Clinical Profile and Surgical Outcomes of Bilateral Duane Syndrome with Exotropia

Distinctive clinical features of bilateral Duane retraction syndrome

Adjustment of the surgical nomogram for surgery on slipped extraocular muscles

Clinical Characteristics of Bilateral Duane Syndrome

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.

Dear Sir, We read with interest the article by Sharma et al.[1] Since Duane's retraction syndrome (DRS) is not garden variety strabismus, certain observations are in order. DRS can only be alleviated, not eliminated. In exo DRS, abnormal with subnormal lateral rectus (LR) along with occasionally subnormal medial rectus (MR) innervation may occur. MR may be stretched out/elongated. Globe retraction and shoots may disappear in large Exo DRS, and both horizontals may be stiff in relatively immobile globes with marked limitation of adduction and abduction. Muscle function tests are core to DRS evaluation but find fleeting reference here. Needless to say that each case is unique and graded procedures are in order rather than un-titrated ones, as has happened here. Control group is lacking, inferences like efficacy may not stand scrutiny of statistical analysis. Cohort is too diverse as both unilateral/bilateral cases have been included. Fixating eye in unilateral cases and whether bilateral cases are fusing or nonfusing DRS is not known. Fixation duress and deviation with either eye fixing are not known. Deviations in forced primary, abnormal head posture (AHP) and shoots are not quantified, but inferences are drawn. Type II DRS has only adduction limitation with normal abduction, authors are likely referring to type III DRS. Primary concerns in DRS are AHP, primary position (PP) deviation, globe retraction/narrowing of the palpebral fissure in adduction, shoots, A/V patterns, fixation duress, and decentration of binocular visual fields. Improvement in ductions/binocular visual fields is secondary as patients often suppress and do not complain of diplopia in side gazes. Lateral rectus periosteal fixation converts DRS into total 6th nerve palsy with attendant muscle sequaele. For aforesaid reasons, 33% patients (2 out of 6) developed an esotropia with rest having residual XT of 6 PD or greater. Authors have to throw some light as to how abduction improved in Group 1. In fact, it should worsen which is also evident in Fig. 1. In Group 2, most cases had undercorrected exotropia in PP, implying that ½ muscle vertical rectus transposition (VRT) adds to PP exotropia and may not have been indicated. Relearning of muscle function does not happen after transposition surgery as 3rd nerve nucleus is in the midbrain and 6th nerve nucleus in the pons, postulated innervational plasticity cannot occur.[2] Following VRT in LR palsy, vertical rectus muscles do not recruit in abduction and change in direction of vector forces was minimal.[34] Figure 2 does not demonstrate improved abduction. Abducting force is generated in PP and improvement in abduction could only be due to relaxation of MR. VRT has been used in eso DRS with minimal abnormal LR innervation.[5] The claims of better abduction in Group 2 as compared to Group 1 are not supported by tests of statistical significance. Adduction improvement was not full in both groups implying that part of the problem lay with MR, which cannot be operated now, and surgery on the other eye is the only option. Under the circumstances, graded options other than periosteal fixation seem to be better.

To evaluate the clinical findings of different types of Duane retraction syndrome (DRS). Retrospective. This study was performed on 691 patients with DRS who underwent surgery. Clinical examinations included laterality, abnormal head posture (AHP), corrected distance visual acuity (CDVA), refractive error, amblyopia, deviation, overshoots, and type of surgery. The mean age of patients with DRS was 16.7 ± 12.5 (range 1.0-73) years. The patients included 396 (57.3%) women and 295 (42.7%) men (P < 0.001). DRS type I, was observed in 429 (62.1%), II in 168 (24.3%), III in 88 (12.7%) and IV in 6 (0.9%) patients. Unilateral DRS was observed OS in 628 (90.9%) [471 (%78.9) and OD in 157 (21.1%) eyes (P < 0.001)]. O ther clinical findings were AHP (n = 522, 78.1%), overshoot (n = 236, 34.2%) and amblyopia (n = 118, 17.1%). The prevalence of overshoot in types I, II, and III was 17.5% (75/429), 60.7% (102/168) and 64.8% (57/88), respectively (P < 0.001). The prevalence of amblyopia was significantly lower in patients with AHP (80/522, 15.3%) compared to patients with normal head posture (38/169, 22.5%) (P = 0.023). The mean angle of deviation in the primary position (PP) at distance was 21.7 ± 11.5 △ for esotropic group and 17.8 ± 12.4 △ for exotropic group. Sixty-two (9.0%) patients required second surgery for resolving residual misalignment (1.1 surgeries for each patient). About two-thirds of DRS patients had AHP, one-third had overshoots, and one-sixth had amblyopia. The results show that different types of DRS are associated with different epidemiological and clinical characteristics.

