Delhi Journal of Ophthalmology

Clinical Analysis, Management and Outcome of Duane’s Retraction Syndrome at a Tertiary Eye Care Centre in South India

Meenakshi Ravindran, Neelam Pawar, Radhika P Karkera, Rajagopal Kunnatur
Pediatric Ophthalmology & Strabismus
Aravind Eye Hospital,
Tirunelveli
Tamil Nadu, India

Corresponding Author:

Meenakshi Ravindran
Senior Consultant & Incharge,
Pediatric Ophthlamology & Strabismus
Aravind Eye Hospital,
Tirunelveli
Tamil Nadu, India
Email id: drmeenakshi@tvl.aravind.org

Published Online: 25-SEP-2013

DOI:http://dx.doi.org/10.7869/djo.2013.21

Abstract
Aim: To study clinical features, management & outcome of Duane’s retraction syndrome (DRS).

Materials & Methods: This prospective, interventional, non randomised study of 102 patients diagnosed with DRS was done from September 2009 to August 2011. Various characteristics studied included sex, age at presentation, laterality, manifest primary position horizontal deviation, upshoot and downshoot, amblyopia, and associated ocular and systemic abnormalities.  Follow up for non surgical patients was for minimum period of 6 months. Surgery was advised in patients with anomalous head posture of more than 15 degrees, cosmetically unacceptable upshoot  or downshoot, significant deviation in primary position and globe retraction. Post operative follow up of surgical cases was done at 1 month and were followed up for a minimum period of 6 months.

Results: : Of the total 102 patients, 92 (90.2%)  had unilateral and 10 (9.8%) had bilateral DRS. Type I (78.57 %) was the most common while Type II (2.68%) was the least common among unilateral as well as bilateral cases. Preponderance for the female gender was seen in unilateral and bilateral cases. Left eye preponderance was seen in unilateral cases. A significant association was found between type of unilateral DRS and type of ocular deviation in primary gaze, direction of face turn, presence of upshoot/downshoot. Exotropia (41.2%) was the most common ocular deviation in primary position in our study. Exotropia was more common in unilateral cases and esotropia more in bilateral cases.Surgical correction was done in 20 patients (22 eyes). Patients with both esotropia and exotropia who underwent surgical correction showed a significant improvement after surgery (p=0.0368 and 0.0011 respectively). There was a significant improvement of abnormal head posture (AHP) and globe retraction post operatively (p =0.014 and p =0.005 respectively).

Conclusion: Unilateral types I, II, and III Duane syndrome and bilateral cases differs in the primary position horizontal deviation, upshoot and downshoot, and associated ocular abnormalities. Surgical intervention is recommended in patients with abnormal head posture (AHP), overshoot, globe retraction and significant deviation in primary position.

Keywords :duane’s retraction syndrome • abnormal head posture • muscle recession

Duane retraction syndrome is a congenital ocular motility disorder characterized by marked limitation of abduction, variable limitation of adduction, and globe retraction with narrowing of the palpebral fissure on adduction. It is the most common type of congenital ocular aberrant innervation with an incidence of 1% to 4% of strabismus patients.[1-2]

Huber2 classified Duane syndrome into 3 types based on the prominent deficiency of duction.Type I is characterized by marked limitation of abduction with normal or minimally defective adduction, type II ischaracterized by marked limitation of adduction with normal or minimally defective abduction, and type III is characterized by marked limitation of both abduction and adduction.

Several retrospective epidemiological studies on clinical, demographic features of Duane syndrome have been published[2-12], however, there is no prospective study including clinical, demographic profile with the follow up of non surgical and surgical cases. The present study was done to analyse the demographic features, clinical diversity, associated ocular and systemic manifestations and therapeutic and surgical intervention in Duane’s retraction syndrome.

Materials and Methods

This prospective, interventional, non randomised study of 102 patients who were diagnosed with DRS at Paediatric ophthalmology and Squint clinic was done from September 2009 to August 2011. After approval of the Ethics Committee, informed consent was taken from all the patients. Patient with history of orbital trauma, previously operated DRS or any muscle surgery, Inverse-Duane’s syndrome were excluded from study. Age group included was 4-67 years. Detailed information regarding age, gender, laterality, family history of strabismus, refractive status and history of strabismus surgery was taken. Visual acuity and refraction, complete orthoptic examination, anterior and posterior segment examination, systemic evaluation was done. Hess charting was done preoperatively where possible. The visual acuity of each patient was assessed by Snellen letters. Sheridan Gardiner test was used for children between 3-5 years of age.

