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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 -
Cold Spring Harbor Perspectives in... Mar 2010This article reviews symptoms and signs of aberrant axon connectivity in humans, and summarizes major human genetic disorders that result, or have been proposed to... (Review)
Review
This article reviews symptoms and signs of aberrant axon connectivity in humans, and summarizes major human genetic disorders that result, or have been proposed to result, from defective axon guidance. These include corpus callosum agenesis, L1 syndrome, Joubert syndrome and related disorders, horizontal gaze palsy with progressive scoliosis, Kallmann syndrome, albinism, congenital fibrosis of the extraocular muscles type 1, Duane retraction syndrome, and pontine tegmental cap dysplasia. Genes mutated in these disorders can encode axon growth cone ligands and receptors, downstream signaling molecules, and axon transport motors, as well as proteins without currently recognized roles in axon guidance. Advances in neuroimaging and genetic techniques have the potential to rapidly expand this field, and it is feasible that axon guidance disorders will soon be recognized as a new and significant category of human neurodevelopmental disorders.
Topics: Animals; Axons; Brain; Developmental Biology; Fibrosis; Genetic Diseases, Inborn; Humans; Kallmann Syndrome; Ligands; Models, Biological; Muscles; Mutation; Nervous System Diseases; Syndrome
PubMed: 20300212
DOI: 10.1101/cshperspect.a001784 -
Indian Journal of Ophthalmology Aug 2014
Topics: Accommodation, Ocular; Duane Retraction Syndrome; Esotropia; Eye Movements; Female; Humans; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Vision, Binocular
PubMed: 25360471
DOI: 10.4103/0301-4738.141070 -
Korean Journal of Ophthalmology : KJO Jun 2017Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in... (Review)
Review
Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in aberrant innervation of the ocular and facial musculature. Its diagnosis could be facilitated by the development of high resolution thin-section magnetic resonance imaging. The purpose of this review is to describe the method to visualize cranial nerves III, IV, and VI and to present the imaging findings of congenital cranial dysinnervation disorders including congenital oculomotor nerve palsy, congenital trochlear nerve palsy, Duane retraction syndrome, Möbius syndrome, congenital fibrosis of the extraocular muscles, synergistic divergence, and synergistic convergence.
Topics: Abducens Nerve; Cranial Nerve Diseases; Duane Retraction Syndrome; Humans; Magnetic Resonance Imaging; Oculomotor Nerve; Trochlear Nerve
PubMed: 28534340
DOI: 10.3341/kjo.2017.0024 -
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 -
Clinical Ophthalmology (Auckland, N.Z.) 2017To report the results of using prismatic glasses for Duane retraction syndrome (DRS).
PURPOSE
To report the results of using prismatic glasses for Duane retraction syndrome (DRS).
METHODS
Data were obtained from the records of patients who were evaluated during the year 2000 in the Strabismus Unit of the Beyoglu Eye Training and Research Hospital. The average follow-up was 12.2±17.7 months. In all cases, 2 main variables were evaluated: horizontal deviation in the primary position and face turn. Prismatic glasses were provided to patients according to the degree of shift in the primary position.
RESULTS
The mean age of patients was 11.2 years. An analysis was performed on the data collected from 12 cases; 7 patients were females (58.3%) and 5 were males (41.7%), with 11 (91.7%) cases being of type I DRS. All 12 patients had abnormal head posture (face turn) and an angle of mean deviation equaling 10 PD (prism dioptri).
CONCLUSION
Treatment was individualized on a case-by-case basis. Prismatic glasses are useful for eliminating abnormal head posture and ocular misalignment in selected cases.
PubMed: 28442887
DOI: 10.2147/OPTH.S124183 -
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 -
Journal of Clinical Medicine Jun 2020To investigate the morphometric characteristics of the oculomotor nerve and its association with horizontal rectus muscle volume in patients with Duane's retraction...
OBJECTIVE
To investigate the morphometric characteristics of the oculomotor nerve and its association with horizontal rectus muscle volume in patients with Duane's retraction syndrome (DRS) according to the presence of the abducens nerve.
METHODS
Fifty patients diagnosed with unilateral DRS were divided into two groups according to high-resolution magnetic resonance imaging (MRI) findings; DRS without an abducens nerve on the affected side (absent group, = 41), and DRS with symmetric abducens nerves on both sides (present group, = 9). Oculomotor nerve diameter was measured on high-resolution MRI in the middle of the cisternal space. The medial rectus muscle (MR) and lateral rectus muscle (LR) volumes were measured on T2-weighted coronal MRI of the orbit. Associations of oculomotor nerve diameter and horizontal rectus muscle volumes were performed according to the presence and absence of the abducens nerve.
