Research
Original Investigation
Superior Rectus Transposition vs Medial Rectus Recession for Treatment of Esotropic Duane Syndrome Shiqiang Yang, MD; Sarah MacKinnon, MSc, OC(C); Linda R. Dagi, MD; David G. Hunter, MD, PhD
IMPORTANCE Superior rectus transposition (SRT) with or without medial rectus recession
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(MRc) has been introduced as an alternative to MRc alone for treatment of esotropic Duane syndrome; however, the effectiveness of these procedures has not been compared previously. OBJECTIVE To compare the safety and efficacy of MRc and SRT in treatment of Duane
syndrome. DESIGN, SETTING, AND PARTICIPANTS Retrospective medical record review of all patients with esotropic Duane syndrome who underwent surgical treatment from January 1, 2006, through December 31, 2012, in a multispecialty, hospital-based pediatric ophthalmology/adult strabismus practice at Boston Children’s Hospital. Patients in the SRT group underwent SRT with or without MRc; those in the non-SRT group underwent unilateral or bilateral MRc. EXPOSURES Surgical treatment of esotropic Duane syndrome. MAIN OUTCOMES AND MEASURES Binocular alignment, ocular ductions, head position, stereopsis, and fundus torsion were recorded before surgery and at the 2-month and final postoperative visits. We also evaluated postoperative drift. RESULTS The medical record review identified 36 patients who underwent 37 procedures, including 19 in the SRT group (13 SRT + MRc and 6 SRT alone) and 18 in the non-SRT group (11 unilateral MRc and 7 bilateral medial rectus resession). Mean MRc was smaller when performed with SRT (3.3 vs 5.3 mm; P = .004). Although the initial deviation was larger in the SRT group, both groups had a similar improvement in esotropia and head turn. Abduction improved by at least 1 unit in 15 of 19 patients in the SRT group (79%) vs 5 of 18 in the non-SRT group (28%). In 24 patients followed up for more than 6 months, mean esotropia decreased from 8.2 to 6.1 prism diopters (Δ) in the SRT group (n = 12) but increased from 7.2 to 10.9Δ in the non-SRT group (n = 12). CONCLUSIONS AND RELEVANCE The combination of SRT and MRc was more effective than MRc or bilateral medial rectus resession at improving abduction while allowing for a smaller recession to align the eyes and eliminate a compensatory head posture. Although any surgery on the vertical rectus muscles should in theory increase the risk for vertical or torsional complications, to date this theory has not been borne out in our patients. Patients treated with SRT appear to have a reduced likelihood of long-term undercorrection. We therefore recommend SRT with adjustable MRc for treatment of Duane syndrome in patients with larger amounts of esotropia.
JAMA Ophthalmol. 2014;132(6):669-675. doi:10.1001/jamaophthalmol.2014.358 Published online April 24, 2014.
Author Affiliations: Department of Ophthalmology, Boston Children’s Hospital, Boston, Massachusetts (Yang, MacKinnon, Dagi, Hunter); now with Department of Strabismus and Pediatric Ophthalmology, Tianjin Eye Hospital, Tianjin Eye Institute, Clinical College of Ophthalmology, Tianjin, China (Yang); Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts (Dagi, Hunter). Corresponding Author: David G. Hunter, MD, PhD, Department of Ophthalmology, Boston Children’s Hospital, 300 Longwood Ave, Fegan 4, Boston, MA 02115 (david.hunter @childrens.harvard.edu).
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Research Original Investigation
Surgical Treatment of Esotropic Duane Syndrome
D
uane syndrome is a congenital cranial dysinnervation disorder characterized by limitation of horizontal eye movement(s) with aberrant innervation causing globe retraction in adduction. Most patients have esotropia in primary gaze, limited abduction, and a head turn toward the involved side (esotropic type 1 Duane syndrome). Various surgical approaches have been proposed to improve binocular alignment and reduce head turn in Duane syndrome. Unilateral or bilateral medial rectus recessions (MRc or BMR, respectively) are common procedures and are usually satisfactory for correcting alignment in primary gaze and reducing torticollis1-4; however, MRc does not improve the abduction limitation.5 Indeed, a large MRc limits adduction,6,7 which in turn may cause difficulty with convergence and in contralateral gaze. In an effort to improve abduction without limiting adduction in patients with Duane syndrome, a variety of transposition procedures have been developed, including augmented transposition of both vertical rectus muscles (VRT). The superior rectus transposition (SRT) was developed subsequently to reduce the number of muscles requiring surgery while decreasing the likelihood of inducing a vertical deviation observed with VRT.8 A recently published experience combining SRT with an adjustable MRc in patients with esotropic Duane syndrome9 found results comparable to those of VRT but with a lower incidence of induced hypertropia. However, whether the potential benefits of SRT or similar transposition procedures warrant the added complexity and potential risk for vertical deviation associated with the procedure remains unclear. The purpose of this study is to address this question by comparing the results of SRT with those of MRc in terms of postoperative alignment, head turn, and ocular ductions and long-term stability of alignment.
Methods This retrospective study was approved by the institutional review board of Boston Children’s Hospital. We used billing records to identify patients treated for Duane syndrome in the Department of Ophthalmology from January 1, 2006, through December 31, 2012. We included patients with esotropic Duane syndrome who were treated with SRT or MRc. Patients were divided into the SRT group, consisting of patients who had SRT alone or in combination with MRc (SRT + MRc), and the nonSRT group, consisting of patients who had unilateral MRc or BMR. Patients were excluded if follow-up was less than 1 month or if VRT surgery had been performed previously. In cases in which patients underwent MRc followed more than 2 years later by SRT, the 2 procedures were analyzed separately: the first in the non-SRT group, the second in the SRT group. Superior rectus transposition was performed as described previously.9-11 Briefly, the superior rectus muscle was isolated, with care taken to clear the muscle of attachments to overlying levator and underlying superior oblique tendon. The muscle was secured, detached, and then reattached to the eye along the spiral of Tillaux, spanning the gap between the original lateral and superior rectus muscle insertions. A loop myopexy was then placed between the lateral and superior rec670
tus muscles 8 to 10 mm posterior to the insertion, incorporating approximately one-quarter of the muscle width, in most cases without a scleral pass. Medial rectus recession was performed per the preference and surgical tables of the individual surgeon. Sensorimotor examination data were extracted from the preoperative evaluation, 2 months after surgical intervention, and at the final follow-up examination. Only patients with follow-up longer than 6 months were included for analysis of long-term stability (postoperative drift). At each point, we recorded the angle of esotropia in near and distance straightahead gaze, head turn in degrees, limitation of abduction (including limitation in elevation and depression), limitation of adduction, stereopsis (by Titmus fly and Randot circles), hypotropia in straight-ahead gaze and elevation, and torsion (by the double-Maddox rod test and/or indirect ophthalmoscopy as available in the record). Alignment was recorded from the orthoptic evaluation of distance deviation. Ductions were graded on a scale from 0 (indicating full duction) through −4 (indicating an eye that was able to move to the midline) and −5 (indicating an eye that approached but was unable to reach the midline) to a maximum of −8 (indicating a rare case in which the eye was nearly fixed in an extremely adducted position). Head turn was measured with a goniometer at distance fixation or using clinical photographs. In SRT cases performed toward the end of the study period, we monitored intraoperative torsional changes as described by Holmes et al12 (Supplement [eFigure 1]). Statistical calculations were performed using commercially available software (Prism 5.0; GraphPad Software, Inc [http://www.graphpad.com]). We used 2-way analysis of variance to compare the preoperative and postoperative results of the SRT and non-SRT groups and 1-way analysis of variance to analyze the change in deviation angle and head turn of the groups undergoing SRT + MRc, SRT alone, MRc alone, and BMR. If a patient in the SRT group had undergone previous MRc, the amount of MRc was included in the calculation of MRc dosage. Titmus stereopsis of greater than 2 lines was considered clinically meaningful. We used Bonferroni post hoc tests for multiple comparisons and an unpaired t test for comparisons where appropriate. P < .05 was considered statistically significant.
