Special clinical features with a novel mutation site of CHN1 gene in a Chinese family with Duane retraction syndrome.

PURPOSE: This study is to describe the special clinical and genotypic features of a Chinese family with variant types of Duane retraction syndrome and to present our experience on managing these cases. METHODS: Four individuals from one family were reviewed by ophthalmologic examinations, in which two affected and two unaffected individuals were revealed. MRI scans were performed on the two patients. Relevant gene mutations were screened by the next-generation sequencing technology and confirmed by Sanger sequencing technology. RESULTS: The six-year-old proband presented with special clinical features of severe horizontal gaze dysfunction, exotropia and mild scoliosis. His mother showed significantly limited binocular abductions, with retraction of eyeballs in adduction. From MRI scans, abducens nerves were not observed in both patients and the oculomotor nerve was slightly thin in the proband. The proband and his mother shared the same CHN1 gene mutation site (c. 62A>G; p.Y21C). Strabismus surgery was performed on the proband to correct the primary gaze exotropia.(NM_001822: exon3 or NM_001025201: exon4: c. 62A>G; p.Y21C). CONCLUSIONS: A novel CHN1 gene mutation was revealed from a Chinese family with Duane retraction syndrome. Remarkably, the proband and his mother presented different clinical features of ocular motility disorder. Strabismus correction surgery and amblyopia training helped to improve the appearance and visual function of the proband.

Refractive features and amblyopia in Duane's Retraction Syndrome: A review of the 582 patients.

PURPOSE: To report the visual and refractive characteristics and the prevalence of amblyopia in patients with different types of Duane's Retraction Syndrome (DRS). METHOD: This retrospective study was performed on hospital records of 582 DRS patients at Farabi Hospital, Iran, from 2012 to March 2022. RESULTS: The mean age of patients was 19.4 ± 11.9 (range, 3-70) years [335 (57.6 %) females and 247 (42.4 %) males (P < .001)]. DRS type I, II, III, and IV were presented in 347 (59.6 %), 148 (25.4 %), 82 (14.1 %), and 5 (0.9 %) patients, respectively. There were 530 (91.1 %) patients with unilateral and 52 (8.9 %) with bilateral involvement. In the unilateral patients, the DRS eyes' corrected distance visual acuity (CDVA) and astigmatism were significantly worse than the Non-DRS Eyes (P < .001). The mean amount of all refractive and visual parameters in bilateral patients' right or left eyes was significantly lower than in unilateral patients' non-DRS eyes (all P < .05). Anisometropia was observed in 75(12.9 %) of the patients. Amblyopia was observed in 18.5 % (98 patients) and 36.5 % (19 patients) of unilateral and bilateral DRS patients, respectively (P < .001). In unilateral patients, amblyopia was found in 57 (16.4 %) patients with Type I, 22 (14.9 %) patients with Type II, 16 (19.5 %) patients with Type III, and 3 (60 %) patients with Type IV. Forty-four (37.6 %) of patients with amblyopia had anisometropia. CONCLUSION: This large-scale study indicates that DRS types differ in terms of refractive error, visual acuity, and the prevalence of amblyopia and anisometropia. Clinicians should be aware of the clinical features associated with different types of DRS.

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.

Superior Rectus Transposition With Medial Rectus Recession Versus Medial Rectus Recession in Esotropic Duane Retraction Syndrome.

PURPOSE: To compare the results of augmented superior rectus transposition (with or without medial rectus recession) with simple medial rectus recession in the treatment of patients with esotropic Duane retraction syndrome. METHODS: This was a prospective, randomized, and interventional comparative study of 20 patients with esotropic type 1 Duane retraction syndrome. Patients were randomly divided into two groups. In the first group (superior rectus transposition group), superior rectus transposition with or without medial rectus recession was performed. In the second group (non-superior rectus transposition group), unilateral medial rectus recession was performed. RESULTS: Each group included 10 patients. Esotropia improved from 20.4 prism diopters (PD) preoperatively to 0.6 PD postoperatively in the superior rectus transposition group and from 22.5 PD preoperatively to 0.9 PD postoperatively in the non-superior rectus transposition group. Face turn improved from 11.5° preoperatively to 0.5° postoperatively in the superior rectus transposition group and from 12° preoperatively to 1.5° postoperatively in the non-superior rectus transposition group. Abduction improved in the superior rectus transposition group from -3.9 preoperatively to -3.1 postoperatively and from -3.9 preoperatively to -3.6 postoperatively in the non-superior rectus transposition group. Vertical deviation developed in two cases in the superior rectus transposition group. CONCLUSIONS: Both superior rectus transposition and medial rectus recession are effective in the elimination of esotropia and face turn in patients with esotropic Duane retraction syndrome. Superior rectus transposition is more effective in improving abduction, but it can be complicated by vertical deviation. [J Pediatr Ophthalmol Strabismus. 2020;57(5):309-318.].

