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Cureus Jun 2021Objective In this study, we aimed to investigate the prevalence of diplopia in cases with type 1 Duane retraction syndrome (DRS). Materials and methods This study was a...
Objective In this study, we aimed to investigate the prevalence of diplopia in cases with type 1 Duane retraction syndrome (DRS). Materials and methods This study was a retrospective review of cases involving patients presenting diagnosed with DRS over a period of 24 years. Among these cases, 28 had type 1 DRS and fulfilled the inclusion criteria. The cases were evaluated in terms of age, gender, affected eye, concomitant ocular motility disorders, presence of amblyopia, manifest shift, abnormal head position (AHP), fusion, and stereopsis. Results Sixteen of the patients (57.1%) in the study were female, and 12 (42.8%) were male; the mean age of the patients was 18.9 years (range: 7-67 years). The right eye was affected in six of the cases (21.4%), and the left eye in 22 (78.6%) of the cases. On examination, diplopia was not observed in 21 (75%) cases, but it was detected in seven (25%). AHP was present in five of the seven cases with diplopia and not present in two, and all seven of the diplopic cases had fusion, while three had stereopsis. The level of stereopsis in all diplopic cases was 400 sn/ark. When the clinical findings of patients with diplopia and those without diplopia were compared, a statistically significant difference was observed only in terms of AHP. Conclusions Although diplopia is not one of the clinical features of DRS, it must be noted that in cases with type 1 DRS, diplopia may occur in directions in which the movement of the eyeball is limited. In the presence of this finding, which might mimic sixth nerve palsy, patient history must be diligently taken, other clinical findings of DRS must be thoroughly examined, and an MRI should be performed when necessary for an easier diagnosis.
PubMed: 34164253
DOI: 10.7759/cureus.15769 -
Optometry and Vision Science : Official... Dec 2019The 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... (Comparative Study)
Comparative Study
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.
Topics: Adolescent; Adult; Child; Child, Preschool; Cross-Sectional Studies; Duane Retraction Syndrome; Esotropia; Exotropia; Facial Asymmetry; Female; Head; Humans; Male; Young Adult
PubMed: 31834151
DOI: 10.1097/OPX.0000000000001452 -
Korean Journal of Ophthalmology : KJO Apr 2020We 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...
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 ( = 0.003 and < 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 ( = 0.001 and < 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.
Topics: Adolescent; Child; Child, Preschool; Duane Retraction Syndrome; Eye Movements; Female; Humans; Incidence; Infant; Infant, Newborn; Male; Oculomotor Muscles; Republic of Korea; Retrospective Studies; Young Adult
PubMed: 32233150
DOI: 10.3341/kjo.2019.0100 -
Journal of Pediatric Ophthalmology and... Jan 2021To report surgical outcomes of patients with exotropic Duane retraction syndrome.
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.].
Topics: Adolescent; Adult; Child; Duane Retraction Syndrome; Female; Humans; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Treatment Outcome; Young Adult
PubMed: 33495792
DOI: 10.3928/01913913-20200910-02 -
Journal of Optometry 2024To report the visual and refractive characteristics and the prevalence of amblyopia in patients with different types of Duane's Retraction Syndrome (DRS).
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.
Topics: Humans; Amblyopia; Male; Female; Retrospective Studies; Adult; Visual Acuity; Child; Child, Preschool; Middle Aged; Young Adult; Adolescent; Aged; Iran; Duane Retraction Syndrome; Prevalence; Refraction, Ocular; Refractive Errors; Anisometropia
PubMed: 38215611
DOI: 10.1016/j.optom.2023.100508 -
Journal of AAPOS : the Official... Feb 2021To 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...
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.
Topics: Duane Retraction Syndrome; Humans; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Sclera
PubMed: 33601039
DOI: 10.1016/j.jaapos.2020.10.006 -
European Journal of Ophthalmology Mar 2021The aim was to evaluate the long-term motor outcome of superior rectus transposition procedure in patients affected by unilateral esotropic Duane retraction syndrome...
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; < 0.05), anomalous head position (5.6 ± 3.9°; < 0.05), and abduction limitation (-2.3 ± 0.8; < 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.