Results The medical record review identified 37 patients with esotropic Duane syndrome who underwent 38 procedures. One procedure was excluded for lack of adequate follow-up, leaving 36 patients who underwent 37 procedures by 9 surgeons, with 20 of the 37 performed by two of us (L.R.D. and D.G.H.), including 13 of 19 SRT procedures. The SRT group included 13 cases of SRT + MRc and 6 cases of SRT alone. Six of 13 SRT + MRc cases underwent muscle-to-muscle augmentation (superior to lateral rectus muscle) without scleral fixation, and 7 underwent muscle-to-sclera augmentation (when the lateral rectus muscle was recessed or lax). The non-SRT group included 11 cases of MRc alone and 7 cases of BMR.
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Among the 6 cases of SRT without MRc, 2 had undergone an MRc procedure more than 2 years previously (for which the MRc amount was included in the calculation of MRc performed); in 2 additional cases, the MRc was reduced to 0 at the time of postoperative suture adjustment. One case of SRT alone was combined with an ipsilateral inferior oblique recession to treat hypertropia in adduction.
Characteristics of patients in the SRT and non-SRT groups are summarized and compared in the Table. The amount of MRc performed (Supplement [eFigure 2]) was significantly larger in the non-SRT group (mean [SD], 5.3 [1.3] mm) than in the SRT group (3.3 [2.3] mm) (P = .004). The mean postoperative follow-up was 14.6 months for the SRT group and 32.0 months for the non-SRT group. Although the initial deviation was greater in the SRT group and both procedures significantly decreased the angle of esotropia, the SRT + MRc procedure appeared to be most effective (Figure 1A-C). Superior rectus transposition alone was comparable in effect to MRc alone and BMR. The SRT and non-SRT groups showed notable improvements in head turn (Figure 1D-F). As with angle of deviation, the SRT + MRc group appeared to show the greatest efficacy, but the difference in head position was not statistically significant. Stereopsis improved in both groups (Table), with 3 patients in each group improving by 3 or more lines on the Titmus test. No patient had a reduction in stereopsis. Preoperative and postoperative limitation of adduction and abduction were available for all procedures. Abduction significantly improved in the SRT group but not in the non-SRT group (Figure 2A and B). We found a small but significant increase in adduction limitation in the SRT and
Table. Patient Characteristics and Stereopsis Groupa Characteristic
SRT
Age, mean (SD), y
Non-SRT
10.3 (12.9)
Male
13.6 (14.1)
11/19 (58)
Follow-up, mean (SD) [range], mo
6/18 (33)
14.6 (16.2) [1-63]
Left eye
32.0 (31.0) [1-99]
16/19 (84)
15/18 (83)
4
1
Preoperative
10/14 (71)
11/14 (79)
Postoperative
14/16 (88)
16/18 (89)
Severe globe retraction or up/down shoot Stereopsis ≥3000 arc seconds
Abbreviation: SRT, superior rectus transposition. a
Unless otherwise indicated, data are expressed as number of cases/number in group for whom data were available (percentage).
Figure 1. Comparison of Surgical Results of Deviation Angle and Head Turn Preoperative
30
b
20 10 0 SRT
c 60 40 c 20 0 –20
Non-SRT
SRT E
30
a a
20
10
0 SRT
Non-SRT
80 c 60 40 c 20 0 –20
Non-SRT
SRT + MRc
SRT Alone
MRc Alone
BMR
SRT + MRc
SRT Alone
MRc Alone
BMR
F
Change of Head Turn, Degrees
D
Head Turn, Degrees
80
Change of Angle in Primary Gaze Position, Δ
a
Non-SRT subgroups
C
Change of Head Turn, Degrees
Angle of Primary Gaze, Δ
40
SRT subgroups B
Change of Angle in Primary Gaze Position, Δ
Final follow-up
A
50 40 30 20 10 0 SRT
Non-SRT
A, Deviation angle before surgery and at final follow-up in the superior rectus transposition (SRT) and non-SRT surgical groups. Positive values represent esotropia; error bars, SEM. B, Box-and-whiskers plot of postoperative change of the deviation angle in both surgical groups. C, Box-and-whiskers plot of postoperative change of deviation angle in each surgical subgroup. D, Head turn before and after surgery in the SRT and non-SRT groups. Positive values represent improvement in head turn (in degrees); error bars, SEM. E, Box-and-whiskers plot of postoperative change in head turn in both surgical groups. F, Box-and-whiskers plot of postoperative change of head turn in each surgical subgroup. Unless otherwise indicated, positive values represent the
50 40 30 20 10 0
difference between preoperative and final values. In the box-and-whiskers plots, the bottom and top of each box represent the 25th and 75th percentiles (the lower and upper quartiles, respectively); the band near the middle of the box, the 50th percentile (the median). The ends of the whiskers represent the minimum and maximum of all the data. BMR indicates bilateral medial rectus resession; MRc, medial rectus recession; and Δ, prism diopter. a P < .001. b P < .05. c P < .01.
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Figure 2. Comparison of Limitation of Abduction and Adduction Before and After Surgery Preoperative Final follow-up A
B
Improvement of Abduction Limitation
Limitation of Abduction
0 –1 –2 –3 –4 –5
a SRT
2 1 0 –1 SRT
Non-SRT
D
0.0
–0.5
–1.0 b
a
SRT
Non-SRT
–1.5
Change of Adduction Limitation
0
Limitation of Adduction
3
Non-SRT
C
a
4
–0.5 –1.0 –1.5 –2.0 –2.5 SRT + MRc
SRT Alone
MRc Alone
BMR
Duction grades range from 0 (full duction) through −4 (eye was able to move to the midline) and −5 (eye approached but was unable to reach the midline). A, Limitation of abduction preoperatively and at final follow-up in the superior rectus transposition (SRT) and non-SRT surgical groups. B, Postoperative improvement of abduction in each surgical subgroup; clinically significant improvement of at least 1.0 units was observed in 15 of 19 SRT patients (79%) and 5 of 18 non-SRT patients (28%). C, Limitation of adduction preoperatively and at final follow-up in the SRT and non-SRT surgical groups. D, Postoperative improvement of adduction in each surgical subgroup. BMR indicates bilateral medial rectus resession; MRc, medial rectus recession; error bars, SEM; markers, individual patients; and bands near the middle, the 50th percentile. a P < .001. b P < .01.
non-SRT groups (Figure 2C and D). Two patients from each group developed postoperative adduction limitation of more than −1. In 15 patients in the SRT group, abduction was measured not only in the horizontal meridian but also in elevation and depression (Figure 3). In some cases, preoperative abduction was slightly better in elevation (Figure 3B) or in depression (Figure 3C). After surgery, we found a trend toward more improvement in abduction in elevation than in depression (Figure 3D and Supplement [eFigure 3]). No patient in either group described torsional diplopia postoperatively. Of 9 patients in whom torsion was measured before and after surgery (all but 1 in the SRT group), 8 showed no change postoperatively. A +1 (asymptomatic) increase in intorsion was observed in 1 SRT patient. Intraoperative monitoring of torsion performed in 3 patients in the SRT group showed no change at the end of the procedure. Small, asymptomatic vertical deviations in adduction were present in 3 of 18 patients in the SRT group and 3 of 19 in the MRc group. No patient developed a symptomatic vertical strabismus after surgery; however, 2 SRT patients had a 672
new postoperative hypotropia or hypophoria. An 18-yearold girl developed hypotropia of 4 prism diopters (Δ) in straight-ahead gaze without diplopia; at the final postoperative visit, she had 20 seconds of stereopsis with a 5° residual head turn. A 5-year-old girl underwent a 6-mm ipsilateral MRc and developed a hypophoria after a subsequent SRT. Three years later, she had a residual esotropia of 8Δ and a persistent left hypophoria of 10Δ but was asymptomatic with good fusion and 70 arc seconds of stereopsis with a 5° residual left head turn. Measurements of long-term drift in postoperative alignment were available for evaluation in 24 patients (12 in the SRT group [mean follow-up, 22 months] and 12 in the non-SRT group [47 months]). Although we found a trend toward further increase in surgical effect in the SRT group (mean esotropia decreased from 8.2Δ to 6.1Δ), the trend was toward a loss of effect in the non-SRT group (mean esotropia increased from 7.2Δ to 10.9Δ) (Figure 4A). These trends were not statistically significant (P = .33). A similar trend was observed in the improvement in head position (Figure 4B), with an improvement over time in the SRT group (decrease from 5.0° to 2.5°) compared with a recurrence over time in the non-SRT group (increase from 3.3° to 5.5°). Again, these observed trends were not statistically significant (P = .14). No significant long-term changes in limitation of abduction (Figure 4C) or adduction (Figure 4D) were observed.
