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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 Child...
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To evaluate the causes of acute acquired comitant esotropia (AACE) in young adults and children in the setting of COVID-19-induced home confinement.
Methods:
A retrospective, clinical study of all patients, who presented to the Pediatric Ophthalmology and Strabismus services of a tertiary eye care center in South India from August 2020...
Purpose:
The aim of our study was to determine the prevalence of amblyopia risk factors in children visiting the American University of Beirut Medical Center (AUBMC) using automated vision screening.
Methods:
This was a hospital-based screening of 1102 children aged between 2 and 6 years. Vision screening was performed using PlusoptiX S12 over 2...
Background:
The American Association for Pediatric Ophthalmology and Strabismus (AAPOS) issued a 2021 update of Uniform Validation Guidelines for instrument-based pediatric vision screeners. With each update it is important for each manufacturer to update the Instrument Referral Criteria (IRC) programed into their devices in order to optimize sens...
Axon guidance is a crucial process for growth of the central and peripheral nervous systems. In this study, 3 axon guidance related disorders, namely- Duane Retraction Syndrome (DRS) , Horizontal Gaze Palsy with Progressive Scoliosis (HGPPS) and Congenital fibrosis of the extraocular muscles type 3 (CFEOM3) were studied using various Systems Biolog...
Citations
... According to Murillo-Corea et al, (10) prevalence of DRS is about 1/1000 in general population, with 60% female predominance. DRS accounts for 4% of all strabismus cases, most commonly is due to congenital aberrant ocular innervation A study conducted in 2020 by Yun Jeong Lee et al (11) showed that DRS was more in males with a percentage of 56.9% compared to females by 43.1%. (11) In this study, no patients had amblyopia, and three patients only had ref-ractive errors, one was myope, and two were hypermetropes. ...
... DRS accounts for 4% of all strabismus cases, most commonly is due to congenital aberrant ocular innervation A study conducted in 2020 by Yun Jeong Lee et al (11) showed that DRS was more in males with a percentage of 56.9% compared to females by 43.1%. (11) In this study, no patients had amblyopia, and three patients only had ref-ractive errors, one was myope, and two were hypermetropes. Association with amblyopia was found in only one case (8.3%) in a study conducted in Turkey by Aygit, E. et al, (13) so this agrees with this study proving the very low incidence of amblyopia. ...
... Left eye was more affected, in 110 patients (46.2%), right eye in 91 patients (38.2%) and bilateral in 37 patients (15.6%). Regarding sex distribution, Gaballah K. et al.(12) and O'Malley ER et al. had cl-ose results to this study, but against the result of Yun Jeong Lee et al.(11) who described male predominance. Concer-ning Laterality, this study proves the Lt. Eye predominance that was also evidenced by other studies. ...
... Eminently, Lee et al. classified DRS into Exo-, Ortho-, and Eso-DRS subtypes according to the horizontal deviation angle at the primary gaze [87]. Compared with the traditional Huber classification for DRS, their new classification system is more convenient and practically useful in clinical settings. ...
Congenital fibrosis of the extraocular muscles (CFEOM) is a genetic disorder belonging to the congenital cranial dysinnervation disorders and is characterized by nonprogressive restrictive ophthalmoplegia. It is phenotypically and genotypically heterogeneous. At least seven causative genes and one locus are responsible for the five subtypes, named CFEOM-1 to CFEOM-5. This review summarizes the currently available molecular genetic findings and genotype–phenotype correlations, as well as the advances in the management of CFEOM. We propose that the classification of the disorder could be optimized to provide better guidance for clinical interventions. Finally, we discuss the future of genetic-diagnosis-directed studies to better understand such axon guidance disorders.
... 58 However, there may be no head turn in some patients with unilateral Duane's retraction syndrome. 57,59 ...
Purpose:
To provide a comprehensive review on different characteristics of abnormal head postures (AHPs) due to different ocular causes, its measurement, and its effect on facial appearance.
Methods:
In this review article, PubMed, Scopus, and Google Scholar search engines were searched for the scientific articles and books published between 1975 and September 2020 based on the keywords of this article. The selected articles were collected, summarized, classified, evaluated, and finally concluded.
Results:
AHP can be caused by various ocular or nonocular diseases. The prevalence of ocular causes of AHP was reported to be 18%-25%. 1.1% of patients presenting to ophthalmology clinics has AHP. The first step in evaluating a patient with AHP is a correct differential diagnosis between nonocular and ocular sources by performing comprehensive eye examinations and ruling out other causes of orthopedic and neurological AHP. Ocular AHP occurs for a variety of reasons, the most important of which include nystagmus, superior oblique palsy, and Duane's retraction syndrome. AHP may be an essential clinical sign for an underlying disease, which can only be appropriately treated by the accurate determination of the cause. Long-standing AHP may lead to facial asymmetry and secondary muscular and skeletal changes.
Conclusion:
In conclusion, a proper differential diagnosis between nonocular and ocular causes, knowledge of the different forms of AHP and their measurement methods, accurate diagnosis of the cause, and proper and timely treatment of ocular AHP can prevent facial asymmetry and secondary muscular and skeletal changes in the patients.
Purpose:
To describe the clinical profile and magnetic resonance imaging findings of the brain in Duane retraction syndrome (DRS) and determine whether there is an association between clinical presentation and magnetic resonance imaging (MRI) brain characteristics.
Materials and methods:
This was a cross-sectional study done at a tertiary care center in South India. We recruited and analyzed the clinical characteristics of 54 patients with DRS. MRI of the brain with fast imaging employing steady-state acquisition (FIESTA) was performed in 41 cases, and the cisternal segment of the sixth nerve was studied. Statistical analysis was done to determine any association between the radiological and clinical features.
