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Turkish Journal of Ophthalmology Feb 2023Duane syndrome (DS) is typically characterized by abduction and/or adduction deficiency accompanied by eyelid and ocular motility disturbances. Maldevelopment or absence...
OBJECTIVES
Duane syndrome (DS) is typically characterized by abduction and/or adduction deficiency accompanied by eyelid and ocular motility disturbances. Maldevelopment or absence of the sixth nerve has been shown to be the causative factor. The aim of the present study was to investigate static and dynamic pupillary characteristics in patients with DS and compare the results with those of healthy eyes.
MATERIALS AND METHODS
Patients with unilateral isolated DS and no history of ocular surgery were enrolled in the study. Healthy subjects with a best corrected visual acuity (BCVA) of 1.0 or higher were assigned to the control group. All subjects underwent complete ophthalmological examination and pupillometry measurements (MonPack One, Vision Monitor System, Metrovision, Perenchies, France) including static and dynamic pupil evaluation.
RESULTS
A total of 74 patients (22 with DS and 52 healthy subjects) were included in the study. The mean age of the DS patients and healthy subjects was 11.05±5.19 and 12.54±4.05 years, respectively (p=0.188). There was no difference in sex distribution (p=0.502). Mean BCVA differed significantly between eyes with DS and healthy eyes, and between healthy eyes and the fellow eyes of DS patients (p<0.05). No significant difference was found in any static or dynamic pupillometry parameters (p>0.05 for all).
CONCLUSION
In the light of the results of the present study, the pupil seems to be not involved in DS. Larger studies including more patients with different types of DS in different age groups or comprising patients with non-isolated DS may reveal different findings.
Topics: Humans; Child, Preschool; Child; Adolescent; Duane Retraction Syndrome; Pupil; Eyelids; Eye Movements; Healthy Volunteers
PubMed: 36847629
DOI: 10.4274/tjo.galenos.2022.26460 -
Eye (London, England) Jun 2021To 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...
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.
Topics: Duane Retraction Syndrome; Esotropia; Humans; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Vision, Binocular
PubMed: 32839562
DOI: 10.1038/s41433-020-1118-3 -
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 -
Oman Journal of Ophthalmology 2022
PubMed: 35937726
DOI: 10.4103/ojo.ojo_141_22 -
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 -
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 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 -
Archivos Argentinos de Pediatria Jun 2023Wildervanck syndrome (also known as cervico-oculo-acoustic dysplasia) is a very rare disease, characterized by the typical triad of cervical vertebral fusion or...
Wildervanck syndrome (also known as cervico-oculo-acoustic dysplasia) is a very rare disease, characterized by the typical triad of cervical vertebral fusion or Klippel-Feil anomaly, Duane syndrome (paresis of the sixth cranial nerve), and hearing loss. Other vascular, cardiac, and musculoskeletal conditions have also been described. In this case report, we describe a patient who met the cardinal triad and also presented additional clinical data that have not been previously reported, which contribute to broadening the disease phenotype. We have also reviewed the bibliography related to this syndrome.
Topics: Humans; Klippel-Feil Syndrome; Duane Retraction Syndrome; Deafness; Abnormalities, Multiple
PubMed: 36413195
DOI: 10.5546/aap.2022-02624.eng -
Saudi Journal of Ophthalmology :... 2019To assess the clinical characteristics of Duane Retraction Syndrome (DRS) in Al-Medina region, in patients attending a pediatric ophthalmology clinic at Ohud Hospital.
PURPOSE
To assess the clinical characteristics of Duane Retraction Syndrome (DRS) in Al-Medina region, in patients attending a pediatric ophthalmology clinic at Ohud Hospital.
METHOD
A cross-sectional observational study was conducted from October 2017 to June 2018 at Ohud Hospital, Al-Medina region, Saudi Arabia. Data was collected using a sheet containing eighteen simple items which include demographic data, family history, surgical history, the clinical characteristics of the disease and the physician's treatment plan. Twenty patients were included in this study.