Clinical Features of Duane Retraction Syndrome: A New Classification.

PURPOSE: We sought to provide a new classification system for Duane retraction syndrome (DRS) according to type and angle of strabismus during primary gaze and to analyze the clinical features of each DRS type. METHODS: The medical records of 65 DRS patients who visited the department of pediatric ophthalmology at Seoul National University Children's Hospital between 2010 and 2017 were retrospectively analyzed. Patients whose angle of exotropia at primary gaze exceeded 3 prism diopters (PDs) were classified as "Exo-Duane," those whose angle of strabismus at primary gaze did not exceed 3 PDs were classified as "Ortho-Duane," and those whose angle of esotropia at primary gaze exceeded 3 PDs were classified as "Eso-Duane." RESULTS: Among 65 DRS patients, Ortho-Duane was the most common (53.8%) type, followed by Eso-Duane (33.8%) and Exo-Duane (12.3%). The mean age at diagnosis was significantly higher in the Exo-Duane group than the Ortho-Duane or Eso-Duane group (p = 0.003 and p < 0.001, respectively). A predominance of left eye involvement was observed in the Ortho-Duane (62.9%) and Eso-Duane (90.9%) groups. The frequencies of upshoot, downshoot, fissure narrowing, and globe retraction were not significantly different among the subgroups. Head-turn was more frequent in Eso-Duane patients than in Exo-Duane or Ortho-Duane patients (p = 0.001 and p < 0.001, respectively). Myopia accounted for the most common refractive error among Exo-Duane patients (71.4%), while hyperopia was found more often in both Ortho-Duane (64.7%) and Eso-Duane (85.0%) patients. The majority of patients showed gross stereoacuity (93.1%), and a large proportion had good stereoacuity (Exo-Duane 60.0%, Ortho-Duane 81.3%, Eso-Duane 87.5%). CONCLUSIONS: Our newly proposed classification of DRS according to type and angle of strabismus at primary gaze was practically useful and showed potential for use as an objective guideline in the clinical setting.

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.

Augmented medial transposition of split lateral rectus in the management of synergistic divergence.

Synergistic divergence is a congenital disorder with abnormal ocular motility. Various management options have been described but with few satisfactory outcomes. We describe 3 cases of synergistic divergence a technique of medial transposition of split lateral rectus augmented with equatorial fixation sutures in 3 children. Postoperatively the synergistic divergence disappeared, and eyes were aligned in the straight-ahead gaze with improved adduction and convergence in all 3 cases.

Facial Asymmetry in Unilateral Duane Retraction Syndrome.

SIGNIFICANCE: The overall objective of this study was to evaluate facial asymmetry in patients with unilateral Duane retraction syndrome (DRS). The results showed a high frequency of facial asymmetry parameters of the opposite side of head turn in unilateral DRS patients. PURPOSE: The purposes of this study were to evaluate the characteristics of facial asymmetry in unilateral exotropic and esotropic DRS and to compare the findings with orthotropic subjects. METHODS: This cross-sectional comparative case series study was performed in 44 consecutive patients with head turn caused by DRS and 44 orthotropic subjects from 2016 to 2019. Four pictures were taken from the patients' faces. The first and second pictures were taken when patients had head turn and when the head was completely straight for calculating the facial angle and relative facial size, respectively. The third and fourth pictures were taken when the head was positioned downward (to compare the size of the cheek) and upward (to evaluate nose asymmetry). RESULTS: The mean ± SD age of DRS patients and orthotropic subjects was 16.23 ± 9.92 and 20.68 ± 11.82 years, respectively. The frequency of facial asymmetry and all facial parameters (cheek compression, nasal tip and columella deviation, and compression of one of the nostrils) was significantly higher in DRS patients compared with orthotropic subjects (P < .001). In DRS patients with facial asymmetry, columella and nasal tip deviation (P = .006) and cheek and face compression (P = .03) were significantly more prevalent in the opposite direction of head turn. In the DRS group, the mean ± SD age of the patients with and without facial asymmetry was 17.37 ± 9.76 and 7.40 ± 6.54 years, respectively (P = .02). CONCLUSIONS: The frequency of facial asymmetry and all facial parameters was significantly higher in DRS patients compared with orthotropic subjects. In unilateral DRS patients, the face was more commonly affected on the opposite side of head turn.

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.