Discussion Debate is ongoing about the best approach for treatment of esotropic Duane syndrome and other causes of abduction limitation. Recession of the ipsilateral medial rectus muscle is an attractive option for its simplicity and predictability, and indeed this approach will improve the deviation and head turn in straight-ahead gaze.1,3-5 Unfortunately, to correct larger deviations and head turns, a large MRc is required, which often induces limitation of adduction w ithout improv ing abduction.6,7 Bilateral MRc is used for larger angles. Recessions of the contralateral medial rectus muscle are sometimes used, but the approach remains controversial,2,13-15 with a paradoxical increase in head turn described in some cases.16,17 Transposition procedures have been proposed as a means of increasing lateral force to the eye to compensate for the lack of lateral rectus muscle function. At present, full, augmented VRT18 is a widely used approach.19-21 Rosenbaum22 showed a marked improvement of abduction and in the field of binocular single vision in patients with Duane syndrome who undergo VRT. Concerns about induced hypotropia have limited widespread adoption of VRT.23 In addition, when the medial rectus muscle is tight, concerns about anterior segment ischemia limit the option of MRc. We adopted the SRT in 2006 after Johnston and colleagues8 first presented results in the treatment of Duane syndrome and sixth nerve palsy. Mehendale and colleagues9 reported successful treatment of patients with complete loss of lateral rectus function. Morad and Kekunnaya24 have also reported good results of SRT + MRc for Duane syndrome.
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Figure 3. Changes in Abduction in Elevation vs Depression A
B
C
Elevation Midline Depression Neither 0
2
4
6
8
10
Maximal Preoperative Abduction D
Elevation Midline Depression Neither 0
2
4
6
Improvement in Abduction
A, Preoperative images of a 6-year-old boy with Duane syndrome of the right eye. Improved abduction of the right eye is observed in elevation (top panel) compared with vertical midline or depression. B, Preoperative images of a 2-year-old girl with Duane syndrome of the left eye. Improved abduction of the
left eye is observed in depression. C, Maximal abduction in the affected eye in elevation and depression before surgery. D, Maximal improvement of abduction in elevation and depression.
Figure 4. Postoperative Drift of Surgical Results of Deviation, Head Turn, and Ductions in the Superior Rectus Transposition (SRT) and Non-SRT Surgical Groups A
B
2 mo
>6 mo
8
Postoperative Head Turn, Degrees
Postoperative Deviation in Primary Gaze, Δ
15
10
5
0
6 4 2 0
SRT
Non-SRT
C
SRT
Non-SRT
SRT
Non-SRT
D
0.0
–1 –2 –3
a a
–4
Postoperative Limitation of Adduction
Postoperative Limitation of Abduction
0
–0.5
–1.0
–1.5 SRT
Non-SRT
A, Postoperative drift of deviation (given in prism diopters [Δ]). Positive values represent the angle of deviation in straight-ahead gaze. B, Postoperative drift of head turn. Positive values represent the angle of head turn. C, Postoperative drift of limitation of abduction. D, Postoperative drift of limitation of adduction. Duction grades range from 0 (full duction) through −4 (eye was able to move to
the midline) and −5 (eye approached but was unable to reach the midline). Negative values represent the limitation of abduction and adduction. Error bars represent SEM. a P < .05.
In our multiphysician strabismus practice, patients with esotropic Duane syndrome were treated with SRT and nonSRT procedures according to surgeon preference, and all patients were examined by the same orthoptic team according
to the same documentation guidelines. More patients undergoing non-SRT procedures had surgery earlier in the study period, which resulted in a longer overall follow-up for the nonSRT group. Although the present study was not a randomized
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prospective trial and is subject to bias of patient selection, incomplete documentation, and an unequal follow-up, the available data allowed us to perform, to our knowledge, the first comparative study of the sensorimotor impact of SRT + MRc in patients with esotropic Duane syndrome. Abduction improved significantly in patients treated with SRT compared with MRc alone. We found a comparable reduction of esotropia in straight-ahead gaze, but the SRT group had a larger preoperative esotropia than the non-SRT group; thus, surgeons might have selected SRT for patients with a larger preoperative angle. The improvement in abduction with SRT (15 of 19 patients [79%]) was similar to the results reported previously in a smaller group of patients.9 The improvement in abduction with MRc (5 of 18 patients [28%]) was similar to the 29% improvement reported by Merino et al4 (although they graded abduction limitation only on a scale of −1 through −3). By combining SRT with MRc, a significantly smaller MRc was required to treat the esotropia. We also found a trend toward better preservation of adduction in the SRT group, which matches our clinical impression. A prospective study collecting more complete documentation of preoperative and postoperative adduction in both groups is necessary to confirm or refute this claim. The SRT patient in our study who developed an early, severe overcorrection is the same patient described in an earlier study by Mehendale and colleagues.9 Overcorrection has also been reported by Morad and Kekunnaya,24 especially when SRT was combined with BMR. We recommend reserving the SRT procedure for patients with a larger angle of deviation and severe abduction limitation. When the deviation is greater than 50Δ, we combine the SRT with BMR. In all cases (SRT and non-SRT), we place the medial rectus muscle(s) on adjustable sutures25,26 to reduce the likelihood of an early overcorrection. In 2 SRT patients in the present series, the medial rectus muscle was adjusted back to a 0-mm recession in the recovery room 1 hour postoperatively, with excellent long-term results in both cases. We observed a trend toward long-term drift in opposite directions in the 2 groups (2.0Δ decrease in esotropia and 2.5° improvement in head turn in the SRT group vs a 3.7Δ increase in esotropia and 2.1° recurrence of head turn in the non-SRT group). Although these differences were not statistically significant, they align with our clinical impression that the SRT procedure seems to have a more sustained effect over time than the MRc procedure. If valid, a difference in drift may occur because the force of the medial rectus muscle is unbalanced with MRc alone, allowing development of further contracture and inelasticity over time. Perhaps a balancing abducting force provided by SRT prevents this evolution. Although the mean follow-up was not identical in the 2 long-term groups, the shorter follow-up for the SRT group was longer than 2 years, which should be sufficient to allow for valid comparison. A change in outward vector force provided by SRT would carry with it a possible increased likelihood of long-term over-
ARTICLE INFORMATION Submitted for Publication: August 17, 2013; final revision received January 6, 2014; accepted January 7, 2014. 674
correction. Velez and colleagues27 reported late exotropia in 14 of 51 patients followed up after VRT with or without subsequent MRc. We advise against targeting an overcorrection when performing a transposition procedure in esotropic Duane syndrome. We observed better abduction in elevation or in depression preoperatively in 7 of 16 patients. The improvement in abduction observed with SRT may have been greater in elevation, which is not unexpected considering the asymmetric transfer of force in the single muscle transposition. In our clinical practice, the field of binocular single vision is not measured routinely after surgery. However, if the improvement in abduction exceeded the limitation of adduction after surgery, one can infer that the field of single binocular vision might have been enhanced in patients treated with SRT. Based on our current state of understanding of extraocular muscle function, one would predict that an unbalanced temporal transposition of the superior rectus muscle would create torsional diplopia. This prediction was not supported by our observations intraoperatively12 or postoperatively. Superior rectus transposition may induce some intorsion; however, our observations suggest that the risk for symptomatic torsional diplopia seems to be very low. We recommend that future studies document torsion with photographic methods before and after surgery. With the transposition of the superior rectus laterally, a portion of the muscle force vector directed vertically was displaced laterally. This loss of vertical force would be expected to cause a hypotropia, but none was observed. We speculate that the hypotropia did not occur when the muscle was attached along the spiral of Tillaux because it was also advanced slightly, which compensated for the reduced vertical force. If torsion and vertical strabismus were induced by surgery, they could adversely affect fusion or stereopsis, but our data show no negative impact, with improved binocular function after surgery.