Abnormal head position was measured pre- and postoperatively by using an orthopedic goniometer. All patients were categorized into types I, II, and III DRS, based on Huber’s classification.2 Patients were categorised into groups by unilaterality versus bilaterality and by type of Duane’s syndrome. Strabismus measurements were done by alternate cover prism test in forced primary position. Versions and ductions were documented on a scale of +4 (marked overaction) to 0 (normal) to –4 (marked underaction, cannot get past midline). Globe retraction was graded as Grade 1 retraction defined patients who had an almost imperceptible level of retraction on adduction. Grade 2 retraction consisted of further narrowing of the palpebral fissure on adduction. Grade 3 retraction defined patients who had retraction as in grade 2, plus minimal retraction of the globe away from the lower lid grade 4 severity if there was a clearly visible recess between the globe and the lower lid.[22]

Glasses were prescribed for patients with refractive errors.Patching of the non amblyopic eye was advised in patients below the age of 10 years .Minimum follow up period of non surgical patients was 6 months. Surgery was advised in patients with anomalous head posture of more than 15 degrees, cosmetically unacceptable upshoot or downshoot, ocular deviation in primary position and globe retraction. . Post operative follow up of surgical cases was done at 1 month and were followed up for a minimum period of 6 months .Surgery was done under general or local anaesthesia, depending upon the age of the patient. The procedure performed was determined by the amount of deviation in primary position and the degree of abnormal head position. Forced duction testing was performed intraoperatively. Ipsilateral medial or lateral rectus muscle recession was the surgical procedure of choice. In esotropic patients, medial rectus (MR) recession was done and in exotropic patients lateral rectus (LR) recession was done. In patients with unacceptable overshoot, lateral rectus recession with Y splitting was done (Figure 2). The amount of horizontal muscle to recess depended upon the angle of deviation and on the amount necessary to free the forced ductions. Force duction test was done on table intraoperatively and after disinserting the muscle.

Data analysis

Chi-square Fisher Exact test Wilcoxon signed-rank test was used to find the significance of study parameters on categorical scale between two or more groups. Values of P < 0.05 were considered significant. Statistical analysis was done by STATA 11.0.

Results

Out of the 102 patients 92 (90.2%) had unilateral and 10 (9.8%) had bilateral DRS. Mean age of patients at first visit was 29.37 ± 19.63 years. (Range 4 -67 years). There were 45 males (44.1%) & 57 females (55.9%) out of 102 patients. In 112 eyes(102 patients ) studied, Type I DRS was present in 88 eyes (78.57%), Type II was present in 3 eyes (2.68%), Type III was present in 21 eyes (18.75%). In unilateral DRS, Type I was present in 72 (78.26 %), Type II was present in 2 (2.17%), Type III was present in 18 (19.57%) patients. In bilateral cases, Type I was seen in 16 (80%) eyes, Type II was present in 1 (5%) eyes and Type III was seen in 3 (15%) eyes. Type I (78.57 %) was the commonest while Type II (2.68%) was the least common among unilateral as well as bilateral cases. Type III DRS was present in 18.75% eyes. In 80 % of bilateral cases there was same type of DRS in both eyes. Female preponderance was seen in unilateral (55.6%) as well as bilateral cases (60 %).

Out of 102 patients, 19 (18.6 %) had right eye DRS and 73 (71.6%) had left eye DRS. 10 (9.8%) cases had bilateral DRS. There was no association between gender and the type of DRS in unilateral cases (p=0.529). In unilateral cases, a predilection for the left eye (71.6%) was seen in our study. There was however no statistically significant association between the type of DRS and the eye involved (p value = 0.221). Congenital malformations were found in 12.75% of the study population and family history of strabismus was positive in 15.7% of the patients.

