Duane Retraction Syndrome Patients Research Articles

Clinical characteristics and surgical approach in Duane retraction syndrome: a study of 691 patients.

To evaluate the clinical findings of different types of Duane retraction syndrome (DRS). Retrospective. 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. 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). 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.

Outcomes of symmetric bilateral medial rectus recession in large-angle esotropic Duane syndrome.

To evaluate the efficacy of symmetric bilateral medial rectus recession in large-angle esotropic Duane retraction syndrome (DRS) with moderate to severe globe retraction. In a retrospective study, medical reports of 30 patients with esotropia of equal or more than 20 prism diopters (pd) and moderate to serve globe retraction due to unilateral DRS who underwent symmetric bilateral medial rectus recession were reviewed. Age, gender, laterality, amblyopia, length of follow-up, pre- and postoperative measurements of primary position deviation, ocular ductions and severity of globe retraction and abnormal head posture were evaluated. A successful result was defined as decreasing esotropia to equal or less than 8 pd or equal or less than 8 pd of consecutive exotropia. The mean age of patients at surgery was 13.7 ± 8.5 years old (range: 3-38). The mean preoperative esotropia measured 28.9 ± 9.1 pd in distance and 25.7 ± 7.2 pd in near, which decreased to 4.9 ± 6.1 pd in distance and 3.9 ± 8.8 pd in near postoperatively. The mean bilateral medial rectus recession was 4.9 ± 0.9 mm (range: 3-6 mm). The mean abnormal head posture improved from 19.1 ± 6.9 degrees (range: 10-30 degrees) to 3.3 ± 4.7 degrees (range: 0-15 degrees) postoperatively. At the last follow-up visit, 23 patients (76.7%) had a successful outcome. In the large-angle esotropic DRS patients with moderate to severe globe retraction, symmetric bilateral medial rectus recession, can be conducted to successfully resolve primary position deviation and abnormal head posture.

Association of lateral rectus muscle volume and ocular motility with the abducens nerve in Duane's retraction syndrome.

To investigate the relationship between ocular motility and lateral rectus (LR) muscle volume according to the presence or absence of the abducens nerve in patients with Duane's retraction syndrome (DRS) using high-resolution magnetic resonance imaging (MRI). A total of 54 unilateral DRS patients were divided into two groups according to high-resolution MRI findings: DRS without an abducens nerve on the affected side (absent CN6 group, n = 45) and DRS with symmetric abducens nerves on both sides (present CN6 group, n = 9). Ocular motility was measured by image analysis based on nine gaze photographs. LR volume was measured on T2-weighted coronal MRI of the orbit, and the ratio of paretic/normal side (P/N) LR volume was investigated. Association of the abducens nerve with various parameters including ocular motility, LR volume, and ratios of P/N LR volume were determined. LR volume was smaller in the affected eye than the non-affected eye in both groups. In the present CN6 group, abducens nerve diameter and the ratio of P/N LR volume showed a positive correlation. A smaller LR volume and more limitation of abduction in the affected eye were predictive of an absent abducens nerve in DRS. LR muscle hypoplasia was apparent in the affected eye of DRS patients. Abducens nerve diameter positively correlated with the ratio of P/N LR volume in the present CN6 group. Graphical abstract.

Clinical Features of Duane Retraction Syndrome: A New Classification.

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. 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." 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 (p = 0.003 and p < 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 (p = 0.001 and p < 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%). 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.

Duane Retraction Syndrome: Clinical Features and a Case Group-Specific Surgical Approach