A case of bilateral Duane's retraction syndrome (DRS) type III associated with Kallmann's syndrome (hypogonadotropic hypogonadism and anosmia) is reported. Both anomalies may result from a disturbance of embryogenesis during the second month of gestation. The authors associate bilateral type III DRS with congenital horizontal gaze paralysis.

Purpose To report the clinical presentation and surgical outcomes of patients with Duane syndrome with primary position hypertropia, with or without coexisting horizontal deviation. Methods We retrospectively reviewed records of patients diagnosed with Duane syndrome with vertical and associated horizontal deviations from January 2008 to July 2017. We collected data regarding patient age, gender, refractive error, presence or absence of amblyopia and history of strabismus surgery, as well as clinical subtype of Duane syndrome, abnormal head posture, measurement of horizontal and vertical deviation, and outcomes of strabismus surgery. Success was defined as post-operative primary position hypertropia <4 prism diopters (PD) and horizontal deviation <10 PD. Results During this period, a total of 590 patients with Duane syndrome were seen. Of these, 18 patients (10 males and 8 females) met the study criteria, giving a prevalence of 3%. Median age was 21 years. Seventy-eight percent cases had type III Duane syndrome. Ten patients underwent surgical correction, of which four patients had to undergo a second procedure after a median of 19 months. Sixty-seven percent of the patients had associated primary position exotropia with median deviation of 30 PD. Seventeen percent of patients had only primary position hypertropia with median 10 PD. All patients had overshoots. Primary position horizontal deviation improved from 25 PD to orthotropia, and vertical deviation improved from 10 PD to orthotropia. Lateral rectus recession with Y split was the most frequent procedure performed, and we saw a median reduction of 10 PD hypertropia with this procedure. Median follow-up period was 13 months. Success was achieved in 60% of the patients who underwent surgery. Given the small sample size, it was difficult to compare the efficacy of different procedures. Conclusion Current study suggests that primary position vertical deviation might exist in 3% of patients with Duane syndrome. All patients had associated overshoots, which were predominantly of a mixed mechanism. Forty percent of the patients needed re-surgery. Well-designed, prospective possibly multicentric studies are necessary to understand the mechanism of overshoot, and consequently the primary position hypertropia to plan accurate management for these patients.

De levenswijze van konijnen in het wild wordt kort beschreven. Een omschrijving wordt gegeven van een Zwitsers groepshuisvestingssyteem. In de groepshuisvesting blijken de konijnen geen gedragsafwijkingen te vertonen. Gekonkludeerd wordt dat kennis van de natuurlijke levenswijze een belangrijke rol zou moeten spelen bij het houden van konijnen. The natural way of living of wild rabbits is described. A description is given of a Swiss groupkeeping system. In this keeping system the rabbits do not show behavioural deviations. It is concluded that knowledge of the natural way of living is important for the keeping of rabbits

AimsTo evaluate the outcomes following surgery on patients with bilateral Duane's Retraction Syndrome (DRS) associated with exotropia or exophoria.MethodsEleven consecutive patients with exotropic DRS were identified in a 10-year period....

The rare association of two syndromes of paradoxical innervation, bilateral Duane's retraction syndrome and congenital crocodile tears is presented. This association assumes a central localization to explain both anomalies.