Conclusions Compared with MRc or BMR alone, SRT + MRc appears to be more effective at improving abduction while allowing for a smaller MRc to align the eyes and eliminate a compensatory head posture in patients with esotropic Duane syndrome. Although not confirmed by this study alone, patients treated with SRT probably will have a reduced likelihood of developing a long-term undercorrection. In our experience, SRT + MRc is technically straightforward and allows for recession of a tight medial rectus muscle with transposition in a single procedure. It also allows for suture adjustment to reduce the effect if required. We therefore continue to recommend SRT combined with adjustable MRc for treatment of patients with profound limitation of abduction and large-angle esotropia.
Published Online: April 24, 2014. doi:10.1001/jamaophthalmol.2014.358. Author Contributions: Drs Yang and Hunter had full access to all the data in the study and take
responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Yang, Hunter.
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Acquisition, analysis, or interpretation of data: All authors. Drafting of the manuscript: Yang, Hunter. Critical revision of the manuscript for important intellectual content: MacKinnon, Dagi, Hunter. Statistical analysis: Yang, Hunter. Obtained funding: Hunter. Administrative, technical, or material support: Hunter. Study supervision: Dagi, Hunter. Conflict of Interest Disclosures: None reported. Funding/Support: This study was supported in part by the Children’s Hospital Ophthalmology Foundation, Boston, Massachusetts (Dr Hunter). Role of the Sponsor: The funding source had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. REFERENCES 1. Barbe ME, Scott WE, Kutschke PJ. A simplified approach to the treatment of Duane’s syndrome. Br J Ophthalmol. 2004;88(1):131-138. 2. Dotan G, Klein A, Ela-Dalman N, Shulman S, Stolovitch C. The efficacy of asymmetric bilateral medial rectus muscle recession surgery in unilateral, esotropic, type 1 Duane syndrome. J AAPOS. 2012;16(6):543-547. 3. Sharma P. Unilateral rectus muscle recession in the treatment of Duane syndrome. J AAPOS. 2012; 16(5):492-493. 4. Merino P, Merino M, Gómez De Liaño P, Blanco N. Horizontal rectus surgery in Duane syndrome. Eur J Ophthalmol. 2012;22(2):125-130. 5. Natan K, Traboulsi EI. Unilateral rectus muscle recession in the treatment of Duane syndrome. J AAPOS. 2012;16(2):145-149. 6. Nelson LB. Severe adduction deficiency following a large medial rectus recession in Duane’s retraction syndrome. Arch Ophthalmol. 1986;104 (6):859-862.
Original Investigation Research
in Duane’s retraction syndrome type 1. J Pediatr Ophthalmol Strabismus. 1999;36(2):98-100. 8. Johnston SC, Crouch ER Jr, Crouch ER. An innovative approach to transposition surgery is effective in treatment of Duane’s syndrome with esotropia [ARVO abstract 2475-B403]. Invest Ophthalmol Vis Sci. 2006;47:eAbstract 2475. 9. Mehendale RA, Dagi LR, Wu C, Ledoux D, Johnston S, Hunter DG. Superior rectus transposition and medial rectus recession for Duane syndrome and sixth nerve palsy. Arch Ophthalmol. 2012;130(2):195-201. 10. Cestari DG, Hunter DG. Learning Strabismus Surgery: A Case-Based Approach. Philadelphia, PA: Lippincott Williams Wilkins; 2012. 11. Aligning the Eyes: Boston Children’s Hospital/OR Live webcast. http://orlive.com /childrenshospitalboston/videos/aligning-the-eyes. Accessed March 7, 2014. 12. Holmes JM, Hatt SR, Leske DA. Intraoperative monitoring of torsion to prevent vertical deviations during augmented vertical rectus transposition surgery. J AAPOS. 2012;16(2):136-140. 13. Greenberg MF, Pollard ZF. Poor results after recession of both medial rectus muscles in unilateral small-angle Duane’s syndrome, type I. J AAPOS. 2003;7(2):142-145. 14. Jampolsky A. Strategies in strabismus surgery. In: Crawford IC, Flynn IT, Haik BG, et al, eds. Pediatric Ophthalmology and Strabismus: Transactions of the New Orleans Academy of Ophthalmology. New York, NY: Raven Press; 1985: 363-368. 15. Farvardin M, Rad AH, Ashrafzadeh A. Results of bilateral medial rectus muscle recession in unilateral esotropic Duane syndrome. J AAPOS. 2009;13(4): 339-342. 16. Wright KW. Color Atlas of Strabismus Surgery: Strategies and Techniques. 3rd ed. New York, NY: Springer Science Business Media LLC; 2007:61-62.
18. Velez FG, Foster RS, Rosenbaum AL. Vertical rectus muscle augmented transposition in Duane syndrome. J AAPOS. 2001;5(2):105-113. 19. Foster RS. Vertical muscle transposition augmented with lateral fixation. J AAPOS. 1997;1(1): 20-30. 20. Wong I, Leo SW, Khoo BK. Loop myopexy for treatment of myopic strabismus fixus. J AAPOS. 2005;9(6):589-591. 21. Struck MC. Augmented vertical rectus transposition surgery with single posterior fixation suture: modification of Foster technique. J AAPOS. 2009;13(4):343-349. 22. Rosenbaum AL. Costenbader lecture: the efficacy of rectus muscle transposition surgery in esotropic Duane syndrome and VI nerve palsy. J AAPOS. 2004;8(5):409-419. 23. Ruth AL, Velez FG, Rosenbaum AL. Management of vertical deviations after vertical rectus transposition surgery. J AAPOS. 2009;13(1): 16-19. 24. Morad Y, Kekunnaya R. Superior rectus transposition and unilateral or bilateral medical rectus recession for Duane syndrome and sixth nerve palsy. [AAPOS abstract 24]. J AAPOS. 2013;17: 119. 25. Nihalani BR, Whitman MC, Salgado CM, Loudon SE, Hunter DG. Short tag noose technique for optional and late suture adjustment in strabismus surgery. Arch Ophthalmol. 2009;127(12):1584-1590. 26. Hunter DG, Dingeman RS, Nihalani BR. Adjustable sutures in strabismus surgery. In: Wilson ME, ed. Pediatric Ophthalmology: Current Thought and a Practical Guide. Heidelberg, Germany: Springer; 2008:213-226. 27. Velez FG, Laursen JK, Pineles SL. Risk factors for consecutive exotropia after vertical rectus transposition for esotropic Duane retraction syndrome. J AAPOS. 2011;15(4):326-330.
17. Archer SM. Contralateral medial rectus muscle recession in patients with Duane syndrome [comment]. J AAPOS. 2004;8(3):296.
