Indian Journal of Ophthalmology

ARTICLES
Year
: 1973  |  Volume : 21  |  Issue : 4  |  Page : 171--177

Duane's retraction syndrome- (a new classification and aetiopathogenesis)


S.R.K Malik, Gurbax Singh 
 Department of Ophthalmology Maulana Azad Medical College New Delhi, India

Correspondence Address:
S.R.K Malik
Department of Ophthalmology Maulana Azad Medical College New Delhi
India




How to cite this article:
Malik S, Singh G. Duane's retraction syndrome- (a new classification and aetiopathogenesis) .Indian J Ophthalmol 1973;21:171-177


How to cite this URL:
Malik S, Singh G. Duane's retraction syndrome- (a new classification and aetiopathogenesis) . Indian J Ophthalmol [serial online] 1973 [cited 2020 Apr 9 ];21:171-177
Available from: http://www.ijo.in/text.asp?1973/21/4/171/34624


Full Text

DUANE [11] was the first to recognise the retraction syndrome of the globe and reported 14 cases. Subsequently similar cases were reported under Duane's retraction syndrome by several authors .[1],[4],[6],[8],[12],[17],[22],[23],[25],[26],[28],[29],[29],[30],[35]

SCOBEE [33] further subdivided Duane's syndrome into two types:­

1. Cases where the lateral rectus was a cord like fibrous band.

2. Cases where fibrous bands were found in relation to the medial rectus muscle. BROWN [9] further modified that classification into (i) typical type-A (ii) typical type-B and (iii) a typical type-C.

In this paper, 17 of our cases of Duane's retraction syndrome have been analysed. As some of these cases do not fit into Brown's or Scobee's classification, a new classi­fication is suggested.

 Methods and Materials



17 cases of Duane's retraction syndrome were detected out of 685 cases of squint investigated in the squint and orthoptic clinic from November, 1969 to September, 1971. The cases were further examined as regards the limitation of movements, variation in size of the palpebral aperture in various directions of gaze and the status of binocularity. Hess charting was done in all the cases.

In 4 cases surgical exploration was carried out. The medial and lateral recti were examined to see if any anomaly of insertion or thickening of muscle sheath was present. The muscles were exposed as far back as possible to find the presence of any fibrous bands. The forced duction test was carried out before and after exposing the muscles. Biopsy was taken from the lateral and medial recti muscles for histopalhology.

 Observations



A. General Factors:

(i) LATERALITY: No case of bilateral Duane's syndrome was seen. 13 cases (76.9%) involved the left eye and the remaining 4 cases the right eye.

(ii) AGE: The age of the patients at the time of first examination ranged from 11 months to 38 years. Most of the patients gave a history of the disability since childhood.

(iii) SEX: Eleven cases were seen in female (64.7%) and six in males.

(iv) NARROWING OF PAL­PEBRAL APERTURE:-Narrow­ ing of palpebral aperture in the primary position was seen in 4 cases, which increased on attempted adduc­tion. Narrowing of the palpebral aperture of the affected eye on attempted adduction and widening on attempt­ed abduction was observed in all cases.

(v) TYPE OF DEVIATION IN PRIMARY POSITION: The angle of deviation was small as compared with the considerable limitation of move­rraent. In four cases, there was no deviation in the primary position. The deviation ranged from 5 to 25° in 9 cases of exotropia and 10 to 20° in 4 cases of esotropia.

(vi) UP-SHOOT OR DOWN SHOOT IN ADDUCTION AND ABDUCTION: In 12 cases (75.7%) there was upshoot of the affected eye on adduction while in 5 cases no vertical shoot was observed. No vertical shoot was observed on abduction.

(vii) BINOCULARITY: Binocular vision up to the grade of stereopsis was seen in 8 cases, upto fusion in 5 cases; while binocularity could not be ascertained in 3 cases on account of very young age and lack of co-operation of the patient.

(viii) OPERATIVE OBSERVA­TION: In 4 cases medial and lateral rectus muscles were exposed. No abnormal fascial bands or mal-insertion of the muscles was seen. To the naked eye the muscles, as far back as could be examined, appeared normal. Muscle biopsy revealed normal structure without any evidence of hypo or hyperplasia. The forced duction test showed no limitation of passive movement in any direction.

B. Classification:

The cases of Duane's retraction syndrome were classified on the basis of limitation of abduction and adduc­tion movement of the globe into the following types:­

Type-A: (11 cases) Cases in which both abduction and adduction move­ments were defective but abduction was more than adduction [Figure 1].

Type-B: (3 cases) Cases in which only abduction movement was restrict­ed or absent while adduction was normal [Figure 2].

Type-C: (1 case) Cases in which both abduction and adduction move­ments were defective but adduction was more than the abduction [Figure 3].

Type-D: (2 cases) Cases in which only adduction movement was defective but abduction was normal. Two cases of this type were seen [Figure 4].

 Discussion



In this study, we noticed that most of the cases of Duane's syndrome affected the left eye for which no suitable explanation can be given. This observation is also reported in the literature. [2],[4],[11],[12],[23],[29]

We found Duane's retraction syndrome more common in females (64.7%)than males (35.3%). The females and males ratio was found to be 2 : 1 by PAWHA AND AWASTHI [28] and all others are in general agreement that females are affected more commonly.