ABSTRACT Purpose: To describe the clinical features of patients with Duane retraction syndrome (DRS) and evaluate the outcomes of surgical approaches based on the characteristics of each patient. Methods: The records of 38 Caucasian subjects with DRS were retrospectively reviewed. The patients were classified as type I, II, or III based on the Huber Classification. Ten patients underwent unilateral medial rectus (MR) recession due to abnormal head posture (AHP) and/or esotropia. Four patients underwent Y-splitting and recession of the lateral rectus (LR) with MR recession due to AHP and/or esotropia, upshoot, and globe retraction. Results: There was a preponderance of unilaterality, female gender, left eye, type I, orthotropia, upshoot, and low refractive error. All patients demonstrated globe retraction and fissure narrowing. AHP was only present in unilateral cases. Nine patients had amblyopia. More than half of the patients over 5 years of age had decreased stereopsis. MR recession decreased AHP to less than 8° in all patients. Y-splitting and recession of the LR eliminated upshoot in all four patients. One patient who underwent an 8-mm MR recession demonstrated −2 adduction limitation. Conclusions: The DRS patients in our study demonstrated features that are consistent with previous reports in the literature. This study emphasizes the need to consider disease classification in the surgical management of DRS patients.

A High Prevalence of Exotropia in Patients With Duane Retraction Syndrome in a Tertiary Eye Care Center in South India

To determine the prevalence and clinical characteristics of patients with Duane retraction syndrome presenting with exotropia in a tertiary eye care center. Medical records of 140 patients with Duane retraction syndrome and exotropia (of the total 441 patients with Duane retraction syndrome) seen from 2000 to 2009 were reviewed. Characteristics studied included age at presentation, gender, laterality, manifest primary position, abnormal head posture, ocular motility, and overshoots. Data were collected regarding associated ocular or systemic abnormalities and amblyopia. The mean age of the patients at presentation was 16.96 ± 15.09 years (range: 1 to 70 years). Of the 140 patients with Duane retraction syndrome and exotropia, 59 (42%) were male. Unilateral type I Duane retraction syndrome accounted for 118 (84.2%), type II for 7 (5%), and type III for 10 (7.1%) patients, whereas 5 (3.6%) had Duane retraction syndrome and bilateral exotropia. Duane retraction syndrome was seen in the left eye, right eye, and both eyes in 97 (69%), 37 (26%), and 6 (4%) patients, respectively. Exotropia ranged from 10 to 60 prism diopters. An abnormal head posture was noted in 101 (72%) and upshoot in 26 (18.6%) patients. Reported ocular and systemic abnormalities in these patients included cataract (6), aniridia (1), nasolacrimal duct block (1), and retinal breaks (1). This is an exclusive study on a large series of patients with Duane retraction syndrome and exotropia. Almost one-third of patients with Duane retraction syndrome patients might have exotropia. Although patients with Duane retraction syndrome and exotropia had clinical characteristics and associations similar to those with Duane retraction syndrome and esotropia, there was an increased propensity for overshoots and face turn opposite to the affected eye, which the authors hypothesize to be due to a tight or short lateral rectus muscle. [J Pediatr Ophthalmol Strabismus. 2017;54(2):117-122.].

Magnetic resonance imaging (MRI) in Duane retraction syndrome

Purpose: To study the magnetic resonance imaging (MRI) findings in Duane retraction syndrome (DRS). Materials and Methods: In this case–control study, 16 consecutive cases of Duane syndrome underwent MRI of the brain and orbit, after informed consent. MRI (fast imaging enhancing state acquisition) was done with special focus on pontomedullary junction to look for the status of abducens nerve and associated abnormalities along with quasi-coronal sections of orbit to estimate thickness and cross sectional area of horizontal extra-ocular muscles (EOMs). Thickness and cross-sectional area of EOMs were compared to the contralateral side and to age-matched controls. Results: Of 16 cases, 14 had DRS Type I, and 2 had DRS Type II. MRI revealed absent abducens nerve on the ipsilesional side in 12 out of 14 in DRS I, whereas two DRS II patients revealed intact abducens nerve on the affected side. Thickness and cross-sectional area of the horizontal recti was statistically comparable to the contralateral side and age-matched controls. Conclusions: All the DRS patients in study had structurally normal horizontal recti muscles, whereas 12 out of 14 of DRS-I patients had an absent abducens nerve on MRI.

