Modified Nishida's procedure for esotropia in Duane syndrome associated with Goldenhar syndrome.

Modified Nishida's procedure (no split, no tenotomy transposition) has been recently described as an effective surgery for monocular elevation deficiency and for traumatic rupture of the inferior rectus muscle. We report the modified Nishida's procedure combined with medial rectus muscle recession for the treatment of esotropia in unilateral Duane syndrome associated with Goldenhar syndrome. Following the surgery and over a 1-year follow-up period, the patient was orthophoric at distance and near, abduction improved, and the abnormal head position resolved.

Clinical characteristics and surgical approach in Duane retraction syndrome: a study of 691 patients.

PURPOSE: To evaluate the clinical findings of different types of Duane retraction syndrome (DRS). STUDY DESIGN: Retrospective. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Hummelsheim procedure combined with medial rectus recession in complete sixth nerve palsy and esotropic Duane Retraction Syndrome.

PURPOSE: To evaluate the effect of half tendon vertical rectus muscle transposition (HVRT) combined with medial rectus muscle recession (MRrec) in complete sixth nerve palsy (CSNP) and esotropic Duane Retraction Syndrome (eDRS). METHODS: A retrospective study of patients with unilateral CSNP or eDRS who underwent HVRT combined with medial rectus recession over the course of 18 years. The patients with previous strabismus surgery and follow up less than 3 months were excluded. Overcorrection was defined as any amount of exotropia, and undercorrection was defined as residual esotropia of ⩾10 prism diopter (PD). RESULTS: A total of 39 patients were enrolled including 22 CSNP and 17 eDRS patients. Esotropia improved from 45.8 ± 22 and 22.5 ± 6.4 PD to 0.8 ± 2.5 and 0.3 ± 1 PD in CSNP group and eDRS group respectively. The angle of face turn improved from 34.3 ± 8.4° and 26.5 ± 9° to 0.6 ± 2.4° and 0.8 ± 1.6° in the CSNP group and eDRS group respectively. Abduction limitation improved from -4.5 ± 0.5 and -4 ± 0 units to -2.9 ± 0.5 and -2.7 ± 0.5 units in CSNP group and eDRS group respectively. No patient developed a new vertical deviation. An overcorrection occurred in one patient of the eDRS group who improved after botulinum toxin injection in the ipsilateral lateral rectus muscle. Undercorrection was seen in two patients. CONCLUSION: HVRT combined with MRrec is an effective procedure to improve esotropia, face turn, and abduction limitation in CSNP and eDRS.

Superior Rectus Transposition and Medial Rectus Recession for Treatment of Duane Retraction Syndrome and Sixth Nerve Palsy.

Purpose: To evaluate the outcome of augmented superior rectus transposition (SRT) with medial rectus (MR) recession in patients with Duane Retraction Syndrome (DRS) and sixth nerve palsy.Methods: Twenty four patients (16 DRS and 8 sixth nerve palsy) that underwent the procedure were included. The superior rectus muscle was secured, detached, and re-attached to the sclera along the spiral of Tilaux, adjacent to lateral rectus insertion. A non-absorbable augmentation suture was passed through the sclera, 8 mm posterior to the insertion of the lateral rectus.Results: At the last follow-up, the effect of surgery in decreasing esotropia in both groups was significant (P = .001 for DRS group, P = .002 for sixth nerve palsy). In both groups, abduction deficit improved significantly (P < .001 for DRS and P = .008 for sixth nerve palsy). After the surgery, small, asymptomatic vertical deviation in primary position was induced in five patients (20.8%). Post-operatively, none of the patients complained of torsional diplopia. In the 6-month follow-up, compared with the first postoperative visit, an eso-drift at distance or near developed in 11 patients (45.8%). Of the 11 patients with eso-drift, overcorrection (exotropia of 3-14 PD) was present at the first post-operative visit in 5 cases. Four cases showed an exo-drift (2-5 PD) at distance or near over time.Conclusion: SRT with medial rectus recession improves esotropia and abduction limitation without inducing significant vertical deviations and torsional diplopia. Some of the cases that underwent SRT with MR recession may show an eso-drift. The eso-drift can correct initial exotropia in some cases.

Novel finding of atrophic extraocular muscles in Loeys-Dietz syndrome: a case report and review of the literature.

