyes in primary position remained unaltered [Figure - 2]D. On dextroversion, the palpebral aperture no longer became narrower due to pseudoptosis and retraction of the globe did not take place [Figure - 2]E, though adduction of the globe had become restricted. Abduction of the left eye was very slightly improved [Figure - 2]F.
Case 4:- A boy aged 6 years came with a convergent squint of 30 in the right eye in primary position. On laevoversion [Figure - 3]A there was an absence of abduction of the left eye and pseudoptosis causing narrowing of the palpebral aperture on the right side which showed a normal adduction but absence of elevation of the right globe. On dextroversion [Figure - 3]B the right eye did not abduct but the left eye showed no ocular retraction, pseudoptosis or narrowing of the palpebral aperture as on the right side. Surgical exploration showed a completely inelastic medial rectus on both the sides but there were no abnormal adhesions.
| Discussion|| |
Clinical Variations:- The following variations in the syndrome are evident from the 4 cases described:- (a) a normal adduction instead of its restriction in all cases, (b) absence of elevation of the globe on adduction in all, and (c) absence of widening of the palpebral aperture in two (cases I & 4) where abduction was so completely absent that the eve did not move beyond the amid line.
Besides the ocular retraction on adduction giving rise to pseudoptosis and narrowing of the palpebral fissure, it can be seen that the only consistent features of this syndrome in this group of cases were restriction of abduction and ocular retraction in all.
Restriction of adduction with elevation of the globe, according to the basic stipulation of the syndrome, therefore, do not appear to be constant features.
Aetiology:- Considering the nature of the horizontal ocular movements wherein the visual axes do not retain parallelism in all directions of the gaze, the whole clinical picture simulates one of an incomitant strabismus. Where limitation of abduction alone is present, as seen in all the cases, the appearance resembles a congenital lateral rectus palsy.
Lyle and Foley (1957) have classified, for the sake of discussion, the entity of Retraction-syndrome under the heading of "Palsy of horizontally acting muscles". Judging from the four cases described the primary anomaly certainly seems to be the congenital palsy of the lateral rectus. It may thus be argued that the difference between a simple case of congenital lateral rectus palsy and a typical case of Duane's retraction syndrome, complete in all respects is one of degree only. This group of cases, perhaps, fall midway between the two to show the connecting link between the simple congenital palsy of the lateral rectus, on the one side, and a typical complicated Duane's retraction syndrome, on the other side. Case 4 of the series lends support to this argument since on one side there was only a lateral rectus palsy and on the other, in addition to this, an ocular retraction also, giving rise to pseucloptosis and narrowing of the palpebral aperture.
The absence of any abnormal structural connections between the muscles and the eyeball, a normal morphology of both the internal and the external recti, and the abolition of retraction and pseudoptosis on adduction after a marginal myotomy of the medial rectus (Case 3). lend a strong support to this view. It is further substantiated from the similarity of this group of cases with those of the medial rectus palsy.
Nutt (1955) in ascribing a cause to this syndrome, uses the term "inelasticity" of the medial rectus. Judging from the similarity of this syndrome to a medial rectus palsy (as discussed in [Table - 2]), the expression of "a contractured state" of the medial rectus in the Retraction syndrome seems more appropriate.
The presence of structural adhesions and supernumerary insertions of the medial rectus may be coincidental occurrences in a few cases as reported by several authors. These, therefore, cannot be accepted as conclusive causes of the syndrome unless demonstrated in a hundred percent cases.
The reason for widening of the palpebral aperture on attempted abduction is not easily understood at the present stage. One thing however is certain, that the theories of migration of regenerating axons-Fuchs (1895) and Bielschowsky (1932), or of an overflow of voluntary reflexes-Gowers (1883) from the nucleus of the VI nerve situated so low down to the branch subserving the levator which is situated so high up, to explain the synkinetic lid retraction are not applicable to this group of cases considering their separation by so many otherwise normally functioning nuclei in between. This also rules out Bushke's view (1925) of a direct intranuclear connection between the nuclei of levator and abduscence to explain such a phenomenon in this group of cases. The lesion in retraction syndrome is through and through congenital and permanent showing no evidence of an attempted regeneration of the palsied lateral rectus.
| Summary|| |
4 cases of the retraction syndrome are described to show clinical variations. It is argued that Duanes retraction syndrome is only a complex variety of congenital palsy of the lateral rectus, the difference between the two being one of degree only.
| References|| |
|1.||Bielschowsky (1935), Arch. of Ophthal. 13, 33. |
|2.||Bushke (1925) Zeits. f. Augen. 55, 344. |
|3.||Fuchs, E. (1895) Beitrag. z. Augen. 2, (11), 12. |
|4.||Gowers (1883) Trans. of Ophthal. Soc., U.K. 3, 286. |
|5.||Jain, N. S. (1957) Brit. J. Ophthal. 41, 247. |
|6.||Lyle, T. K. and Foley, J. (1957) Brit. J. Ophthal. 41, 129. |
|7.||Nutt, A. B. (1955) Annals Royal College of Surgeons, England, 30, 59. |
[Figure - 1], [Figure - 2], [Figure - 3]
[Table - 1], [Table - 2]