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Journal of Research in Medical Sciences... 2020Duane's retraction syndrome is a congenital eye movement anomaly with narrowing of the palpebral fissure and globe retraction on attempted adduction. There are several...
Unilateral medial rectus muscle recession combined lateral rectus muscle marginal myotomy for the treatment of Duane's retraction syndrome: A promising surgical procedure.
BACKGROUND
Duane's retraction syndrome is a congenital eye movement anomaly with narrowing of the palpebral fissure and globe retraction on attempted adduction. There are several surgical approaches to treat the narrowing of the palpebral fissure. The purpose of the present study was to evaluate the efficacy of unilateral medial rectus recession (MRR) muscle combined lateral rectus (LR) muscle marginal myotomy (MM) with unilateral MRR alone in the management of narrowing of the palpebral fissure of patients with Type 1 Duane's retraction syndrome (DRS).
MATERIALS AND METHODS
Twenty-eight patients with unilateral DRS Type 1 were randomly divided into two groups (14 eyes of 14 patients in each group). Age ≥5 years with DRS Type 1 with <20 prism diopters in primary position who were candidates for surgery were consecutively enrolled in this randomized controlled trial. Patients were divided into treatment groups to receive unilateral MR recession with simultaneous MM group or with unilateral MR recession alone. The amount of deviation in primary position, abnormal head position, palpebral fissure width (PFW), and up/down shoot was evaluated before and 3 months after the surgery. This study was registered at the Iranian Registry of Clinical Trials under the registration code IRCT20131229015975N3.
RESULTS
PFW increased within MRR/MM group at the end of the study (8.86 ± 1.51) compared with the baseline (7.79 ± 1.48) ( < 0.001). In contrast, in the MRR/MM group, PFW did not increase statistically significantly within the MRR group at the end of the study (8.14 ± 1.35) compared with the baseline (8.07 ± 1.38) ( = 0.67). Mean ± standard deviation of PFW (mm) in MRR/MM group after surgery (8.86 ± 1.51) was statistically significantly higher than that in the MRR group (8.14 ± 1.35), ( = 0.002).
CONCLUSION
The results of our study demonstrate PFW significantly increased after unilateral MRR muscle combined LR muscle MM.
PubMed: 32765624
DOI: 10.4103/jrms.JRMS_836_19 -
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 Jul 2020A 10-year-old boy with unilateral cryptorchidism and renal aplasia displayed features of unilateral congenital pupil sparing third cranial nerve palsy with exotropia...
A 10-year-old boy with unilateral cryptorchidism and renal aplasia displayed features of unilateral congenital pupil sparing third cranial nerve palsy with exotropia manifesting novel dysinnervation encompassing synergistic divergence with upshoot, convergence on attempted upgaze, gaze-evoked phasic conjugate torsion, and gaze-evoked nystagmus. Congenital third nucleus/nerve hypoplasia with secondary dysinnervation is classfied as congenital cranial dysinnervation disorder (CCDD). It is speculated that miswiring between prenuclear structures, otolithic pathways, interstitial nucleus of Cajal (INC), nucleus prepositus hypoglossi, and third and sixth nerve nuclei likely resulted in this novel dysinnervation. Cryptorchidism and renal aplasia if seen may point towards an overlapping phenotype with Duane-radial ray syndrome and acro-renal-ocular/IVIC syndromes.
Topics: Child; Duane Retraction Syndrome; Humans; Male; Oculomotor Muscles; Oculomotor Nerve; Oculomotor Nerve Diseases; Paralysis
PubMed: 32587205
DOI: 10.4103/ijo.IJO_1627_19 -
Journal of AAPOS : the Official... Jun 2020To describe the clinical features and surgical outcomes of patients with unilateral exotropic Duane retraction syndrome (DRS).
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 24.37 ± 12.34 (range, 6-77) and 19.67 ± 10.76 (range, 4-60) to 4.25 ± 8.61 (range, 0-50) and 2.62 ± 6.15 (range, 0-35) 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 2.79 ± 0.64/mm for near and 2.45 ± 0.67/mm for distance.
CONCLUSIONS
Simple lateral rectus recession (with or without Y-splitting), even in the presence of significant deviation (through 35), seems to be a successful procedure for management of patients with unilateral exotropic DRS.
Topics: Adolescent; Adult; Child; Duane Retraction Syndrome; Female; Humans; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Treatment Outcome; Vision, Binocular; Young Adult
PubMed: 32479998
DOI: 10.1016/j.jaapos.2020.03.005 -
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 -
Seminars in Ophthalmology Jan 2020: To evaluate the surgical outcome of exotropic Duane syndrome and the factors that can influence the outcome.: A retrospective study was performed on patients who had...
: To evaluate the surgical outcome of exotropic Duane syndrome and the factors that can influence the outcome.: 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.: 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 ( = .03) and smaller angle of deviation ( = .04). The mean follow-up was 6.3 months.: Surgical results in exotropic Duane syndrome are better in small angles of deviation and in younger age groups.