7. Shiratori A, Kameyama C, Sibasaki K. Adduction deficiency following a large medial rectus recession
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Original Investigation
Superior Rectus Transposition vs Medial Rectus Recession for Treatment of Esotropic Duane Syndrome Shiqiang Yang, MD; Sarah MacKinnon, MSc, OC(C); Linda R. Dagi, MD; David G. Hunter, MD, PhD
IMPORTANCE Superior rectus transposition (SRT) with or without medial rectus recession
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(MRc) has been introduced as an alternative to MRc alone for treatment of esotropic Duane syndrome; however, the effectiveness of these procedures has not been compared previously. OBJECTIVE To compare the safety and efficacy of MRc and SRT in treatment of Duane
syndrome. DESIGN, SETTING, AND PARTICIPANTS Retrospective medical record review of all patients with esotropic Duane syndrome who underwent surgical treatment from January 1, 2006, through December 31, 2012, in a multispecialty, hospital-based pediatric ophthalmology/adult strabismus practice at Boston Children’s Hospital. Patients in the SRT group underwent SRT with or without MRc; those in the non-SRT group underwent unilateral or bilateral MRc. EXPOSURES Surgical treatment of esotropic Duane syndrome. MAIN OUTCOMES AND MEASURES Binocular alignment, ocular ductions, head position, stereopsis, and fundus torsion were recorded before surgery and at the 2-month and final postoperative visits. We also evaluated postoperative drift. RESULTS The medical record review identified 36 patients who underwent 37 procedures, including 19 in the SRT group (13 SRT + MRc and 6 SRT alone) and 18 in the non-SRT group (11 unilateral MRc and 7 bilateral medial rectus resession). Mean MRc was smaller when performed with SRT (3.3 vs 5.3 mm; P = .004). Although the initial deviation was larger in the SRT group, both groups had a similar improvement in esotropia and head turn. Abduction improved by at least 1 unit in 15 of 19 patients in the SRT group (79%) vs 5 of 18 in the non-SRT group (28%). In 24 patients followed up for more than 6 months, mean esotropia decreased from 8.2 to 6.1 prism diopters (Δ) in the SRT group (n = 12) but increased from 7.2 to 10.9Δ in the non-SRT group (n = 12). CONCLUSIONS AND RELEVANCE The combination of SRT and MRc was more effective than MRc or bilateral medial rectus resession at improving abduction while allowing for a smaller recession to align the eyes and eliminate a compensatory head posture. Although any surgery on the vertical rectus muscles should in theory increase the risk for vertical or torsional complications, to date this theory has not been borne out in our patients. Patients treated with SRT appear to have a reduced likelihood of long-term undercorrection. We therefore recommend SRT with adjustable MRc for treatment of Duane syndrome in patients with larger amounts of esotropia.
JAMA Ophthalmol. 2014;132(6):669-675. doi:10.1001/jamaophthalmol.2014.358 Published online April 24, 2014.
Author Affiliations: Department of Ophthalmology, Boston Children’s Hospital, Boston, Massachusetts (Yang, MacKinnon, Dagi, Hunter); now with Department of Strabismus and Pediatric Ophthalmology, Tianjin Eye Hospital, Tianjin Eye Institute, Clinical College of Ophthalmology, Tianjin, China (Yang); Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts (Dagi, Hunter). Corresponding Author: David G. Hunter, MD, PhD, Department of Ophthalmology, Boston Children’s Hospital, 300 Longwood Ave, Fegan 4, Boston, MA 02115 (david.hunter @childrens.harvard.edu).
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Research Original Investigation
Surgical Treatment of Esotropic Duane Syndrome
D
uane syndrome is a congenital cranial dysinnervation disorder characterized by limitation of horizontal eye movement(s) with aberrant innervation causing globe retraction in adduction. Most patients have esotropia in primary gaze, limited abduction, and a head turn toward the involved side (esotropic type 1 Duane syndrome). Various surgical approaches have been proposed to improve binocular alignment and reduce head turn in Duane syndrome. Unilateral or bilateral medial rectus recessions (MRc or BMR, respectively) are common procedures and are usually satisfactory for correcting alignment in primary gaze and reducing torticollis1-4; however, MRc does not improve the abduction limitation.5 Indeed, a large MRc limits adduction,6,7 which in turn may cause difficulty with convergence and in contralateral gaze. In an effort to improve abduction without limiting adduction in patients with Duane syndrome, a variety of transposition procedures have been developed, including augmented transposition of both vertical rectus muscles (VRT). The superior rectus transposition (SRT) was developed subsequently to reduce the number of muscles requiring surgery while decreasing the likelihood of inducing a vertical deviation observed with VRT.8 A recently published experience combining SRT with an adjustable MRc in patients with esotropic Duane syndrome9 found results comparable to those of VRT but with a lower incidence of induced hypertropia. However, whether the potential benefits of SRT or similar transposition procedures warrant the added complexity and potential risk for vertical deviation associated with the procedure remains unclear. The purpose of this study is to address this question by comparing the results of SRT with those of MRc in terms of postoperative alignment, head turn, and ocular ductions and long-term stability of alignment.
Methods This retrospective study was approved by the institutional review board of Boston Children’s Hospital. We used billing records to identify patients treated for Duane syndrome in the Department of Ophthalmology from January 1, 2006, through December 31, 2012. We included patients with esotropic Duane syndrome who were treated with SRT or MRc. Patients were divided into the SRT group, consisting of patients who had SRT alone or in combination with MRc (SRT + MRc), and the nonSRT group, consisting of patients who had unilateral MRc or BMR. Patients were excluded if follow-up was less than 1 month or if VRT surgery had been performed previously. In cases in which patients underwent MRc followed more than 2 years later by SRT, the 2 procedures were analyzed separately: the first in the non-SRT group, the second in the SRT group. Superior rectus transposition was performed as described previously.9-11 Briefly, the superior rectus muscle was isolated, with care taken to clear the muscle of attachments to overlying levator and underlying superior oblique tendon. The muscle was secured, detached, and then reattached to the eye along the spiral of Tillaux, spanning the gap between the original lateral and superior rectus muscle insertions. A loop myopexy was then placed between the lateral and superior rec670
tus muscles 8 to 10 mm posterior to the insertion, incorporating approximately one-quarter of the muscle width, in most cases without a scleral pass. Medial rectus recession was performed per the preference and surgical tables of the individual surgeon. Sensorimotor examination data were extracted from the preoperative evaluation, 2 months after surgical intervention, and at the final follow-up examination. Only patients with follow-up longer than 6 months were included for analysis of long-term stability (postoperative drift). At each point, we recorded the angle of esotropia in near and distance straightahead gaze, head turn in degrees, limitation of abduction (including limitation in elevation and depression), limitation of adduction, stereopsis (by Titmus fly and Randot circles), hypotropia in straight-ahead gaze and elevation, and torsion (by the double-Maddox rod test and/or indirect ophthalmoscopy as available in the record). Alignment was recorded from the orthoptic evaluation of distance deviation. Ductions were graded on a scale from 0 (indicating full duction) through −4 (indicating an eye that was able to move to the midline) and −5 (indicating an eye that approached but was unable to reach the midline) to a maximum of −8 (indicating a rare case in which the eye was nearly fixed in an extremely adducted position). Head turn was measured with a goniometer at distance fixation or using clinical photographs. In SRT cases performed toward the end of the study period, we monitored intraoperative torsional changes as described by Holmes et al12 (Supplement [eFigure 1]). Statistical calculations were performed using commercially available software (Prism 5.0; GraphPad Software, Inc [http://www.graphpad.com]). We used 2-way analysis of variance to compare the preoperative and postoperative results of the SRT and non-SRT groups and 1-way analysis of variance to analyze the change in deviation angle and head turn of the groups undergoing SRT + MRc, SRT alone, MRc alone, and BMR. If a patient in the SRT group had undergone previous MRc, the amount of MRc was included in the calculation of MRc dosage. Titmus stereopsis of greater than 2 lines was considered clinically meaningful. We used Bonferroni post hoc tests for multiple comparisons and an unpaired t test for comparisons where appropriate. P < .05 was considered statistically significant.