Results:
Type 1 DRS was predominant, followed by Type 3 DRS and Type 2 DRS. 9.3% of cases were bilateral and 11.1% were familial. Orthotropia was most common, followed by esotropia and exotropia. The MRI brain showed the absence of the cisternal part of the sixth nerve on the affected side in 82% of Type 1 and 75% of Type 3 unilateral DRS. Both the abducens nerves were visualized in 19.5% of the patients with unilateral DRS. There was no statistically significant association between MRI brain findings and the clinical features.
Conclusions:
MRI brain with FIESTA shows absent or hypoplastic sixth nerve in most cases of Type 1 and Type 3 DRS. However, around 20% of DRS cases may show the presence of the cisternal part of the sixth nerve. Hence, clinicians must be cautious when ruling out DRS on the basis of MRI brain findings. Although aplasia of the sixth nerve is the most frequent MRI finding, it may not be the sole etiologic factor.
Purpose:
To evaluate the clinical findings of different types of Duane retraction syndrome (DRS).
Study design:
Retrospective.
Methods:
This study was performed on 691 patients with DRS who underwent surgery. Clinical examinations included laterality, abnormal head posture (AHP), corrected distance visual acuity (CDVA), refractive error, amblyopia, deviation, overshoots, and type of surgery.
Results:
The mean age of patients with DRS was 16.7 ± 12.5 (range 1.0-73) years. The patients included 396 (57.3%) women and 295 (42.7%) men (P < 0.001). DRS type I, was observed in 429 (62.1%), II in 168 (24.3%), III in 88 (12.7%) and IV in 6 (0.9%) patients. Unilateral DRS was observed OS in 628 (90.9%) [471 (%78.9) and OD in 157 (21.1%) eyes (P < 0.001)]. O ther clinical findings were AHP (n = 522, 78.1%), overshoot (n = 236, 34.2%) and amblyopia (n = 118, 17.1%). The prevalence of overshoot in types I, II, and III was 17.5% (75/429), 60.7% (102/168) and 64.8% (57/88), respectively (P < 0.001). The prevalence of amblyopia was significantly lower in patients with AHP (80/522, 15.3%) compared to patients with normal head posture (38/169, 22.5%) (P = 0.023). The mean angle of deviation in the primary position (PP) at distance was 21.7 ± 11.5 △ for esotropic group and 17.8 ± 12.4 △ for exotropic group. Sixty-two (9.0%) patients required second surgery for resolving residual misalignment (1.1 surgeries for each patient).
Conclusions:
About two-thirds of DRS patients had AHP, one-third had overshoots, and one-sixth had amblyopia. The results show that different types of DRS are associated with different epidemiological and clinical characteristics.
Objective
To study the long-term efficacy and the complications of several transposition techniques for the treatment of Duane syndrome. These included, full vertical rectus transposition, partial vertical rectus transposition, transposition without muscle disinsertion, and superior rectus transposition.
Material and methods
A retrospective study of the patients diagnosed with Duane syndrome, and who underwent any of the different transposition techniques associated or not to the medial rectus recession with a follow-up longer than 12 months. A good result was considered a final deviation ≤10 prism dioptres (pd) in primary position, anomalous head posture <10°, and an improvement of the abduction without diplopia.
Results
Seven cases were included (6 women, 6 unilateral), and a mean age of 37.71 years. Pre-operative central gaze esotropia of 28 ± 11.68 pd decreased to 6 ± 4.62 pd at the final visit (p = .009). The anomalous head posture decreased in 6 patients, and the abduction improved one degree from −3.14 to −2.14 (mean). An induced vertical deviation ≤8 pd was observed in 4 cases. None experienced diplopia at the final visit. The percentage of reoperations was 71.42%. Only 28.57% had a favourable outcome with a single surgery, which increased to 71.42% with further surgeries at the final follow-up. Mean evolution time was 52 ± 31.65 months.
Conclusion
Vertical rectus transpositions in DS with moderate or severe clinical signs have only been effective in a small percentage of the cases. Most of them required further surgeries to resolve the complications or the under-corrections.
Resumen
Objetivo
Estudiar la eficacia a largo plazo y las complicaciones de varios tipos de transposición en el tratamiento del síndrome de Duane: la transposición muscular de rectos verticales total o parcial, la transposición sin desinserción muscular y la transposición del recto superior.
Material y métodos
Estudio retrospectivo de los pacientes diagnosticados de síndrome de Duane con esotropía operados con cualquier tipo de transposición muscular asociada o no al debilitamiento del recto medio con seguimiento mayor de 12 meses. Se consideró un buen resultado una desviación ≤ 10 dioptrías prismáticas (dp) en posición primaria de la mirada, tortícolis < 10° con mejoría de la abducción y sin diplopía.
Resultados
Un total de 7 casos fueron incluidos (6 mujeres, 6 unilaterales), con una edad media de 37,71 años. La esotropía inicial en posición primaria de la mirada de 28 ± 11,68 dp disminuyó a 6 ± 4,62 dp al final del seguimiento (p = 0,009). El tortícolis mejoró en 6, y la abducción mejoró un grado en todos (media: −3,14 a −2,14). En 4 casos apareció una desviación vertical ≤ 8 dp. Ningún paciente tuvo diplopía final. El porcentaje de reintervenciones fue del 71,42%; solo un 28,57% obtuvo un buen resultado con una cirugía y ascendió a un 71,42% al final del seguimiento con cirugías adicionales. El tiempo de evolución medio fue de 52 ± 31,65 meses.
Conclusiones
Las transposiciones en el síndrome de Duane con signos clínicos moderados o severos han sido solo eficaces en una pequeña proporción de los casos. La mayoría requirió una segunda cirugía para corregir las complicaciones o las hipocorrecciones.