To report the postoperative results of full-tendon vertical rectus transposition (VRT) augmented with lateral fixation suture for the treatment of type 1 Duane syndrome and sixth nerve palsy and to determine whether there was a decrease in the effect of the Foster suture over time. This retrospective, consecutive case series included patients who underwent a full-tendon VRT transposition with lateral fixation for type 1 Duane syndrome or sixth nerve palsy. The primary outcome measures included deviation, abnormal head posture(AHP), abduction deficiency, and postoperative binocular single visual field (BSVF). Eighty-seven patients (87 eyes: 40 eyes with Duane syndrome and 47 eyes with sixth nerve palsy) were included in this study. In Duane syndrome patients, the deviation was reduced by a mean of 95%, the AHP was eliminated in 86% of patients, the abduction was improved by 42%, and a useful BSVF of ≈ 67% of normal was achieved at 1 year post operation. In sixth nerve palsy patients, the deviation was reduced by 99%, the abduction was improved by 59%, and a useful BSVF of ≈ 71% of normal was achieved at 1 year post operation. In both groups, the improvements in deviation angle and abduction were stable postoperatively. Sixteen patients needed reoperation for undercorrection. VRT surgery with posterior fixation is an effective treatment method for complete sixth nerve palsy and Duane syndrome with esotropia, AHP, and abduction deficiency. The procedure carries a small risk of reoperation for undercorrection. The effect of the Foster suture did not decline over time.

To determine the prevalence and clinical characteristics of patients with Duane retraction syndrome presenting with exotropia in a tertiary eye care center. Medical records of 140 patients with Duane retraction syndrome and exotropia (of the total 441 patients with Duane retraction syndrome) seen from 2000 to 2009 were reviewed. Characteristics studied included age at presentation, gender, laterality, manifest primary position, abnormal head posture, ocular motility, and overshoots. Data were collected regarding associated ocular or systemic abnormalities and amblyopia. The mean age of the patients at presentation was 16.96 ± 15.09 years (range: 1 to 70 years). Of the 140 patients with Duane retraction syndrome and exotropia, 59 (42%) were male. Unilateral type I Duane retraction syndrome accounted for 118 (84.2%), type II for 7 (5%), and type III for 10 (7.1%) patients, whereas 5 (3.6%) had Duane retraction syndrome and bilateral exotropia. Duane retraction syndrome was seen in the left eye, right eye, and both eyes in 97 (69%), 37 (26%), and 6 (4%) patients, respectively. Exotropia ranged from 10 to 60 prism diopters. An abnormal head posture was noted in 101 (72%) and upshoot in 26 (18.6%) patients. Reported ocular and systemic abnormalities in these patients included cataract (6), aniridia (1), nasolacrimal duct block (1), and retinal breaks (1). This is an exclusive study on a large series of patients with Duane retraction syndrome and exotropia. Almost one-third of patients with Duane retraction syndrome patients might have exotropia. Although patients with Duane retraction syndrome and exotropia had clinical characteristics and associations similar to those with Duane retraction syndrome and esotropia, there was an increased propensity for overshoots and face turn opposite to the affected eye, which the authors hypothesize to be due to a tight or short lateral rectus muscle. [J Pediatr Ophthalmol Strabismus. 2017;54(2):117-122.].

Vertical Rectus Muscle Transposition for Bilateral Duane Syndrome

To evaluate the efficacy of symmetric bilateral medial rectus recession in large-angle esotropic Duane retraction syndrome (DRS) with moderate to severe globe retraction. In a retrospective study, medical reports of 30 patients with esotropia of equal or more than 20 prism diopters (pd) and moderate to serve globe retraction due to unilateral DRS who underwent symmetric bilateral medial rectus recession were reviewed. Age, gender, laterality, amblyopia, length of follow-up, pre- and postoperative measurements of primary position deviation, ocular ductions and severity of globe retraction and abnormal head posture were evaluated. A successful result was defined as decreasing esotropia to equal or less than 8 pd or equal or less than 8 pd of consecutive exotropia. The mean age of patients at surgery was 13.7 ± 8.5 years old (range: 3-38). The mean preoperative esotropia measured 28.9 ± 9.1 pd in distance and 25.7 ± 7.2 pd in near, which decreased to 4.9 ± 6.1 pd in distance and 3.9 ± 8.8 pd in near postoperatively. The mean bilateral medial rectus recession was 4.9 ± 0.9 mm (range: 3-6 mm). The mean abnormal head posture improved from 19.1 ± 6.9 degrees (range: 10-30 degrees) to 3.3 ± 4.7 degrees (range: 0-15 degrees) postoperatively. At the last follow-up visit, 23 patients (76.7%) had a successful outcome. In the large-angle esotropic DRS patients with moderate to severe globe retraction, symmetric bilateral medial rectus recession, can be conducted to successfully resolve primary position deviation and abnormal head posture.