A 19-year-old man with Loeys-Dietz syndrome and right exotropic Duane syndrome after bilateral lateral rectus recessions at age 22 months presented with recurrent progressive exotropia 17 years after his initial surgery. Surgical correction was aborted intraoperatively when extreme atrophy of the right medial rectus, lateral rectus, and superior rectus muscles was observed, later corroborated by orbital magnetic resonance imaging.

The presence of anomalous extraocular bands in Duane retraction syndrome.

PURPOSE: To determine the prevalence of anomalous extraocular bands in patients who underwent surgery for Duane syndrome and to compare the clinical findings in patients with and without bands. METHODS: Thirty-one patients with Duane syndrome who had their first surgery on rectus muscles to correct the primary deviation and abnormal head posture were included in this retrospective study. Patients were divided into two groups depending on the identification of anomalous extraocular bands intraoperatively. Baseline clinical characteristics were compared between the groups. RESULTS: A total of 31 patients were included. Anomalous bands were found in 6 of 19 (32%) patients with esotropic Duane syndrome and 9 of 12 (75%) with exotropic Duane syndrome (P = 0.02). In esotropic Duane syndrome, the bands were localized under the medial rectus muscle in 5 patients and under the lateral rectus muscle in 1 patient. All of the bands in patients with exotropic Duane syndrome were under the lateral rectus muscle. The amount of preoperative primary deviation, globe retraction, and up- or downshoot were similar between groups. All of the bands had distinct tight insertion on the sclera, requiring a sharp dissection for disinsertion. In 7 cases, the anomalous band was a translucent structure that could be identified under the surgical microscope as scleral indentation during forced duction testing. Histological examination of 6 cases revealed only fibrous tissue in 4 and accompanying striated muscle tissue in 2 patients. CONCLUSIONS: The present study highlights the incidence of anomalous bands in Duane syndrome. Repeating forced duction testing after disinsertion of the affected muscle and excision of the anomalous band is helpful for intraoperative identification of these structures.

Bilateral augmented superior rectus transposition with medial rectus recession for bilateral esotropic Duane retraction syndrome.

Superior rectus transposition with medial rectus recession is commonly performed for unilateral esotropic Duane syndrome. For bilateral esotropic Duane syndrome, bilateral medial rectus recession is the most frequently performed surgery and usually provides satisfactory alignment in primary position; however, there is limited improvement in abduction. We report the outcomes of bilateral augmented superior rectus transposition and medial rectus recession in 4 patients with bilateral esotropic Duane syndrome. Postoperatively, abduction was improved in all patients; 3 were orthotropic in the primary position, and 1 had residual esotropia.

Variant types of Duane retraction syndrome: synergistic divergences and convergences.

PURPOSE: To present the clinical features of 4 patients with Duane retraction syndrome characterized by synergistic divergence or convergence, abnormal vertical movements, and accessory fibrotic bands. METHODS: The medical records of 4 patients were reviewed retrospectively for the following clinical manifestations: visual acuity, refraction, ocular alignments, ocular motility, head position, magnetic resonance imaging, surgical techniques, and outcomes. RESULTS: All 4 cases were diagnosed as variants of Duane retraction syndrome. Two cases (cases 1 and 2) had synergistic divergence with unilateral adduction deficit, and 2 (cases 3 and 4) had synergistic convergence with bilateral abduction deficit. Case 2 manifested abnormal vertical movements of the right eye, which goes down with adduction of the left eye and goes up oppositely with abduction of left eye. Accessory fibrotic bands were detected beside the medial rectus muscle of both eyes in case 3. Synergistic convergence in case 4 occurred only after the first strabismus surgery. Weakening of the misinnervated horizontal rectus muscle improved ocular alignment and head position in cases 1, 3, and 4. CONCLUSIONS: Synergistic divergence and convergence are extremely rare and may present with a great diversity of clinical features. A good outcome is very difficult to achieve; however, weakening of the misinnervated horizontal rectus muscle was therapeutic in these patients.

Contralateral lateral rectus muscle recession in a patient with unilateral exotropic Duane retraction syndrome type II: A case report.

A fixation preference for the affected eye is uncommon in patients with unilateral Duane retraction syndrome (DRS), and surgery on the fellow eye is rarely advocated. We are presenting a case report of a 9-year-old boy with unilateral DRS type II in the left eye who received lateral rectus muscle recession in his right amblyopic eye. The patient was orthophoric and his face turn was gone 6 months postoperatively. Surgery on the fellow amblyopic eye is a good choice for unilateral DRS where the affected eye dominants the fixation, and the satisfactory outcome suggests that alignment in the primary position can correct the face turn effectively despite the muscle duction deficit in the affected eye and further extend the binocular single visual field.