Topics: Adolescent; Adult; Child; Child, Preschool; Duane Retraction Syndrome; Exotropia; Eye Movements; Female; Follow-Up Studies; Humans; Infant; Male; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Retrospective Studies; Treatment Outcome; Vision, Binocular; Young Adult
PubMed: 32126862
DOI: 10.1080/08820538.2020.1730915 -
Journal of Ophthalmic & Vision Research 2020To report unilateral congenital cataract in a case of ipsilateral Duane retraction syndrome.
PURPOSE
To report unilateral congenital cataract in a case of ipsilateral Duane retraction syndrome.
CASE REPORT
In this case, we present a six year old girl who was referred with ocular deviation. She had a history of congenital cataract surgery in the left eye at the age of two years. The subject had no associated systemic disease, developmental delay, or positive family history. She was finally diagnosed as having Duane retraction syndrome in the same eye.
CONCLUSION
Duane retraction syndrome can be associated with congenital cataract due to the matching time of gestational development of the lens to that of ocular and non-ocular anomalies associated with Duane syndrome. As both of these disorders are rare, coincidence of both in the same person and the same eye by chance is a very remote possibility.
PubMed: 32095213
DOI: 10.18502/jovr.v15i1.5952 -
International Journal of Ophthalmology 2020To study the different treatment modalities needed in cases of Duane's Retraction Syndrome (DRS).
AIM
To study the different treatment modalities needed in cases of Duane's Retraction Syndrome (DRS).
METHODS
This prospective study undergone in more than four years, in Alexandria, included 238 patients of DRS, including type I, 162 patients (68%), type II 12 patients (5%) and type III 64 patients (27%). Surgery was indicated in 98 (41%) of them, to eliminate abnormal head posture, deviation of the eye in primary position, severe retraction of the globe or cosmetically unacceptable upshoot with attempted adduction.
RESULTS
Type I was the most common and type II was the least. Females were predominant in this study, constituting 125 patients (52.5%), and males 113 patients (47.5%). Left eye was more affected, in 110 patients (46.2%), right eye in 91 patients (38.2%) and bilateral in 37 patients (15.6%). Amblyopia was found in 27 patients (11.3%) and treated in 13 patients under 10 years of age, by patching the normal eye. Ninety-eight patients (41%) were operated, the results were most satisfactory and a nomogram is followed in the surgical plan.
CONCLUSION
The surgical management is needed in less than half of the cases and should be planned for every case individually according to the clinical findings, planned nomogram and modified intraoperatively according to the anatomical findings during surgery.
PubMed: 32090038
DOI: 10.18240/ijo.2020.02.12 -
International Journal of Pediatric... Apr 2020Classically, Pierre Robin Sequence (PRS) is a triad of micrognathia, glossoptosis, and airway obstruction, although frequently associated with cleft palate. Current...
OBJECTIVES
Classically, Pierre Robin Sequence (PRS) is a triad of micrognathia, glossoptosis, and airway obstruction, although frequently associated with cleft palate. Current literature reports that Stickler syndrome is the most common syndrome associated with PRS, and 22q11 deletion syndrome (22q11 DS) as the second most common. This study identifies associations between PRS and genetic syndromes.
METHODS
A retrospective chart review was performed to identify patients diagnosed with PRS over a 10-year period from 4/1/2007 to 4/1/2017 at a tertiary children's hospital.
RESULTS
4,052 consecutive charts were reviewed and 234 patients had a diagnosis of PRS confirmed with the triad of micrognathia, glossoptosis, and airway obstruction. Of note, all of these patients had cleft palate. Of the 234 patients with PRS, 65 patients had syndromic diagnoses (28%). One patient had 22q11 DS (0.43%), and 31 patients had Stickler syndrome (13.2%). Additionally, 3 patients had central hypoventilation syndrome, 3 patients had Duane syndrome, 2 patients had Cornelia de Lange syndrome, 2 patients had Emanuel syndrome, 2 patients had Gordon syndrome, 2 patients had Mobius syndrome, 2 patients had Nager syndrome. Multiple other syndromes were identified, but occurred in isolated cases.
CONCLUSION
This study supports literature that PRS is most commonly associated with Stickler Syndrome but rarely associated with 22q11 DS given that only 1 patient had both PRS and 22q11 DS.
Topics: 22q11 Deletion Syndrome; Adolescent; Arthritis; Arthrogryposis; Child; Child, Preschool; Chromosome Disorders; Cleft Palate; Clubfoot; Connective Tissue Diseases; De Lange Syndrome; Duane Retraction Syndrome; Female; Hand Deformities, Congenital; Hearing Loss, Sensorineural; Heart Defects, Congenital; Humans; Hypoventilation; Infant; Infant, Newborn; Intellectual Disability; Male; Mandibulofacial Dysostosis; Mobius Syndrome; Muscle Hypotonia; Pierre Robin Syndrome; Retinal Detachment; Retrospective Studies; Sleep Apnea, Central
PubMed: 31927149
DOI: 10.1016/j.ijporl.2019.109842 -
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