Duane′s retraction syndrome with severe upshoot and ipsilateral superior rectus contracture: A rare presentation

Type III Duane's retraction syndrome (DRS) is a rare condition and represents only 1% of all cases of Duane's syndrome. It consists of limited/absent abduction as well as adduction of the affected eye. A characteristic upshoot/downshoot/both, in adduction, and globe retraction may occur in DRS patients. Secondary muscle changes like medial rectus contracture or superior rectus contracture (SRC) may rarely be associated with DRS. SRC has been reported in patients with unilateral superior oblique palsy and dissociated vertical deviation. However, no case report of association of SRC with DRS has been reported. We report a rare case of type III DRS with severe upshoot, globe retraction and ipsilateral SRC. It is hypothesized that a long standing severe upshoot could be the reason for development of secondary SRC. The condition was treated with Y-split of lateral rectus and superior rectus recession.

Auditory brainstem response and otoacoustic emissions in Duane retraction syndrome

We aimed to investigate the origin of hearing loss with relevance to auditory brainstem responses and otoacoustic emissions in patients with Duane's retraction syndrome (DRS). 17 patients with DRS were included in the study. 14 patients were diagnosed as DRS type 1 and the remaining three as type 3. The control group consisted of nine healthy subjects. Pure tone audiogram, auditory brainstem response, as well as distortion product otoacoustic emissions were recorded in all the patients and the controls. Among the 17 patients with DRS, 15 demonstrated normal hearing according to pure tone audiogram. One patient with DRS had profound sensorineural hearing loss with a pure tone average of 110 dB, and one patient had moderate sensorineural hearing loss with a pure tone average of 60 dB. Auditory brainstem response latencies of the waves I, III and V, and interwave latencies were calculated in 15 patients with DRS and were compared with the control group. There was statistically significant increase in wave I latency of left ear in the DRS group compared to the controls (P<0.05). Amplitudes of distortion product otoacoustic emissions were also recorded at frequencies of 2, 3, 4, 6, and 8 kHz in both groups and the comparison of the DRS patients and controls revealed no statistically significant difference. We recommend auditory examinations be undertaken in patients with DRS by auditory brainstem response tests, as well as distortion product otoacoustic emissions, whereas all patients with normal results should be followed-up for future hearing loss.

Duane’s retraction syndrome: its sensory features

To investigate binocularity in Duane's retraction syndrome (DRS) and to evaluate whether or not there is a relationship between the sensory and clinical features of the syndrome. Clinical and sensory findings of 29 patients with DRS were recorded. Binocularity was tested with the Bagolini glasses (BG), Worth four-dot (W4D), TNO and the stereo-fly plate of the Titmus test. Twenty-four (83%) patients showed fusion with the BG at near and 23 (79%) had fusion at distance. With the W4D, 23 (79%) patients had fusion at near and 19 (65%) had fusion at distance. Seven (24%) patients demonstrated normal stereoacuity, 15 (52%) had reduced stereoacuity and the remaining seven (24%) patients had no measurable stereoacuity. In patients without stereoacuity, amblyopia (p < 0.001), type 2 and 3 DRS (p = 0.031) and exotropia (p = 0.003) in primary position were more common than in those with reduced or with normal stereoacuity. Restriction of ocular ductions was also more severe in patients without stereoacuity than in those with reduced or normal stereoacuity (p = 0.019, p = 0.016). Patients with type 2 and 3 DRS were significantly more likely to have amblyopia (p = 0.037), large-angle heterotropia (p = 0.005) in primary position, upshoot or downshoot (p = 0.010) than those with type 1 DRS. Although approximately 75% of DRS patients had fusion and measurable stereoacuity, only 25% demonstrated normal binocularity. This report provides new data on the relationship of sensory features to most of the clinical findings of this syndrome. Sensory features, as well as most clinical features of the syndrome, are better in patients with type 1 DRS.

Duane Retraction Syndrome on the Arabian Peninsula

Purpose To describe the clinical features of patients from the Arabian Peninsula with Duane retraction syndrome (DRS). Methods Retrospective chart review of patients referred to the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia from 1982 to 2003 with a diagnosis of DRS. Patients having had prior strabismus surgery were excluded. Results Of 404 DRS patients, 347 (86%) were unilateral, 57 (14%) were bilateral, and 111 (27%) had amblyopia. There were 221 (55%) females and 182 (45%) males. The Huber classification was as follows: 315 (78%) Type I, 16 (4%) Type II, and 77 (19%) Type III. Of the 57 bilateral cases, 25 (44%) were female and 32 (56%) were male. Discussion Overall, the clinical features of DRS patients referred to a Riyadh eye hospital are similar to those reported in series throughout the world. However, our referred bilateral DRS patients are more commonly male. The clinical features of bilateral DRS deserve further worldwide study.