Surgical Outcomes of Exotropic Duane Retraction Syndrome From a Tertiary Eye Care Center.

PURPOSE: To report surgical outcomes of patients with exotropic Duane retraction syndrome. METHODS: A retrospective review of the medical records from patients with exotropic Duane retraction syndrome who underwent strabismus surgery and had at least 2 months of postoperative follow-up was conducted. Surgical success was defined as a postoperative horizontal deviation within 8 prism diopters (PD), abnormal head posture (AHP) less than 5 degrees, and a two-step decrease in overshoots. RESULTS: Seventy-three patients with exotropic Duane retraction syndrome (38, 52% male, mean age 14 ± 7.9 years) met the study criteria. Unilateral type 3 Duane retraction syndrome was seen in 56 (77%) patients and type 1 in 14 (19%) patients. The mean follow-up period was 22 weeks (range: 8 to 209 weeks). Unilateral and bilateral lateral rectus recession were performed in 48 (65%) and 25 (34%) patients, respectively. In addition to recession, a lateral rectus Y-splitting was performed in 42 (56%) patients. Five patients underwent differential medial rectus and (larger) lateral rectus recession to alleviate severe globe retraction. The mean primary position reduced from 23 PD (CI: 19.6 to 26 PD) preoperatively to 9 PD (CI: 7.02 to 11.02 PD) postoperatively (P < .0001). Postoperatively 50% of patients were orthotropic in the primary position. Surgical success for primary position deviation, AHP, and overshoots were achieved in 74%, 81%, and 71% of patients, respectively. CONCLUSIONS: In the current study, type 3 Duane retraction syndrome was the most common subtype necessitating surgery for exotropic Duane retraction syndrome. Good surgical outcomes were obtained for various indications. Unilateral or bilateral lateral rectus recession was successful in improving motor alignment and AHP. The addition of lateral rectus Y-splitting corrected overshoots greater than grade 2 in most patients with exotropic Duane retraction syndrome. [J Pediatr Ophthalmol Strabismus. 2021;58(1):9-16.].

Duane-minus (Duane sine retraction and Duane sine limitation): possible incomplete forms of Duane retraction syndrome.

PURPOSE: To report ocular motility patterns that mimic, but do not fulfil the full clinical picture of Duane retraction syndrome (DRS) and to describe their clinical features and surgical management. METHODS: This is a retrospective case series study conducted on patients with DRS, mimicking non-comitant exotropia or esotropia and a face turn. Patients were included only if they lacked either globe retraction on adduction (sine retraction) or limitation of adduction or abduction on ductions (sine limitation not >0.5). Any overshoots or pattern strabismus was recorded. The ocular motility and alignment, details of surgery and their surgical outcomes were analysed. RESULTS: Twenty-one patients were identified; 13 in the sine retraction and 8 in the sine limitation group. All patients presented with a compensatory face turn. Overshoots were present in 10 (77%) and 7 patients (88%) in the sine retraction and sine limitation groups, respectively. Forced duction test showed tightness of the ipsilateral medial and the ipsilateral lateral rectus muscle in esotropic (n = 3) and exotropic patients (n = 18), respectively. Orthotropia was achieved in 82% of patients following ipsilateral medial or lateral rectus muscle recession. CONCLUSIONS: There is a subset of patients who present with motility pattern similar to DRS but lack its complete diagnostic criteria. The presence of a face turn, overshoots on adduction or an ipsilateral tightness of the affected muscle should make one consider DRS sine retraction/sine limitation. The patients in our study responded well to lines of management similar to those of DRS.

Effect of lateral rectus muscle resection on abduction in Duane retraction syndrome type 1.