A modified technique for attaching the lateral rectus muscle to the orbital periosteum through a skin incision over the lateral orbital rim.

BACKGROUND: The traditional approach for periosteal fixation of the lateral rectus muscle involves securing the muscle using nonabsorbable sutures by exposing the orbital periosteum 5 mm to the inside of the orbital margin. We present a simplified approach that exposes the orbital periosteum through a skin incision, providing more room and avoiding extraconal fat manipulation. METHODS: We used our technique to expose the lateral orbital periosteum and suture the lateral rectus muscle to the periosteum using nonabsorbable sutures in 2 patients with exotropic Duane retraction syndrome and 1 patient with congenital oculomotor nerve palsy. RESULTS: All 3 patients had satisfactory postoperative alignment, with abduction limitation of 3-. Adduction improved in all patients. The patient with oculomotor nerve palsy had a small overcorrection in primary position that remained stable during follow-up of 18 months. There were no intraoperative complications. CONCLUSIONS: This modified approach to extraocular muscle periosteal fixation may be simpler than the standard approach. Further evaluation in a larger series of patients is warranted.

Binocular Function in Subjects with Orthotropic Duane Retraction Syndrome.

Introduction and Purpose: The aim of this study is to evaluate binocular vision parameters such as near point of accommodation (NPA), near point of convergence (NPC), fusional vergence ranges in subjects with orthotropic Duane retraction syndrome (DRS) and to compare with normal age-matched controls Patients and Methods: A total of 24 subjects (14 Type 1, 2 Type 2, 8 Type 3 DRS) who were diagnosed to have DRS and 15 normals were included in the study. The following binocular vision parameters were assessed in all subjects: amount of the deviation (for near and distance in all gazes), NPC, NPA, positive and negative fusional vergence (PFV and NFV) reserve and stereopsis. Results: Accommodative amplitude was normal in subject with DRS (median (95% confidence interval (CI):15D (11.9-18.1)) compared to age-matched controls. However, NPC was significantly receded (median [95% CI]:17 cm [14.4-19.6]). PFV reserve and NFV reserve were reduced in subjects with DRS. Subjects with Type 3 DRS have poor PFV reserve and NFV reserve for distance when compared to Type 1 DRS (p = 0.009 and 0.006 respectively). Stereopsis was poor in the subjects with DRS (p < 0.001). Conclusion: Subjects with DRS have good accommodation, but have poor convergence and fusional vergence. Stereopsis was noted to be poor as well.

Increased restriction from an accessory lateral rectus in exotropic Duane syndrome.

Supernumerary extraocular muscles can cause restrictive strabismus, unusual ocular movements, and a persistent positive forced duction test. Even among patients with clinically typical strabismus, intraoperative testing and surgical exploration may reveal the presence of supernumerary extraocular muscles. We report the case of a patient with exotropic Duane syndrome found intraoperatively to have an accessory lateral rectus muscle, with histopathologically confirmed striated fibers.

Duane Retraction Syndrome: Clinical Features and a Case Group-Specific Surgical Approach.

PURPOSE: To describe the clinical features of patients with Duane retraction syndrome (DRS) and evaluate the outcomes of surgical approaches based on the characteristics of each patient. METHODS: The records of 38 Caucasian subjects with DRS were retrospectively reviewed. The patients were classified as type I, II, or III based on the Huber Classification. Ten patients underwent unilateral medial rectus (MR) recession due to abnormal head posture (AHP) and/or esotropia. Four patients underwent Y-splitting and recession of the lateral rectus (LR) with MR recession due to AHP and/or esotropia, upshoot, and globe retraction. RESULTS: There was a preponderance of unilaterality, female gender, left eye, type I, orthotropia, upshoot, and low refractive error. All patients demonstrated globe retraction and fissure narrowing. AHP was only present in unilateral cases. Nine patients had amblyopia. More than half of the patients over 5 years of age had decreased stereopsis. MR recession decreased AHP to less than 8° in all patients. Y-splitting and recession of the LR eliminated upshoot in all four patients. One patient who underwent an 8-mm MR recession demonstrated -2 adduction limitation. CONCLUSIONS: The DRS patients in our study demonstrated features that are consistent with previous reports in the literature. This study emphasizes the need to consider disease classification in the surgical management of DRS patients.

Adhesion after Y-split procedure can affect its mechanism for treating overshoots in Duane's syndrome.