The number of patients with orthophoric DRS was 21 (20.6%), 39 (38.2%) were esotropic and 42 (41.2%) were exotropic out of all the studied patients. Out of 16 orthophoric patients 13 (18.1%) were unilateral DRS Type I, 1 (50%) was type II, 2 (11.1%) were type III. Out of 35 esotropic , 33 (45.8%) were unilateral type I and 2 (11.1%) were type III. Out of 41 exotropic patients, 26 (36.1%) were type I, 1 (50%) was Type II, 14 (77.8 %) were Type III. Association of ocular deviation and Type of DRS was found to be significant (p=0.004, Fisher’s exact test). Out of 10 bilateral cases, 5 (50%) had orthophoria, 4 (40%) had esotropia, and 1 had (10%) had exotropia. Exotropia was more common than esotropia in unilateral cases and esotropia more common than exotropia in bilateral cases.

The overshoots were more common in unilateral type III DRS (83.3%) compared with patients of the other types (p-value < 0.002). Of 67 patients with unilateral DRS with abnormal head posture, 35.29 % of Type I patients showed face turn predominantly to the same side while 85.71% of Type III patients showed face turn on opposite side. There was a highly significant association between Type of DRS and the direction of face turn (p=<0.002). Abnormal head posture was absent in 80 % of the bilateral cases. In unilateral cases retraction was present in 90.3 % of Type I ,100 % of Type II and 18 % of Type III .It was absent in 9.7% of Type I patients In bilateral cases, retraction and narrowing of palpebral fissure was present in all 20 (100%) eyes.

All patients were carefully evaluated for refractive errors and 53 patients were given glasses. Out of 102 patients 51(50%) patients were emmetropic, 25 (24.51%) were myopic and 26 (25.49%) were hypermetropic. Best corrected visual acuity (BCVA) of 6/6-6/9 was present in 95 (84.8%) eyes, 13 (11.6 %) eyes had BCVA in range of 6/12-6/18, 4 (3.6%) eyes had BCVA in range of 6/60 or less. Glasses were given to 40(39.22%) patients, 3 (2.94%) patients who were amblyopic and below 8 years of age, were given glasses and started on occlusion therapy, 10 (9.80%) patients were prescribed glasses and underwent squint surgery, 10 (9.80%) patients underwent only squint surgery, 39 (38.24%) patients were advised observation. Out of 18 patients who had amblyopia 3 were below 8 years age, so occlusion therapy was started in them after 1 month of glass prescription. BCVA improved to 6/9 in 2 cases from 6/12 and 6/18 respectively. The third case had pre occlusion BCVA of 6/60 which improved to 6/36 after 1 year. In unilateral cases strabismic amblyopia was present in 1 (1.4%) Type I, 1 (5.6%) Type III cases. Anisometropic amblyopia was present in 6 (8.3%) Type I cases and 1 (5.6%) Type III cases. Mixed amblyopia was present in 3 (4.2%) Type.In bilateral DRS, out of the 20 eyes, amblyopia was present in 3 (15%) while absent in 17(85 %).

Twenty patients (22 eyes) underwent surgeries, of which 10 patients of esotropia in primary position had medial rectus muscle recession and 10 patients with exotropic had lateral rectus recession. Among patients with esotropia there was a significant improvement (p=0.0368) in the degree of strabismus while a similar significant improvement (p=0.0011), was seen among those with exotropia also (Figure 1a and 1b).

Abnormal head posture > 15° was present in 18 patients out of 20 operated patients. Abnormal head posture was eliminated in 6 patients post operatively. There was significant improvement post operatively (P =0.014, Chi-square test) . Two eyes underwent lateral rectus recession with Y-split and both of them showed elimination of downshoot postoperatively. (Figure 3) There was significant improvement in retraction after appropriate post surgical intervention (p=0.005) and retraction was eliminated in 13 eyes.





Discussion

There are various reports in which some clinical features of Duane syndrome have been analyzed separately in unilateral and bilateral cases2-12 (Table 1). In our study out of 102 patients of DRS, 92 (90.2%) had unilateral and 10 (9.8%) had bilateral DRS. Similarly, Duane[1] in his study of 54 patients and Raab[5] in his study of 70 patients found 90 % of the cases were unilateral and 10 % were bilateral . In our study, Type I was found in 78.57% , Type II in 2.68 %, Type III in 18.75 % eyes. Mohan et al[10] and Kekunnaya et al[11] also reported Type I to be common in unilateral cases and type II to be least common in 331 and 441 patients studied respectively. But O’Malley et al[7] in their study of 97 eyes had found Type III DRS least common with 2 eyes presenting with it while Type II DRS was present in 11 eyes.