PURPOSE: To assess the effect of lateral rectus muscle resection on abduction in Duane retraction syndrome (DRS) type 1. METHODS: The medical records of patients with DRS type 1 were reviewed retrospectively. Fifteen patients who underwent lateral rectus resection were included. Prism and cover test and the Krimsky test were used to detect deviations. Ocular ductions, abnormal head position (AHP), and globe retraction were recorded. RESULTS: Nine (60.0%) patients were female. The mean age was 13.1 ± 2.3 (range, 2-34) years. Left eyes were included in ten (66.7%) patients. Mean follow-up time was 37.6 ± 16.6 (range, 6-70) months. All patients had AHP, 13 patients had mild globe retraction, and 2 patients had no globe retraction preoperatively. Mean lateral rectus resection was 3.1 ± 0.7 (range, 2.0-4.5) mm, and the mean medial rectus recession was 4.4 ± 0.6 (range, 3.0-5.0) mm. The mean preoperative deviation decreased from 23.3 ± 6.9 (range, 14-35) prism diopters (pd) to 2.2 ± 4.1 (range, 0-10) pd at near, and from 23.6 ± 7.1 (range, 14-35) pd to 1.8 ± 3.5 (range, 0-10) pd at distance, at 6 months postoperatively (p = 0.01). The mean limitation in abduction decreased from - 3.2 ± 0.9 to - 1.3 ± 1.1 postoperatively (p < 0.001). AHP resolved in all patients. There was not a limitation in adduction or a worsening of globe retraction in any patient. CONCLUSION: Lateral rectus resection can be used to improve abduction in patients with DRS type 1 who have mild globe retraction. We assume that this procedure has no worsening effect on globe retraction in appropriate cases.

Reoperation in esotropic Duane retraction syndrome: Long-term motor outcome of superior rectus transposition.

AIM: The aim was to evaluate the long-term motor outcome of superior rectus transposition procedure in patients affected by unilateral esotropic Duane retraction syndrome with residual esotropia and anomalous head position. METHODS: A retrospective analysis of medical records of patients affected by esotropic Duane retraction syndrome who underwent superior rectus transposition procedure as reoperation for residual esotropia and/or residual anomalous head position. Amount of deviation, anomalous head position, duction limitation, globe retraction, presence of upshoot/downshoot, and vertical deviation were analyzed before and after superior rectus transposition procedure. RESULTS: Twenty patients were selected. All patients underwent unilateral medial rectus recession or bilateral medial rectus recession, for unilateral esotropic Duane retraction syndrome at least 2 years before superior rectus transposition reoperation. Mean age at surgery (superior rectus transposition) was 12 ± 6.8 years, and the follow-up period was 2.7 ± 0.6. Mean deviations at distance and near before surgery were 19.5 ± 5.7 and 15.2 ± 6.8, respectively. Two patients showed upshoot. Head turn was 11.4 ± 5.1°; abduction limitation was -2.6 ± 0.9. After superior rectus transposition, all patients showed an improvement of esotropia at distance and near (8.1 ± 5.7 and 5.1 ± 5.6, respectively; p < 0.05), anomalous head position (5.6 ± 3.9°; p < 0.05), and abduction limitation (-2.3 ± 0.8; p < 0.05). No statistically significant changes occurred in globe retraction. No adduction limitation, vertical deviation, and upshoot/downshoot were present after superior rectus transposition procedure. Results were stable during follow-up. CONCLUSION: Superior rectus transposition procedure is an effective procedure in esotropic Duane retraction syndrome patients who previously undergone unilateral/bilateral medial rectus recession, with residual esotropia and anomalous head position. It allows improvement of esotropia, head turn, and partial recovery of abduction in a significant percentage of patients (30%) with no vertical complications.

Outcomes of symmetric bilateral medial rectus recession in large-angle esotropic Duane syndrome.

PURPOSE: To evaluate the efficacy of symmetric bilateral medial rectus recession in large-angle esotropic Duane retraction syndrome (DRS) with moderate to severe globe retraction. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Incidence of symptomatic vertical and torsional diplopia after superior rectus transposition for esotropic Duane syndrome and abducens nerve palsy.