RESULTS
Oculomotor nerve diameter on the affected side was thicker than that of the non-affected side in the absent group ( < 0.001), but not in the present group ( = 0.623). In the absent group, there was a positive correlation between oculomotor nerve diameter and MR volume ( = 0.779, < 0.001), as well as the LR volume ( = 668, = 0.023) of the affected eye.
CONCLUSIONS
In DRS patients with an absent abducens nerve, the oculomotor nerve diameter was thicker in the affected eye compared to the non-affected eye. Oculomotor nerve diameter was associated with MR and LR volumes in the absent group. This study provides structural correlates of aberrant innervation of the oculomotor nerve in DRS patients.
PubMed: 32599889
DOI: 10.3390/jcm9061983 -
Indian Journal of Ophthalmology Oct 2020The purpose of this study was to investigate the structural changes (axial length, central macular thickness (CMT), subfoveal choroidal thickness, and keratometry) in...
PURPOSE
The purpose of this study was to investigate the structural changes (axial length, central macular thickness (CMT), subfoveal choroidal thickness, and keratometry) in subjects with unilateral Duane retraction syndrome (DRS) as compared with the normal fellow eye.
METHODS
In this prospective study, we included 34 subjects with unilateral DRS from January 2016 to December 2016 seen at our institute. Data was collected for axial length, keratometry using partial coherence interferometry, CMT, subfoveal choroidal thickness using the enhanced depth imaging-optical coherence tomography (EDI-OCT). All these measurements were compared between the affected and fellow eye.
RESULTS
During this period, we included 34 subjects with unilateral DRS (22 Type I, 1 Type II, and 11 Type III). The mean age (±SD) of subjects was 14 ± 8 years (range: 5-28 years). There were 15 males and 19 females. Eyes with DRS were significantly shorter (median axial length 22.4 mm, interquartile range (IQR): 21.56 - 23.17) as compared to fellow eye (median axial length 22.7 mm, IQR: 22.35-23.55), P = 0.04. Choroidal thickness, CMT, and average keratometry were similar in DRS and fellow eyes (P = 0.39, 0.06, and 0.11, respectively). A significant difference in axial length was found only between Type I and Type III DRS (P = 0.03).
CONCLUSION
This study suggests that in subjects with DRS, the affected eye has shorter median axial length when compared with the fellow eye. Prevalence of refractive error in eye with DRS was higher compared to fellow eye. But, there was no difference in magnitude of refractive error found between eye with DRS and normal fellow eye.
Topics: Adolescent; Adult; Axial Length, Eye; Child; Child, Preschool; Choroid; Duane Retraction Syndrome; Female; Humans; Male; Prospective Studies; Tomography, Optical Coherence; Visual Acuity; Young Adult
PubMed: 32971639
DOI: 10.4103/ijo.IJO_123_20 -
BMC Ophthalmology May 2017Duane retraction syndrome (DRS) consists of abduction deficit, globe retraction and upshoots or downshoots with adduction. The abducens nerve on the affected side is...
BACKGROUND
Duane retraction syndrome (DRS) consists of abduction deficit, globe retraction and upshoots or downshoots with adduction. The abducens nerve on the affected side is absent in type 1 DRS. After bilateral medial rectus muscle recession in unilateral type 1 DRS may improve the abduction limitation, but still more than -3 limitation remains.
CASE PRESENTATION
A 6-month-old boy presented with esotropia which had been noticed in early infancy. He showed limited abduction, fissure narrowing on attempted adduction and a small upshoot OS. Left abducens nerve was not identified on magnetic resonance imaging compatible with Duane retraction syndrome type 1. He showed full abduction after bilateral medial rectus recession of 6.0 mm at the age of 9 months, and remained orthotropia with full abduction OU 2 years postoperatively. He is my only patient with Duane retraction syndrome who showed full abduction after bilateral medial rectus recession.
CONCLUSIONS
A patient with the type 1 Duane retraction syndrome rarely may show full abduction after bilateral medial rectus recession mimicking infantile esotropia.
Topics: Abducens Nerve; Duane Retraction Syndrome; Esotropia; Eye Movements; Humans; Infant; Magnetic Resonance Imaging; Male; Oculomotor Muscles; Postoperative Period; Vision, Binocular
PubMed: 28526001
DOI: 10.1186/s12886-017-0475-6