Our study showed female preponderance in both unilateral and bilateral cases. Isenberg and Urist4 reported a similar figure quoting 57 % females. Most studies have reported a female preponderance[1-5], except Tredici and von noorden11 study which had 60 % male out of 70 DRS patients studied. Mohan et al10 found that Type II DRS had no sex predilection while Type I and III had more females. Family history of strabismus was positive in 15.7% of the patients. Raab[5] reported 30% out of 64 patients. Ahuwalia et al[3] had 25% myopic patients which was similar to our study which showed 24.51% myopes. Congenital malformations were found in 12.75% of our patients. Several authors have reported 7% to 33% incidence of associated congenital malformations.1,11 Overshoot was present in 49.1 % eyes in our study which was similar to the incidence of 45% found by Isenberg and Urist.[4] Kekunnaya et al[11] also reported overshoot to be more common in types I (45%) and III (80%) compared to type II (20%). Mohan et al10 reported 76 % Type III and 75% Type II showing overshoot while only 31% in Type I presented with it. In present study mean angle of deviation was 43.91 prism diopters for all esotropic cases which reduced to 21.27 prism diopter (PD) after MR recession (p=0.0032). Pressman and Scott8 did medial rectus recession in 7 patients who had esotropia. They reported mean angle of deviation preoperatively 26.28 prism diopter which reduced to 2.71 prism diopter. In exotropic patients, the mean angle of deviation was 33PD, which reduced to 10.54 after lateral rectus recession in our study (p=0.0033). Horizontal muscle recession was proposed by Duane1 in 1905 and has been used extensively with success. Kraft SP19, von Noorden GK20 have also supported the effectiveness of appropriate muscle recession in treatment of DRS. There was a significant improvement of AHP post operation (p=0.014) in our study. Pressman and Scott7 reported 79% elimination of AHP and 100% significant improvement of AHP in 19 DRS patients who underwent appropriate horizontal muscle recession. Barbe et al15 reported 93% achievement of a postoperative alignment of =15° AHP and 66% showed = 5° AHP out of the 59 patients who were treated with either unilateral or bilateral medial or lateral rectus recession. Rao et al[6] reported 10 patients who underwent lateral rectus recession with Y-split. This procedure led to a dramatic improvement in upshoot or downshoot which was also seen in our study. When combined with simultaneous recession of the medial rectus muscle, it improves globe retraction and corrects ocular deviation. Das et al[18] also reported significant decrease in upshoot and downshoot and globe rotation with Y-splitting of the lateral rectus muscle. There was a significant improvement in retraction post operation (p value=0.005) in our study. Pressman and Scott8 also reported[19] patients in whom appropriate horizontal muscle recession eliminated globe retraction. Our study has some important limitations that follow up was of short period and number of surgeries done was less so long term follow up and study of large surgical series is warrented. The observations from our study indicate that unilateral types I, II, and III Duane’s retraction syndrome differ in their epidemiologic and clinical characteristics.Bilateral Duane’s retraction syndrome differs from the unilateral mainly in the frequency and type of primaryposition horizontal deviation,abnormal head posture and upshoot and downshoot. Appropriate ipsilateral muscle recession in treatment of DRS gives promising results in treating primary position deviation, abnormal head posture and retraction. Unilateral types I, II, and III Duane syndrome differ from bilateral cases in the primary position horizontal deviation, upshoot and downshoot, and associated ocular abnormalities. Surgical intervention is recommended in patients with abnormal head posture, overshoot, globe retraction and significant deviation in primary position.







Financial & competing interest disclosure

The authors do not have any competing interests in any product/procedure mentioned in this study. The authors do not have any financial interests in any product / procedure mentioned in this study

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Ravindran M, Pawar N, Karkera RP, Kunnatur RClinical Analysis, Management and Outcome of Duane’s Retraction Syndrome at a Tertiary Eye Care Centre in South India.DJO 2013;24:97-101

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Ravindran M, Pawar N, Karkera RP, Kunnatur RClinical Analysis, Management and Outcome of Duane’s Retraction Syndrome at a Tertiary Eye Care Centre in South India.DJO [serial online] 2013[cited 2020 Sep 30];24:97-101. Available from: http://www.djo.org.in/articles/24/2/clinical-analysis-management-and.html