We did not observe any case of bilateral Duane's retraction syndrome. However 18 cases of bilateral Duane's syndrome have been reported in the literature. [1],[4],[11],[12],[26],[28],[30]

In this study all the cases were sporadic with no family history. DUANE; [11] KEITH; [22] PAWHA AND AWASTHI [28] and PAWHA [29] too did not find any hereditary tendency. However WAARDENBERG [36] and FRANCOIS [14] discovered an irregular autosomal dominant mode of inheritance in some of the cases.

Narrowing of the palpebral aperture (Pseudo-Ptosis) on attempted adduc­tion is a well known feature of the syndrome and was present in all our cases. In this study, we found a narrowed palpebral aperture even in primary gaze in four of our cases. The narrowing of palpebral aperture on adduction has been explained by the influences of obliques; [11] posteroward traction by the insertion of the medial r-ectus or its strip posterior to the equator, [14],[29] vicarious contraction of the vertical recti muscle or by synergistic action of 3rd and 7th cranial nerve. [1] HOYT AND NACHTI­GALLER [20] explained the pseudo-ptosis due to the supply of the lateral rectus by an extra branch of the 3rd nerve. We agree with HOYT AND NACHTI­GALLER that the abberant nerve supply of lateral rectus muscle increases the tendency for narrowing of the palpebral aperture on adduction by its synergistic action with the medial rectus. It is possible that synergistic contraction might occasionally be present in the primary position. This view is further supported by the absence of bands, abnormal insertion, abnormal thicken­ing of sheath of muscles in cases explored surgically.

The classification of Brown's and Scobee's although widely accepted are unsatisfactory because some of the cases cannot be included in either. Hence it was felt necessary to reclassify the subject of Duane's retraction syndrome. (see observations).

Aetiopathogenesis

The aetiology of Duane's syndrome is a controversial subject. Many hypothesis have been put forward to explain it. There are two schools of thought. One school believes that the defect is peripheral and the second school thinks it to be central.

Those who believe in the peripheral defect explain this syndrome on the basis of anomaly in the extra ocular muscles or their tendons. [1],[37]

Some assumed that the insertion of the medial rectus muscle is far posterior on the globe and has inelastic adhesions. [14],[16],[18]

GIFFORD [25] suggested birth trauma of the nature of haemorrhage in the muscle sheath of lateral rectus as the etiological factor of this syndrome, a concept no longer believed now, as Duane's syndrome is reported in cases delivered even in Caesarean section.

This peripheral mechanistic and myogenic view is supported by demons­tration of abnormalities in the form of bands [1],[23] histological demonstration of hypoplasia of the lateral rectus and hyperplasia of medical rectus [26] thicken­ing of Tenon's capsule or muscle insertion. Histological examination did not reveal any hypo or hyperplasia in muscle biopsies in the 4 cases explored surgically.

The workers of the second school of thought believe in a defective innervational mechanism of supra­nuclear origin. Their concept embraces the paradoxical inner­vational pattern or abnormal synergism in the firing of impulses to the medial and lateral recti muscles. The support to this view is led by the demonstra­tion of the electromyographic study cases of Duane's retraction syndrome by BREININ; [5] SATO; [32] PAPST AND ESSLEN; [31] ORLOWSKI AND WOJITOWICZ; [27] BURGER, [10] BLODI et al, [6] and ZOLOG et al. [38] However the view does not explain the existence of the unilateral abnormalities in the muscles and absence of the signs of brain stem involvement.

PAHWA AND AWASTHI, [28] PAHWA, [29] and ALEXANDER [2] are of the view that more than one aetiological factor is operating in the different types of Duane's syndrome.

We, however, believe that the mechanism which produced Duane's retraction' syndrome is peripheral innervational defect of omission, substitution or duplication of the nerve supply of the lateral rectus. This is hinted by HUBER et al[33] and HOYT AND NACHTIGALLER.[22]

In type A & B of Duane's retraction syndrome, we presume that the abducent nerve to the lateral rectus has either regressed or not at all developed, hence the lateral rectus gets its nerve supply from the 3rd nerve. This will explain normal lateral and medial rectus muscles on naked eye and histological examina­tion and presence of normal forced duction test. The paradoxical electro myographic discharge in the horizontal recti can also be explained by this view. Support is lent to this view by the absence of 6th nerve and its nuclei in the cadavers [34],[35] and in one human embryo.[36] A branch of 3rd nerve en­tering the lateral rectus muscle was found in these cases.

Type C & D could be due to dual nerve supply of the lateral rectus i.e. 6th nerve and a branch from 3rd nerve. Dual nerve supply of the lateral rectus was found by FASEBECK [37] AND HENLE [38] in cadavers. Hence abduction may be slightly limited or normal depending upon the nervous control of muscles by each branch. Adduction will be defective as the lateral rectus will undergo simultaneous co-contraction on attempted adduction. This will also explain the retraction of the globe in the adducted position.

 Summary



1 . 17 cases of Duane's retraction syndrome are presented. All these cases were unilateral.

2. The left eye was involved more commonly than the right.

3. The condition was more common in females.

4. Some of the cases did not fit into the present day classification and therefore a new classification has been suggested. Type A, B, C, & D cases are illustrated.

5. Aetiopathogenesis of the syndrome is discussed in which peripheral innervation defect of sub­stitution, omission and duplication of the nerve supply of lateral rectus muscle is considered to be the causative factor and supporting evidence is presented.

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