PURPOSE: To report the incidence of symptomatic vertical and torsional diplopia after superior rectus transposition (SRT) for esotropic Duane syndrome and abducens nerve palsy. METHODS: The medical records of patients with esotropic Duane syndrome or abducens nerve palsy seen at Boston Children's Hospital (2006-2018) and treated with unilateral SRT with or without augmentation was performed. The primary outcome was incidence of postoperative vertical or torsional diplopia in primary position. The secondary outcome was induced vertical deviation in affected side gaze. RESULTS: A total of 69 patients met inclusion criteria: 32 with abducens nerve palsy and 37 with esotropic Duane syndrome. Vertical alignment changed in both hyper- and hypotropic directions. Median pre- and postoperative vertical deviation in primary gaze was 1.1Δ (10th-90th percentile, 0Δ-6Δ hypertropia) and 0.4Δ (10th-90th percentile, 6Δ hypotropia to 8 Δ hypertropia), respectively. Postoperative vertical diplopia occurred in 7%, including 4 of 49 treated with loop myopexy (8%), 1 of 13 without augmentation (8%), and 0 of 7 treated with sclera-fixated augmentation. All but one was successfully treated with prism or secondary surgery. Intorsional change predominated, but no patient had torsional diplopia post adjustment. Vertical misalignment in affected side gaze increased from 19% to 45% after SRT (P = 0.01). CONCLUSIONS: In this largest-to-date review of patients treated with SRT, with or without MR recession, no patient developed persistent torsional diplopia, while 7% developed symptomatic vertical diplopia in primary position, similar to the reported incidence after balanced vertical rectus transposition. Vertical misalignment in affected side gaze increased, however fusion is already limited by unresolved esotropia in this field.

Adjustable graded augmentation of superior rectus transposition for treatment of abducens nerve palsy and Duane syndrome.

PURPOSE: To report the results of adjustable graded augmentation of superior rectus transposition, a novel modification of superior rectus transposition (SRT) designed to reduce postoperative vertical or torsional diplopia. METHODS: The medical records of patients who underwent adjustable graded augmentation of SRT with or without adjustable medial rectus recession (MRc) from February 2017 to December 2019 were reviewed retrospectively. A Mendez ring was used to monitor torsional change after transposition of the superior rectus muscle to the lateral rectus muscle and after sequential placement of 2 or 3 augmentation sutures by superior rectus-lateral rectus loop myopexy. If excessive mechanical intorsion was induced, the responsible augmentation suture was severed intraoperatively. If torsional or vertical diplopia was noted after recovery, the distal-most augmentation suture was cut. Exotropia was managed by severing the distal-most augmentation suture or by medial rectus adjustment. RESULTS: A total of 8 patients who underwent adjustable graded augmentation of SRT were included (6 using the 3-suture technique): 3 for esotropic Duane syndrome, 2 for abducens nerve palsy, 1 for Moebius syndrome, and 2 for combined trochlear and abducens nerve palsies. Of the 8 patients, 4 had prior strabismus surgery, and 1 patient had previously undergone treatment with botulinum toxin. Severing one augmentation suture in 3 cases resolved vertical (n = 2) or torsional (n = 1) diplopia and consecutive exotropia (n = 2), resulting in excellent alignment and reduction of torticollis to ≤4° in 7 cases. The technique proved insufficient in 1 patient, who had undergone 3 prior strabismus procedures. CONCLUSIONS: In this study cohort, adjustable graded augmentation of SRT effectively managed the risk of postoperative vertical or torsional diplopia.

Surgical outcome of patients with unilateral exotropic Duane retraction syndrome.

PURPOSE: To describe the clinical features and surgical outcomes of patients with unilateral exotropic Duane retraction syndrome (DRS). METHODS: The medical records of patients with unilateral exotropic DRS who underwent surgery between March 2015 and February 2018 were reviewed retrospectively. RESULTS: A total of 40 patients (mean age, 18.75 ± 12.54 years; 21 males [53%]) were included. In 28 patients (70%) the left eye was involved. All patients had globe retraction and head turn toward the opposite side of the affected eye. Remarkable upshoot/downshoot movement was detectable in 11 patients (28%). The mean deviation for near and distance improved from 24.37Δ ± 12.34Δ (range, 6Δ-77Δ) and 19.67Δ ± 10.76Δ (range, 4Δ-60Δ) to 4.25Δ ± 8.61Δ (range, 0Δ-50Δ) and 2.62Δ ± 6.15Δ (range, 0Δ-35Δ) after the first surgery (P <0.001 for near and far deviation). Mean postoperative follow-up was 7.82 ± 9.45 months. Two patients required reoperation. Different types of surgeries, including lateral rectus recession (with or without Y-splitting), lateral and medial rectus recession (with or without Y-splitting of the lateral rectus muscle), bilateral lateral rectus recession, and lateral rectus recession with vertical rectus nasal transposition were performed. Twenty-four patients (60%) were successfully treated with only a single recession of the ipsilateral lateral rectus muscle. The mean lateral rectus recession was 7.45 ± 0.73 mm (range, 6-8.5 mm), and the mean dose-response for lateral rectus recession was 2.79Δ ± 0.64Δ/mm for near and 2.45Δ ± 0.67Δ/mm for distance. CONCLUSIONS: Simple lateral rectus recession (with or without Y-splitting), even in the presence of significant deviation (through 35Δ), seems to be a successful procedure for management of patients with unilateral exotropic DRS.