PURPOSE: To investigate the extent of adhesion and changes in the Y configuration after the Y-split procedure, compared with the posterior fixation suture. METHODS: Twelve New Zealand white rabbits were included in the study. The 10-mm Y-split procedure was performed in the superior rectus muscle (SR) of one eye, and the 10-mm posterior fixation suture was made in the SR of the other eye. Six weeks after surgery, the Y arm lengths and lengths of adherence to the sclera were measured. If the adhesion involved the whole Y arm, the distance between the original SR insertion and most proximal part of the adhered SR was measured. In the eyes with posterior fixation suture, the distance between the SR insertion and most proximal part of the adhered SR was evaluated. RESULTS: The average nasal and temporal Y arm lengths were 6.37 ± 0.65 and 6.54 ± 0.63 mm, respectively, a significant decrease from those measured immediately after surgery (P = 0.002 and 0.002, respectively). Adhesions involved the entire Y arms in 11 of 12 SRs (91.7%), with an average adhesion length of 7.01 ± 1.04 mm. In SRs with posterior fixation sutures, the average adhesion was 9.18 ± 0.62 mm from the insertion, which was only 2.17 mm posterior to proximal portion of adhesion in Y-split SR (P < 0.001). CONCLUSIONS: Healing process reduces the Y arm length. Adhesion may involve the entire Y arm and could weaken or alter the therapeutic mechanism after the Y-split procedure.

Management of Duane retraction syndrome: A simplified approach.

Duane retraction (or co-contraction) syndrome is a congenital restrictive strabismus which can occur either as an isolated entity or in conjunction with other congenital anomalies and is now listed as a congenital cranial dysinnervation disorder. It is characterized by co-contraction of horizontal recti on attempted adduction causing globe retraction along with variable amounts of upshoots or downshoots. It may have limited abduction or adduction or both and present as esotropic, exotropic, or orthotropic Duane. The diagnosis of this disease is usually clinical. However, recent research has provided a greater insight into the genetic basis of this disease paving a way for a greater role of genetics in the diagnosis and management. This disease can have a varied presentation and hence the treatment plan should be tailor-made for every patient. The indications for surgery are abnormal head posture, deviations in the primary position, retraction and narrowing of palpebral aperture and up- or downshoots during adduction, and sometimes also to improve abduction. The arrival of newer surgical techniques of periosteal fixation (PF) of lateral rectus (LR), partial vertical rectus transposition, or superior or inferior rectus transposition in addition to LR recession with Y-split has vastly improved the management outcomes, providing not only primary position orthophoria but also increased binocular visual fields as well.

Lateral rectus muscle recession for intermittent exotropia with anomalous head position in type 1 Duane's retraction syndrome.

BACKGROUND: We questioned how to treat for intermittent exotropia in type 1 Duane's retraction syndrome (DRS). To avoid secondary abduction deficit and late overcorrection on the affected eye following ipsilateral lateral rectus (LR) recession, we performed less correction of the lateral rectus (LR) recession to correct exodeviation and anomalous head position (AHP). We report the surgical outcomes of LR recession in patients with unilateral type 1 DRS. METHODS: Four patients who underwent less correction of LR recession in the affected eye to correct intermittent exotropia and AHP to the contralateral side in type 1 DRS were enrolled. Data on preoperative and postoperative angle of exodeviation, degree of AHP, ocular motility, global retraction, palpebral fissure change, and complications were retrospectively obtained. Success was defined as postoperative deviation within 8 prism diopters (PD) and AHP < 5°. RESULTS: The preoperative angles of exodeviation and AHP were significantly improved after LR recession. The median grade of abduction limitation was improved from - 1.3 to - 0.8 postoperatively. Final median value of deviation was orthotropia in the primary position of the eye with the normal motility. All patients had successful outcomes without overcorrection or further abduction limitation in DRS eyes. CONCLUSIONS: Less correction of ipsilateral LR recession may be useful for correcting intermittent exotropia and AHP in patients with type 1 DRS.

Augmented superior rectus transposition procedure in Duane retraction syndrome compared with sixth nerve palsy.

PURPOSE: Superior rectus transposition (SRT) with medial rectus recession has been used for the treatment of sixth nerve palsy and esotropic Duane retraction syndrome (DRS). The purpose of this study was to compare the results of augmented SRT (with scleral fixation) without medial rectus recession in DRS and sixth nerve palsy. METHODS: Patients with unilateral esotropic DRS (DRS group) and sixth nerve palsy were included in this prospective, comparative study and underwent SRT. Preoperative forced duction testing was negative or slightly positive in both groups. Prospective measurements were compared between the two groups. RESULTS: There were 11 patients in the DRS group and 11 patients in the sixth nerve palsy group. The mean preoperative esotropia decreased from 20.9 ± 6.0 prism diopter (PD) at far to 13.2 ± 5.8 PD in the DRS group (P = 0.003). The same measurement improved from 28.0 ± 8.5 PD to 8.4 ± 7.3 PD in the sixth nerve palsy group (P = 0.003). In the sixth nerve palsy group, the improvement in primary gaze esotropia and abnormal head posture was more than the DRS group (Both P < 0.001).The average dose effect for SRT was 7.8 ± 2.2 PD in the DRS group and 19.2 ± 4.6 PD in the sixth nerve palsy group. Although objective intorsion was significantly induced after SRT, subjective torsion was not significant after surgery in both groups. CONCLUSION: SRT appears to be more effective in improving primary gaze deviation and head posture in sixth nerve palsy compared with DRS. Subjective torsional and vertical diplopia were rare in both groups.