RESULTS
All twenty patients included in the study were diagnosed with DRS type I. Male and female were symmetrical in numbers as both 10 (50.0%) respectively. The mean age of the subjects was 8.7 years (SD 9.6). 95% of the subject were unilaterally affected while five percent of them were affected both sides of the eyes. 75% of the patients were affected on the left eye while 20% were affected on the right side. 35% of the patients had amblyopia in the affected eye. 25% of the patients had an abnormal head position. Abnormal eye movement (upshoot and downshoot) more common when the right eye is affected (-0.035). 75% of the patients were orthotropic while 30% had esotropia. 75% of patients were positive of parents' consanguinity. 16 patients (80.0%) were from Al-Medina city while four of them (20.0%) were from the different area of Al-Medina region (Al Hejeria, Al Henakia, Omluj, Yatmah).
CONCLUSION
This cross-sectional observational study is the first one that evaluates DRS in Al-Medina region. Associated abnormal eye movement more common when the right eye is affected. Parents' consanguinity may play a role in the occurrence of DRS as it is not considered as a rare disease in our region. Therefore, more studies are needed to establish the relationship between the occurrence of DRS and parents' consanguinity in our region. Also, further studies are needed to establish the relationship between abnormal eye movement and the involvement of the right eye.
PubMed: 31920443
DOI: 10.1016/j.sjopt.2019.07.007 -
Journal of Neuro-ophthalmology : the... Mar 2024Duane retraction syndrome (DRS) is known to relate to the absence of the abducens nucleus, with abnormal innervation of the lateral rectus (LR) muscle by branchesof the...
BACKGROUND
Duane retraction syndrome (DRS) is known to relate to the absence of the abducens nucleus, with abnormal innervation of the lateral rectus (LR) muscle by branchesof the oculomotor nerve (CN III). The purposes of this study were to investigate the morphological characteristics of the oculomotor nerve (CN III), the abducens nerve (CN VI), and the extraocular muscles in patients with clinically diagnosed Duane retraction syndrome (DRS) using MRI. In addition, we assessed the association between ocular motility, horizontal rectus muscle volumes, and CN III/VI in patients with Duane retraction syndrome (DRS).
METHODS
The study comprised 20 orthotropic control subjects (40 eyes) and 42 patients with Duane syndrome (48 eyes), including 20 patients with DRS Type I (24 eyes), 5 patients with DRS Type II (6 eyes), and 17 patients with DRS Type III (18 eyes). Three-dimensional (3D) T1/2 images of the brainstem and orbit were obtained to visualize the cranial nerves, especially the abducens (VI) and oculomotor (III) nerves, as well as extraocular muscles.
RESULTS
Based on the clinical classification, among 42 patients, MRI showed that the abducens nerves (CN VI) on the affected side were absent in 24 of 24 eyes (100%; 20 patients) with Type I DRS and in 16 of 18 eyes (88%; 16 patients) with Type III DRS. However, CN VI was observed in 6 of 6 eyes (100%; 5 patients) with Type II DRS and in 2 of 18 eyes (11%) with Type III DRS. CN III was observed in all patients. The oculomotor nerves on the affected side were thicker than those on the nonaffected contralateral side in DRS Type I ( P < 0.05) and Type III ( P < 0.05), but not in DRS Type II. Smaller LR and larger MR volumes were shown in the affected eye than that in the nonaffected eye in DRS Types I and III. Based on the presence or absence of CN VI, there was a tendency for thicker oculomotor nerves in the affected eye than in the nonaffected eye in the absence groups ( P < 0.05). However, no significant difference was found in the present group. In the CN VI absence groups, similar results were found in the affected eyes than in the nonaffected eyes as in DRS Types I and III. In addition, the presence of CN VI was correlated with better abduction ( P = 0.008). The LR and MR volumes have positive correlations with the oculomotor nerve diameter in the affected eye. However, there was no correlation between the range of adduction/abduction and the LR/MR ratio in patients with or without an abducens nerve.
CONCLUSIONS
Different types of DRS have different characteristic appearances of CN VI and CN III on MRI. Horizontal rectus muscles have morphological changes to adapt to dysinnervation of CN VI and aberrant innervation of CN III. Thus, these neuroimaging findings may provide a new diagnostic criterion for the classification of DRS, improving the comprehension of the physiopathogenics of this disease.
Topics: Humans; Duane Retraction Syndrome; Abducens Nerve; Oculomotor Muscles; Orbit; Magnetic Resonance Imaging
PubMed: 37682628
DOI: 10.1097/WNO.0000000000001909