Presence of the abducens nerve according to the type of Duane's retraction syndrome

To analyze whether the presence of the abducens nerve might depend upon the type of Duane's retraction syndrome (DRS). Observational case series. Sixteen patients with type 1 DRS, 2 patients with type 2 DRS, and 5 patients with type 3 DRS. Ophthalmologic examination and thin-sectioned magnetic resonance imaging (MRI) at the brain stem level were performed in 23 DRS patients. To confirm the accuracy of the procedure, we compared the results obtained with those of a control group of 30 individuals using the same technique. Examination of ductions and versions and the presence of the abducens nerve on MRI. The abducens nerve on the affected side could not be observed using MRI in 18 (100%) of 18 eyes (16 patients) with type 1 DRS and in 3 of 5 eyes with type 3 DRS. Conversely, the abducens nerve was observed in 2 of 2 eyes with type 2 DRS and 2 of 5 eyes with type 3 DRS. The abducens nerve was observed in 60 (100%) of 60 eyes screened as controls. The abducens nerve on the affected side was absent in type 1 DRS patients and some type 3 DRS patients, but present in type 2 DRS patients as well as in some type 3 DRS patients. In terms of the presence or absence of the abducens nerve, type 1 and type 2 DRS were homogenous, and type 3 DRS was heterogenous.

Klinische und molekulargenetische Befunde beim isolierten sporadischen Retraktionssyndrom nach Stilling-Türk-Duane

Duane retraction syndrome (DURS) accounts for 1 - 4 % of all cases of strabismus. Approximately 90 % of the cases are sporadic with a preponderance for females and the left eye. Many associated ocular and systemic findings have been described. Recently, mutations of SALL4 have been found in patients with autosomal-dominantly inherited Okihiro syndrome (DURS associated with forearm malformations). The aim of this study was the clinical examination of patients with isolated sporadic DURS and the molecular genetic analysis of SALL4 in these patients. Twenty-five patients with non-familial DURS (aged 1 - 75 years, 16 female, 9male) were examined clinically and were interviewed concerning associated pathologies. DNA was prepared from peripheral lymphocytes, and the complete coding region of SALL4 was sequenced. In 18 patients DURS affected the left eye, in four the right eye, and was bilateral in three patients. One patient had fused vertebrae, one had a cone-rod-dystrophy. No hearing impairments or malformation of the upper limbs were observed. No mutation in the coding region of SALL4 could be detected. Associated conditions in DURS patients most commonly involve the ear, the spinal column, the kidneys and the heart and the upper limbs. No mutations in SALL4 could be detected in patients with isolated sporadic DURS as opposed to findings in familial Okihiro syndrome. However, Okihiro syndrome shows marked intra- and interfamilial variability, suggesting that in rare cases of isolated DURS a causative SALL4 mutation may be found.

Duane retraction syndrome.

Duane retraction syndrome (DRS) consists of deficient horizontal eye movements, eyelid retraction, palpebral fissure narrowing, and abnormal vertical eye movements. Most cases are sporadic and unilateral (usually left side) with a slight female predominance. Several associated ocular and systemic conditions have been described in DRS patients. In most cases, the abducens nucleus and nerve are absent or hypoplastic, and the lateral rectus muscle is innervated by a branch of the oculomotor nerve. However, there may be contributing mechanical abnormalities. Type I DRS (primary gaze position esotropia with limitation of abduction) comprises the majority of cases. Approximately 50% of type I DRS patients are orthophoric in primary gaze. Esotropia is the most common type of strabismus encountered, and characteristic up shoots and down shoots occur in adduction. Surgical intervention has gradually become more popular in order to improve the primary gaze alignment and mitigate some of the associated abnormalities in ocular motility. However, patients are rarely rendered clinically normal, and limited expectations are appropriate.