Surgical outcome of superior rectus transposition in esotropic Duane syndrome and abducens nerve palsy.

PURPOSE: To evaluate surgical outcome of superior rectus transposition (SRT) in esotropic Duane syndrome (DS) and abducens nerve palsy. METHODS: Retrospective medical record analysis of all patients with esotropic DS and abducens nerve palsy treated with SRT at our center with minimum follow-up of 6 months. Primary outcome measures were esotropia in primary position and abduction limitation. Secondary outcome measures included head turn, stereopsis, and cyclovertical deviations. RESULTS: A total of 20 eyes of 19 patients were included: 9 with DS and 10 with traumatic abducens nerve palsy. One patient had bilateral esotropic DS. Mean age of DS patients was 12.5 ± 10.1 years; of abducens nerve palsy patients, 25.4 ± 11.3 years. Medial rectus recession (MRc) of 3.5 mm was additionally performed in 5 DS eyes. An adjustable MRc 5.6 ± 2.2 mm with or without augmentation suture was performed in all abducens nerve palsy patients. In DS patients, esotropia improved from 27.5Δ ± 5.4Δ to 3.6Δ ± 6.4Δ (P < 0.001), abduction limitation reduced from -3.8 to -1.8 (P < 0.001), and head posture improved from 20° to 4° (P < 0.001) at 6 months. In abducens nerve palsy patients, esotropia improved from 51.5Δ ± 18.8Δ to 6.1Δ ± 10.7Δ (P < 0.001), abduction limitation reduced from -3.8 to -2, and head posture improved from 25° to 8° (P < 0.001). Stereopsis improved in 4 patients (P = 0.12). No patient had vertical deviation or torsional diplopia. CONCLUSIONS: In our patient cohort with esotropic DS or abducens nerve palsy, SRT reduced esotropia and improved abduction. Because of a long-term exotropic drift, initial undercorrection in the immediate postoperative period may prevent eventual overcorrection.

The Efficacy of Bilateral Lateral Rectus Recession According to Secondary Deviation Measurements in Unilateral Exotropic Duane Retraction Syndrome.

PURPOSE: To evaluate the surgical results of asymmetric bilateral lateral rectus recession in exotropic Duane retraction syndrome with abnormal face turn toward the opposite side according to secondary deviation measurements. METHODS: Retrospective chart review. RESULTS: Seven cases of unilateral exotropic Duane retraction syndrome were reviewed. All cases had globe retraction on adduction and exotropia with limited adduction, five of which also had mild limitation of abduction. Four cases had upshoot/downshoot on adduction and all patients had face turn. Exotropia was measured in forced primary position. The average lateral rectus recession was 6.36 mm (range: 5.5 to 7.5 mm) in the affected eye and 7.36 mm (range: 6.5 to 8.5 mm) in the healthy eye. The mean follow-up period was 282 days. Mean exotropia in the forced primary position improved from 27.9 ± 5.7 prism diopters (PD) preoperatively to 7.9 ± 16.8 PD postoperatively (P = .025). Head position resolved completely in all but one case (P =.031). There were no significant changes in ductions. CONCLUSIONS: The results suggest that asymmetric bilateral lateral rectus recession in exotropic Duane retraction syndrome with abnormal head turn posture successfully eliminates abnormal head turn posture and exotropia in most cases. [J Pediatr Ophthalmol Strabismus. 2018;55(1):47-52.].

Bilateral Superior Rectus Transposition With Bilateral Medial Rectus Recession for Möbius Syndrome.

Bilateral superior rectus transposition with bilateral medial rectus recession has been described successfully for treatment of Duane's syndrome but never for Möbius syndrome. The authors describe a child with Möbius syndrome who presented with large bilateral abduction deficits and esotropia. This surgical treatment resulted in marked improvement of said deficits. [J Pediatr Ophthalmol Strabismus. 2017;54:e81-e82.].