Topics: Adolescent; Adult; Child; Disease Progression; Duane Retraction Syndrome; Eye Movements; Female; Follow-Up Studies; Humans; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Reoperation; Retrospective Studies; Time Factors; Young Adult
PubMed: 31888377
DOI: 10.1177/1120672119897889 -
Clinical Ophthalmology (Auckland, N.Z.) 2022Abduction limitation in esotropic Duane retraction syndrome (DRS), esotropic Mobius syndrome, and sixth nerve palsy is one of the difficult-to-manage problems in... (Review)
Review
Abduction limitation in esotropic Duane retraction syndrome (DRS), esotropic Mobius syndrome, and sixth nerve palsy is one of the difficult-to-manage problems in strabismus surgery. The procedure of superior rectus transposition (SRT) was introduced by Johnston et al. In this procedure, the superior rectus (SR) muscle is disinserted and sutured adjacent to the insertion of lateral rectus (LR) muscle. The purpose of this review is to explore literature about efficacy and safety of SRT and its usage in strabismus surgery.
PubMed: 36444206
DOI: 10.2147/OPTH.S359313 -
Journal of Pediatric Ophthalmology and... May 2023Duane retraction syndrome (DRS) is a congenital cranial dysinnervation disorder that is characterized by paradoxical lateral rectus muscle innervation of the affected...
Duane retraction syndrome (DRS) is a congenital cranial dysinnervation disorder that is characterized by paradoxical lateral rectus muscle innervation of the affected eye by axons meant to innervate the ipsilateral medial rectus muscle, with resultant varying degrees of co-contraction. It is characterized by severe abduction deficiency, variable limitation of adduction, globe retraction with narrowing of the palpebral fissure, and oblique elevation or depression on adduction. A total of 16 patients with unilateral DRS were identified. The mean age was 13 ± 8 years (range: 6 to 28 years). There were 5 males and 11 females. The cohort included 8 patients with DRS type I, 3 patients with DRS type II, 4 patients with DRS type III, and 1 patient with synergistic divergence (DRS type IV). The mean width of the palpebral fissure in primary gaze was 9.95 ± 0.25 mm, increased in abduction to 11.11 ± 1.16 mm, and changed on adduction to 10.03 ± 1.19 mm. The mean reduction in the size of the palpebral fissure on adduction was 11.7 ± 10.2% (range: 0 to 30%). The difference in the palpebral fissure width between adduction and abduction was statistically significant ( = .0018). Of the 16 patients, 8 (50%) showed narrowing of the palpebral fissure of the contralateral eye on adduction compared to abduction of more than 10%. In this case series of unilateral Duane retraction syndrome, there was a common association between widening of the palpebral fissure of the unaffected eye and adduction of the eyes with DRS in DRS types II, III, and IV and DRS type I with upshoot or downshoot. .
Topics: Male; Female; Humans; Child, Preschool; Child; Adolescent; Young Adult; Adult; Duane Retraction Syndrome; Oculomotor Muscles; Eyelids; Strabismus
PubMed: 37227993
DOI: 10.3928/01913913-20230217-01 -
European Journal of Medical Genetics Apr 2021Duane retraction syndrome is a congenital eye movement disorder characterized by a failure of abducens nerve to develop normally, resulting in restriction or absence of...
Duane retraction syndrome is a congenital eye movement disorder characterized by a failure of abducens nerve to develop normally, resulting in restriction or absence of abduction, adduction, or both, and narrowing of the palpebral fissure and retraction of the globe on attempted adduction. There is a genetic heterogeneity in Duane retraction syndrome (DURS). DURS maps to chromosome 8q13 in some patients, and pathogenic variants in CHN1 and MAFB genes are known to lead to DURS. We report here a child and his father with Duane retraction syndrome, associated to swallowing difficulties and unilateral trapeze aplasia. A whole exome sequencing revealed a heterozygous missense variant in CHN1 gene. This gene encodes GTPase-activating protein and is involved in the assembly of neuronal locomotor circuits. A patient with a 8q deletion has previously been described with a Duane retraction syndrome associated to trapeze aplasia. We provide an additional description to support the role in cranial nerves development of the CHN1 gene.
Topics: Child; Chimerin 1; Cranial Nerves; Deglutition; Duane Retraction Syndrome; Humans; Male; Middle Aged; Mutation, Missense; Pedigree; Phenotype
PubMed: 33667650
DOI: 10.1016/j.ejmg.2021.104188