Results The medical record review identified 37 patients with esotropic Duane syndrome who underwent 38 procedures. One procedure was excluded for lack of adequate follow-up, leaving 36 patients who underwent 37 procedures by 9 surgeons, with 20 of the 37 performed by two of us (L.R.D. and D.G.H.), including 13 of 19 SRT procedures. The SRT group included 13 cases of SRT + MRc and 6 cases of SRT alone. Six of 13 SRT + MRc cases underwent muscle-to-muscle augmentation (superior to lateral rectus muscle) without scleral fixation, and 7 underwent muscle-to-sclera augmentation (when the lateral rectus muscle was recessed or lax). The non-SRT group included 11 cases of MRc alone and 7 cases of BMR.
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Among the 6 cases of SRT without MRc, 2 had undergone an MRc procedure more than 2 years previously (for which the MRc amount was included in the calculation of MRc performed); in 2 additional cases, the MRc was reduced to 0 at the time of postoperative suture adjustment. One case of SRT alone was combined with an ipsilateral inferior oblique recession to treat hypertropia in adduction.
Characteristics of patients in the SRT and non-SRT groups are summarized and compared in the Table. The amount of MRc performed (Supplement [eFigure 2]) was significantly larger in the non-SRT group (mean [SD], 5.3 [1.3] mm) than in the SRT group (3.3 [2.3] mm) (P = .004). The mean postoperative follow-up was 14.6 months for the SRT group and 32.0 months for the non-SRT group. Although the initial deviation was greater in the SRT group and both procedures significantly decreased the angle of esotropia, the SRT + MRc procedure appeared to be most effective (Figure 1A-C). Superior rectus transposition alone was comparable in effect to MRc alone and BMR. The SRT and non-SRT groups showed notable improvements in head turn (Figure 1D-F). As with angle of deviation, the SRT + MRc group appeared to show the greatest efficacy, but the difference in head position was not statistically significant. Stereopsis improved in both groups (Table), with 3 patients in each group improving by 3 or more lines on the Titmus test. No patient had a reduction in stereopsis. Preoperative and postoperative limitation of adduction and abduction were available for all procedures. Abduction significantly improved in the SRT group but not in the non-SRT group (Figure 2A and B). We found a small but significant increase in adduction limitation in the SRT and
Table. Patient Characteristics and Stereopsis Groupa Characteristic
SRT
Age, mean (SD), y
Non-SRT
10.3 (12.9)
Male
13.6 (14.1)
11/19 (58)
Follow-up, mean (SD) [range], mo
6/18 (33)
14.6 (16.2) [1-63]
Left eye
32.0 (31.0) [1-99]
16/19 (84)
15/18 (83)
4
1
Preoperative
10/14 (71)
11/14 (79)
Postoperative
14/16 (88)
16/18 (89)
Severe globe retraction or up/down shoot Stereopsis ≥3000 arc seconds
Abbreviation: SRT, superior rectus transposition. a
Unless otherwise indicated, data are expressed as number of cases/number in group for whom data were available (percentage).
Figure 1. Comparison of Surgical Results of Deviation Angle and Head Turn Preoperative
30
b
20 10 0 SRT
c 60 40 c 20 0 –20
Non-SRT
SRT E
30
a a
20
10
0 SRT
Non-SRT
80 c 60 40 c 20 0 –20
Non-SRT
SRT + MRc
SRT Alone
MRc Alone
BMR
SRT + MRc
SRT Alone
MRc Alone
BMR
F
Change of Head Turn, Degrees
D
Head Turn, Degrees
80
Change of Angle in Primary Gaze Position, Δ
a
Non-SRT subgroups
C
Change of Head Turn, Degrees
Angle of Primary Gaze, Δ
40
SRT subgroups B
Change of Angle in Primary Gaze Position, Δ
Final follow-up
A
50 40 30 20 10 0 SRT
Non-SRT
A, Deviation angle before surgery and at final follow-up in the superior rectus transposition (SRT) and non-SRT surgical groups. Positive values represent esotropia; error bars, SEM. B, Box-and-whiskers plot of postoperative change of the deviation angle in both surgical groups. C, Box-and-whiskers plot of postoperative change of deviation angle in each surgical subgroup. D, Head turn before and after surgery in the SRT and non-SRT groups. Positive values represent improvement in head turn (in degrees); error bars, SEM. E, Box-and-whiskers plot of postoperative change in head turn in both surgical groups. F, Box-and-whiskers plot of postoperative change of head turn in each surgical subgroup. Unless otherwise indicated, positive values represent the
50 40 30 20 10 0
difference between preoperative and final values. In the box-and-whiskers plots, the bottom and top of each box represent the 25th and 75th percentiles (the lower and upper quartiles, respectively); the band near the middle of the box, the 50th percentile (the median). The ends of the whiskers represent the minimum and maximum of all the data. BMR indicates bilateral medial rectus resession; MRc, medial rectus recession; and Δ, prism diopter. a P < .001. b P < .05. c P < .01.
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Figure 2. Comparison of Limitation of Abduction and Adduction Before and After Surgery Preoperative Final follow-up A
B
Improvement of Abduction Limitation
Limitation of Abduction
0 –1 –2 –3 –4 –5
a SRT
2 1 0 –1 SRT
Non-SRT
D
0.0
–0.5
–1.0 b
a
SRT
Non-SRT
–1.5
Change of Adduction Limitation
0
Limitation of Adduction
3
Non-SRT
C
a
4
–0.5 –1.0 –1.5 –2.0 –2.5 SRT + MRc
SRT Alone
MRc Alone
BMR
Duction grades range from 0 (full duction) through −4 (eye was able to move to the midline) and −5 (eye approached but was unable to reach the midline). A, Limitation of abduction preoperatively and at final follow-up in the superior rectus transposition (SRT) and non-SRT surgical groups. B, Postoperative improvement of abduction in each surgical subgroup; clinically significant improvement of at least 1.0 units was observed in 15 of 19 SRT patients (79%) and 5 of 18 non-SRT patients (28%). C, Limitation of adduction preoperatively and at final follow-up in the SRT and non-SRT surgical groups. D, Postoperative improvement of adduction in each surgical subgroup. BMR indicates bilateral medial rectus resession; MRc, medial rectus recession; error bars, SEM; markers, individual patients; and bands near the middle, the 50th percentile. a P < .001. b P < .01.
non-SRT groups (Figure 2C and D). Two patients from each group developed postoperative adduction limitation of more than −1. In 15 patients in the SRT group, abduction was measured not only in the horizontal meridian but also in elevation and depression (Figure 3). In some cases, preoperative abduction was slightly better in elevation (Figure 3B) or in depression (Figure 3C). After surgery, we found a trend toward more improvement in abduction in elevation than in depression (Figure 3D and Supplement [eFigure 3]). No patient in either group described torsional diplopia postoperatively. Of 9 patients in whom torsion was measured before and after surgery (all but 1 in the SRT group), 8 showed no change postoperatively. A +1 (asymptomatic) increase in intorsion was observed in 1 SRT patient. Intraoperative monitoring of torsion performed in 3 patients in the SRT group showed no change at the end of the procedure. Small, asymptomatic vertical deviations in adduction were present in 3 of 18 patients in the SRT group and 3 of 19 in the MRc group. No patient developed a symptomatic vertical strabismus after surgery; however, 2 SRT patients had a 672
new postoperative hypotropia or hypophoria. An 18-yearold girl developed hypotropia of 4 prism diopters (Δ) in straight-ahead gaze without diplopia; at the final postoperative visit, she had 20 seconds of stereopsis with a 5° residual head turn. A 5-year-old girl underwent a 6-mm ipsilateral MRc and developed a hypophoria after a subsequent SRT. Three years later, she had a residual esotropia of 8Δ and a persistent left hypophoria of 10Δ but was asymptomatic with good fusion and 70 arc seconds of stereopsis with a 5° residual left head turn. Measurements of long-term drift in postoperative alignment were available for evaluation in 24 patients (12 in the SRT group [mean follow-up, 22 months] and 12 in the non-SRT group [47 months]). Although we found a trend toward further increase in surgical effect in the SRT group (mean esotropia decreased from 8.2Δ to 6.1Δ), the trend was toward a loss of effect in the non-SRT group (mean esotropia increased from 7.2Δ to 10.9Δ) (Figure 4A). These trends were not statistically significant (P = .33). A similar trend was observed in the improvement in head position (Figure 4B), with an improvement over time in the SRT group (decrease from 5.0° to 2.5°) compared with a recurrence over time in the non-SRT group (increase from 3.3° to 5.5°). Again, these observed trends were not statistically significant (P = .14). No significant long-term changes in limitation of abduction (Figure 4C) or adduction (Figure 4D) were observed.