Surgical outcome of exotropic Duane syndrome.

Purpose: To evaluate the surgical outcome of exotropic Duane syndrome and the factors that can influence the outcome.Methods: A retrospective study was performed on patients who had surgery for exotropic Duane syndrome with a minimum follow-up of 3 months. The ocular motility, the angles of deviation, the presence of abnormal head turn or overshoots, the surgical procedures performed, and their outcome were analyzed.Results: Sixty-seven patients were identified (mean age, 11.3 years). In unilateral cases with exotropia <25 PD (n = 28), ipsilateral lateral rectus (LR) recession was performed in all cases, and orthophoria was achieved in 25 patients (89.3%). In unilateral cases with angle ≥25 PD, ipsilateral LR recession was performed in 13 patients, bilateral symmetrical LR recession in eight patients, and unilateral LR recession and medial rectus (MR) resection in seven patients. Sequential contralateral LR recession for undercorrection was performed in seven patients. The success rate was highest in those who had unilateral LR recession and MR resection (66.7%). An increase in face turn occurred in 6 of 15 patients (40%) who had a simultaneous or sequential bilateral LR recession. In bilateral Duane syndrome, bilateral LR recession was performed in 10 patients, and was combined with ipsilateral MR resection in two patients with a success rate of 75%. Higher success rate was associated with young age at surgery (P = .03) and smaller angle of deviation (p = .04). The mean follow-up was 6.3 months.Conclusions: Surgical results in exotropic Duane syndrome are better in small angles of deviation and in younger age groups.

Combined surgical strategy for management of unilateral exotropic Duane retraction syndrome associated with limitation of abduction.

PURPOSE: To report the effect of asymmetrical bilateral lateral rectus recession combined with augmented partial vertical rectus transposition (VRT) in the management of exotropia, head turn, limited abduction, and anomalous vertical movements associated with unilateral exotropic Duane retraction syndrome (XT-DRS). METHODS: The medical records of all patients with unilateral XT-DRS associated with limitation of abduction who underwent surgery during a 5-year period from 2013 to 2018 with at least 6 months' follow-up were reviewed retrospectively. Outcome measures were changes in head turn, primary position distance and near exodeviation, degree of limited abduction, and anomalous vertical movements on adduction. RESULTS: A total of 11 patients (6 males) were included. Mean patient age was 16.3 years (range, 6-29). Exodeviation at distance and near fixation were corrected by means of 26.4Δ and 24.8Δ, respectively. Head turn was improved by a mean of 17.3°. Limited abduction and anomalous vertical movements were corrected by means of 1.6 and 1.5 units, respectively. No patients developed symptomatic induced vertical deviation or anterior segment ischemia. CONCLUSIONS: In our study cohort, the combined strategy of asymmetrical bilateral lateral rectus recession with unilateral augmented partial VRT yielded satisfactory results in the management of unilateral XT-DRS associated with limited abduction with no recorded intra- or postoperative complications.

Oculo-Auriculo-Fronto-Nasal Syndrome With Duane Retraction Syndrome and Dysplastic Bony Structure in the Midline of Nose.

Oculo-auriculo-fronto-nasal syndrome (OAFNS) is a rare anomaly characterized by features overlapping those of frontonasal dysplasia (FND) and the oculo-auriculo-vertebral spectrum (OAVS). The FND features malformation of frontonasal process-derived structures, characterized by anomalies in the central portion of the face. The OAVS is characterized by developmental anomalies of the first and second pharyngeal arches. The OAFNS is a condition with clinical features of both FND and OAVS.Here, the authors present the case of a male with OAFNS who not only exhibited typical OAFNS symptoms but also a dysplastic bony structure that bridged the anterior nasal spine and inferior nasal bones, and unilateral type 3 Duane retraction syndrome (absence of right-eye abduction). Abnormal nasal bones are characteristic of OAFNS; such abnormalities are absent from FND and OAVS. The authors reduced the dysplastic nasal bony structure via open external rhinoplasty, followed by lateral nasal osteotomy when he was 16 years of age. The nasal dorsum appeared natural after surgery and he was satisfied with the result.