Discussion Debate is ongoing about the best approach for treatment of esotropic Duane syndrome and other causes of abduction limitation. Recession of the ipsilateral medial rectus muscle is an attractive option for its simplicity and predictability, and indeed this approach will improve the deviation and head turn in straight-ahead gaze.1,3-5 Unfortunately, to correct larger deviations and head turns, a large MRc is required, which often induces limitation of adduction w ithout improv ing abduction.6,7 Bilateral MRc is used for larger angles. Recessions of the contralateral medial rectus muscle are sometimes used, but the approach remains controversial,2,13-15 with a paradoxical increase in head turn described in some cases.16,17 Transposition procedures have been proposed as a means of increasing lateral force to the eye to compensate for the lack of lateral rectus muscle function. At present, full, augmented VRT18 is a widely used approach.19-21 Rosenbaum22 showed a marked improvement of abduction and in the field of binocular single vision in patients with Duane syndrome who undergo VRT. Concerns about induced hypotropia have limited widespread adoption of VRT.23 In addition, when the medial rectus muscle is tight, concerns about anterior segment ischemia limit the option of MRc. We adopted the SRT in 2006 after Johnston and colleagues8 first presented results in the treatment of Duane syndrome and sixth nerve palsy. Mehendale and colleagues9 reported successful treatment of patients with complete loss of lateral rectus function. Morad and Kekunnaya24 have also reported good results of SRT + MRc for Duane syndrome.
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Original Investigation Research
Figure 3. Changes in Abduction in Elevation vs Depression A
B
C
Elevation Midline Depression Neither 0
2
4
6
8
10
Maximal Preoperative Abduction D
Elevation Midline Depression Neither 0
2
4
6
Improvement in Abduction
A, Preoperative images of a 6-year-old boy with Duane syndrome of the right eye. Improved abduction of the right eye is observed in elevation (top panel) compared with vertical midline or depression. B, Preoperative images of a 2-year-old girl with Duane syndrome of the left eye. Improved abduction of the
left eye is observed in depression. C, Maximal abduction in the affected eye in elevation and depression before surgery. D, Maximal improvement of abduction in elevation and depression.
Figure 4. Postoperative Drift of Surgical Results of Deviation, Head Turn, and Ductions in the Superior Rectus Transposition (SRT) and Non-SRT Surgical Groups A
B
2 mo
>6 mo
8
Postoperative Head Turn, Degrees
Postoperative Deviation in Primary Gaze, Δ
15
10
5
0
6 4 2 0
SRT
Non-SRT
C
SRT
Non-SRT
SRT
Non-SRT
D
0.0
–1 –2 –3
a a
–4
Postoperative Limitation of Adduction
Postoperative Limitation of Abduction
0
–0.5
–1.0
–1.5 SRT
Non-SRT
A, Postoperative drift of deviation (given in prism diopters [Δ]). Positive values represent the angle of deviation in straight-ahead gaze. B, Postoperative drift of head turn. Positive values represent the angle of head turn. C, Postoperative drift of limitation of abduction. D, Postoperative drift of limitation of adduction. Duction grades range from 0 (full duction) through −4 (eye was able to move to
the midline) and −5 (eye approached but was unable to reach the midline). Negative values represent the limitation of abduction and adduction. Error bars represent SEM. a P < .05.
In our multiphysician strabismus practice, patients with esotropic Duane syndrome were treated with SRT and nonSRT procedures according to surgeon preference, and all patients were examined by the same orthoptic team according
to the same documentation guidelines. More patients undergoing non-SRT procedures had surgery earlier in the study period, which resulted in a longer overall follow-up for the nonSRT group. Although the present study was not a randomized
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prospective trial and is subject to bias of patient selection, incomplete documentation, and an unequal follow-up, the available data allowed us to perform, to our knowledge, the first comparative study of the sensorimotor impact of SRT + MRc in patients with esotropic Duane syndrome. Abduction improved significantly in patients treated with SRT compared with MRc alone. We found a comparable reduction of esotropia in straight-ahead gaze, but the SRT group had a larger preoperative esotropia than the non-SRT group; thus, surgeons might have selected SRT for patients with a larger preoperative angle. The improvement in abduction with SRT (15 of 19 patients [79%]) was similar to the results reported previously in a smaller group of patients.9 The improvement in abduction with MRc (5 of 18 patients [28%]) was similar to the 29% improvement reported by Merino et al4 (although they graded abduction limitation only on a scale of −1 through −3). By combining SRT with MRc, a significantly smaller MRc was required to treat the esotropia. We also found a trend toward better preservation of adduction in the SRT group, which matches our clinical impression. A prospective study collecting more complete documentation of preoperative and postoperative adduction in both groups is necessary to confirm or refute this claim. The SRT patient in our study who developed an early, severe overcorrection is the same patient described in an earlier study by Mehendale and colleagues.9 Overcorrection has also been reported by Morad and Kekunnaya,24 especially when SRT was combined with BMR. We recommend reserving the SRT procedure for patients with a larger angle of deviation and severe abduction limitation. When the deviation is greater than 50Δ, we combine the SRT with BMR. In all cases (SRT and non-SRT), we place the medial rectus muscle(s) on adjustable sutures25,26 to reduce the likelihood of an early overcorrection. In 2 SRT patients in the present series, the medial rectus muscle was adjusted back to a 0-mm recession in the recovery room 1 hour postoperatively, with excellent long-term results in both cases. We observed a trend toward long-term drift in opposite directions in the 2 groups (2.0Δ decrease in esotropia and 2.5° improvement in head turn in the SRT group vs a 3.7Δ increase in esotropia and 2.1° recurrence of head turn in the non-SRT group). Although these differences were not statistically significant, they align with our clinical impression that the SRT procedure seems to have a more sustained effect over time than the MRc procedure. If valid, a difference in drift may occur because the force of the medial rectus muscle is unbalanced with MRc alone, allowing development of further contracture and inelasticity over time. Perhaps a balancing abducting force provided by SRT prevents this evolution. Although the mean follow-up was not identical in the 2 long-term groups, the shorter follow-up for the SRT group was longer than 2 years, which should be sufficient to allow for valid comparison. A change in outward vector force provided by SRT would carry with it a possible increased likelihood of long-term over-
ARTICLE INFORMATION Submitted for Publication: August 17, 2013; final revision received January 6, 2014; accepted January 7, 2014. 674
correction. Velez and colleagues27 reported late exotropia in 14 of 51 patients followed up after VRT with or without subsequent MRc. We advise against targeting an overcorrection when performing a transposition procedure in esotropic Duane syndrome. We observed better abduction in elevation or in depression preoperatively in 7 of 16 patients. The improvement in abduction observed with SRT may have been greater in elevation, which is not unexpected considering the asymmetric transfer of force in the single muscle transposition. In our clinical practice, the field of binocular single vision is not measured routinely after surgery. However, if the improvement in abduction exceeded the limitation of adduction after surgery, one can infer that the field of single binocular vision might have been enhanced in patients treated with SRT. Based on our current state of understanding of extraocular muscle function, one would predict that an unbalanced temporal transposition of the superior rectus muscle would create torsional diplopia. This prediction was not supported by our observations intraoperatively12 or postoperatively. Superior rectus transposition may induce some intorsion; however, our observations suggest that the risk for symptomatic torsional diplopia seems to be very low. We recommend that future studies document torsion with photographic methods before and after surgery. With the transposition of the superior rectus laterally, a portion of the muscle force vector directed vertically was displaced laterally. This loss of vertical force would be expected to cause a hypotropia, but none was observed. We speculate that the hypotropia did not occur when the muscle was attached along the spiral of Tillaux because it was also advanced slightly, which compensated for the reduced vertical force. If torsion and vertical strabismus were induced by surgery, they could adversely affect fusion or stereopsis, but our data show no negative impact, with improved binocular function after surgery.
Conclusions Compared with MRc or BMR alone, SRT + MRc appears to be more effective at improving abduction while allowing for a smaller MRc to align the eyes and eliminate a compensatory head posture in patients with esotropic Duane syndrome. Although not confirmed by this study alone, patients treated with SRT probably will have a reduced likelihood of developing a long-term undercorrection. In our experience, SRT + MRc is technically straightforward and allows for recession of a tight medial rectus muscle with transposition in a single procedure. It also allows for suture adjustment to reduce the effect if required. We therefore continue to recommend SRT combined with adjustable MRc for treatment of patients with profound limitation of abduction and large-angle esotropia.
Published Online: April 24, 2014. doi:10.1001/jamaophthalmol.2014.358. Author Contributions: Drs Yang and Hunter had full access to all the data in the study and take
responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Yang, Hunter.
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Surgical Treatment of Esotropic Duane Syndrome
Acquisition, analysis, or interpretation of data: All authors. Drafting of the manuscript: Yang, Hunter. Critical revision of the manuscript for important intellectual content: MacKinnon, Dagi, Hunter. Statistical analysis: Yang, Hunter. Obtained funding: Hunter. Administrative, technical, or material support: Hunter. Study supervision: Dagi, Hunter. Conflict of Interest Disclosures: None reported. Funding/Support: This study was supported in part by the Children’s Hospital Ophthalmology Foundation, Boston, Massachusetts (Dr Hunter). Role of the Sponsor: The funding source had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. REFERENCES 1. Barbe ME, Scott WE, Kutschke PJ. A simplified approach to the treatment of Duane’s syndrome. Br J Ophthalmol. 2004;88(1):131-138. 2. Dotan G, Klein A, Ela-Dalman N, Shulman S, Stolovitch C. The efficacy of asymmetric bilateral medial rectus muscle recession surgery in unilateral, esotropic, type 1 Duane syndrome. J AAPOS. 2012;16(6):543-547. 3. Sharma P. Unilateral rectus muscle recession in the treatment of Duane syndrome. J AAPOS. 2012; 16(5):492-493. 4. Merino P, Merino M, Gómez De Liaño P, Blanco N. Horizontal rectus surgery in Duane syndrome. Eur J Ophthalmol. 2012;22(2):125-130. 5. Natan K, Traboulsi EI. Unilateral rectus muscle recession in the treatment of Duane syndrome. J AAPOS. 2012;16(2):145-149. 6. Nelson LB. Severe adduction deficiency following a large medial rectus recession in Duane’s retraction syndrome. Arch Ophthalmol. 1986;104 (6):859-862.
Original Investigation Research
in Duane’s retraction syndrome type 1. J Pediatr Ophthalmol Strabismus. 1999;36(2):98-100. 8. Johnston SC, Crouch ER Jr, Crouch ER. An innovative approach to transposition surgery is effective in treatment of Duane’s syndrome with esotropia [ARVO abstract 2475-B403]. Invest Ophthalmol Vis Sci. 2006;47:eAbstract 2475. 9. Mehendale RA, Dagi LR, Wu C, Ledoux D, Johnston S, Hunter DG. Superior rectus transposition and medial rectus recession for Duane syndrome and sixth nerve palsy. Arch Ophthalmol. 2012;130(2):195-201. 10. Cestari DG, Hunter DG. Learning Strabismus Surgery: A Case-Based Approach. Philadelphia, PA: Lippincott Williams Wilkins; 2012. 11. Aligning the Eyes: Boston Children’s Hospital/OR Live webcast. http://orlive.com /childrenshospitalboston/videos/aligning-the-eyes. Accessed March 7, 2014. 12. Holmes JM, Hatt SR, Leske DA. Intraoperative monitoring of torsion to prevent vertical deviations during augmented vertical rectus transposition surgery. J AAPOS. 2012;16(2):136-140. 13. Greenberg MF, Pollard ZF. Poor results after recession of both medial rectus muscles in unilateral small-angle Duane’s syndrome, type I. J AAPOS. 2003;7(2):142-145. 14. Jampolsky A. Strategies in strabismus surgery. In: Crawford IC, Flynn IT, Haik BG, et al, eds. Pediatric Ophthalmology and Strabismus: Transactions of the New Orleans Academy of Ophthalmology. New York, NY: Raven Press; 1985: 363-368. 15. Farvardin M, Rad AH, Ashrafzadeh A. Results of bilateral medial rectus muscle recession in unilateral esotropic Duane syndrome. J AAPOS. 2009;13(4): 339-342. 16. Wright KW. Color Atlas of Strabismus Surgery: Strategies and Techniques. 3rd ed. New York, NY: Springer Science Business Media LLC; 2007:61-62.
18. Velez FG, Foster RS, Rosenbaum AL. Vertical rectus muscle augmented transposition in Duane syndrome. J AAPOS. 2001;5(2):105-113. 19. Foster RS. Vertical muscle transposition augmented with lateral fixation. J AAPOS. 1997;1(1): 20-30. 20. Wong I, Leo SW, Khoo BK. Loop myopexy for treatment of myopic strabismus fixus. J AAPOS. 2005;9(6):589-591. 21. Struck MC. Augmented vertical rectus transposition surgery with single posterior fixation suture: modification of Foster technique. J AAPOS. 2009;13(4):343-349. 22. Rosenbaum AL. Costenbader lecture: the efficacy of rectus muscle transposition surgery in esotropic Duane syndrome and VI nerve palsy. J AAPOS. 2004;8(5):409-419. 23. Ruth AL, Velez FG, Rosenbaum AL. Management of vertical deviations after vertical rectus transposition surgery. J AAPOS. 2009;13(1): 16-19. 24. Morad Y, Kekunnaya R. Superior rectus transposition and unilateral or bilateral medical rectus recession for Duane syndrome and sixth nerve palsy. [AAPOS abstract 24]. J AAPOS. 2013;17: 119. 25. Nihalani BR, Whitman MC, Salgado CM, Loudon SE, Hunter DG. Short tag noose technique for optional and late suture adjustment in strabismus surgery. Arch Ophthalmol. 2009;127(12):1584-1590. 26. Hunter DG, Dingeman RS, Nihalani BR. Adjustable sutures in strabismus surgery. In: Wilson ME, ed. Pediatric Ophthalmology: Current Thought and a Practical Guide. Heidelberg, Germany: Springer; 2008:213-226. 27. Velez FG, Laursen JK, Pineles SL. Risk factors for consecutive exotropia after vertical rectus transposition for esotropic Duane retraction syndrome. J AAPOS. 2011;15(4):326-330.
17. Archer SM. Contralateral medial rectus muscle recession in patients with Duane syndrome [comment]. J AAPOS. 2004;8(3):296.
7. Shiratori A, Kameyama C, Sibasaki K. Adduction deficiency following a large medial rectus recession
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