Duane's Syndrome Type Research Articles

Lateral rectus recession for consecutive exotropia in esotropic Duane syndrome: A case report

Consecutive exotropia is considered as a rare complication after medial rectus recession that is performed for cases of esotropic Duane syndrome. Here, we present a case of 5-year-old female initially presented with the limitation of abduction, narrowing of the palpebral fissure, and globe retraction of the left eye along with a mild left face turn. She was diagnosed with Duane syndrome Type 1. She was treated with the medial rectus recession (6.5 mm). Postsurgery, she developed a consecutive exotropia. This was managed successfully with the ipsilateral lateral rectus recession (8 mm). Following this surgery, the patient was orthoptic in the primary position and her abnormal head posture was markedly improved.

Evaluation of augmented temporal superior rectus transposition combined with adjustable medial rectus recession in sixth nerve palsy and esotropic Duane syndrome type 1

Evaluation of augmented temporal superior rectus transposition combined with adjustable medial rectus recession in sixth nerve palsy and esotropic Duane syndrome type 1

Botulinum toxin injection in the patients with Duane syndrome type 1

PurposeTo evaluate the efficacy of botulinum toxin injection in the patients with type 1 Duane syndrome and identify the predictive factors for success. MethodsSixteen patients with esotropic type 1 Duane syndrome without history of ocular surgery were selected for this interventional case series. The botulinum toxin was injected in the medial rectus of all patients. Visual acuity, dry refraction, cyclo-refraction, ocular motility, and amount of deviation were measured. Complete success, partial success, and failure were defined as residual deviation/face turn less than 8 prism diopters (PD)/5°, 8-20 PD/5-15°, and equal or greater than 20 PD/15°, respectively. ResultsSixteen cases (6 males) were included in our study. The mean esotropia was 26.27 ± 8.35 (12-40 PD) which was reduced significantly to 13.5 ± 12.39 PD during 6 months follow-up (p < 0.001). Face turn was improved significantly from a preoperative mean of 18.27° to: 0.094° at 1 week, 0.11° at 1 month, 3.31° at 3 months, and 7° at 6 months (p < 0.001). Complete success was seen in 6 patients (37.5%), partial success in 4 patients (25%), and failure in 6 patients (37.5%). There was a significant relation between the amount of forced duction testing (FDT) and the success rate (p: 0.019). No complication was seen during injections. ConclusionsBotulinum toxin could be an alternative treatment in Duane syndrome with appropriate case selection. FDT could be a predictive factor for response to botulinum toxin.

Medial rectus tendon elongation with bovine pericardium for type 1 Duane's retraction syndrome

Some patients with esotropic Duane's retraction syndrome (type 1) adopt a head turn to gain binocular vision. For some patients recession of the ipsilateral medial rectus muscle is not sufficient to eliminate head turn and squint angle. Surgery with tendon elongation allows the correction of larger angles and maintains a sufficient arc of contact. We report our results and experience. We retrospectively reviewed the medical records of 10 patients with unilateral, esotropic Duane syndrome type 1 who had tendon elongation with bovine pericardium to correct a head turn or squint angle. Two patients had primary surgery with tendon elongation due to preoperative excessive angles, eight patients had tendon elongations as secondary procedures. Nine of the patients had their follow-up examination at least six weeks after surgery. The median preoperative angle of squint in primary position was + 27.5 pdpt, the median postoperative angle + 0 pdpt. The median dose effect relation for secondary interventions was 2.4 pdpt reduction of squint angle/mm surgery, for one patient with primary surgery and long-term follow-up 2.75 pdpt/mm. The median, preoperative head turn of seven patients with preoperative binocular functions was 20°. Postoperatively, six patients with long-term follow-up showed a median head turn of 0°. The median dose-effect relation was 3.3° reduction of head turn/mm surgery. All nine patients with long-term follow-up had a maximal head turn of 10°, six of the patients had no persisting head turn. Binocular functions were stable or better than preoperatively. For patients with retraction syndrome type 1, tendon elongation of the medial rectus muscle using Tutopatch® is a good option for secondary interventions or excessive preoperative squint angle or head turn.

Surgical outcomes following ocular re-alignment in various types of squints

Background: Strabismus is one of the causes for visual impairment in childhood. It affects the development of binocular single vision. Therefore, early detection and timely management of strabismus is important. Aim: This study was aimed to describe types of strabismus patients presenting to a tertiary care center and the various management modalities including the surgical outcomes of ocular re-alignment Materials and Methods: Retrospective chart analysis of all patients with strabismus in any age group over a period of 5 years. Patients with cerebral palsy were excluded from this study. Surgical outcomes were graded as good if the eyes were orthophoric post-operatively, satisfactory if there was a residual squint of less than 10 degrees, and it was considered to be a poor outcome if the patients were left with more than 10 degrees of residual squint. Results: Chart analysis of 53 cases with strabismus comprising of 33 males and 20 females was done. The mean age of patients was 25 years. Thirty-two (60%) cases had exotropia, and 14 (26%) had esotropia. Three cases were of congenital superior oblique palsy, and one case was of lateral rectus palsy. Duane syndrome type I was seen in four cases. Of the 38 (72%) patients who were advised surgical correction, only 20 individuals underwent surgery. Reasons for refusal of surgery varied from fear factor to financial constraints and a disregard for cosmesis. Surgeries had been performed on both children (7) as well as adults (13). Seventy-five percent of the operated patients had exotropia. Eighteen patients underwent single-staged surgery. Bilateral recessions were the most common type of surgery noted in this study. Fifty percent of them remained orthophoric up to one year of post-operative follow up. Conclusion: Exotropia was the most common type of strabismus observed in this study. Successful ocular re-alignment was seen in majority of the patients following a single-stage procedure. Greater effort needs to be made to educate the public on need for early diagnosis and management.

Recurrent Meningitis Associated With Duane Syndrome Type 1

We present the case of a 17-month-old male patient with a bilateral Duane syndrome type 1 associated to unilateral cochleovestibular dysplasia, perilymphatic fistula and recurrent meningitis. Diagnosis was carried out by MRI and CT scan. His management and treatment are described, as well as the postoperative evolution. We believe this is an exceptional case due to the low frequency of this syndrome, as well as to the otoneurological complications.

Meningitis de repetición asociada a síndrome de Duane tipo 1

We present the case of a 17-month-old male patient with a bilateral Duane syndrome type 1 associated to unilateral cochleovestibular dysplasia, perilymphatic fistula and recurrent meningitis. Diagnosis was carried out by MRI and CT scan. His management and treatment are described, as well as the postoperative evolution. We believe this is an exceptional case due to the low frequency of this syndrome, as well as to the otoneurological complications.

Nishida’s procedure combined with medial rectus recession for large-angle esotropia in Duane syndrome

Two patients presented large-angle esotropia due to unilatelal Duane syndrome type I. We report the course of a simple muscle transposition procedure (the Nishida procedure) with medial rectus muscle recession for large-angle esotropia in two cases of unilateral Duane syndrome type I. Case 1: A 5-year-old boy had Duane syndrome type I OS. He had esotropia of 40 prism diopters, and the left eye could not abduct to the midline. Postoperatively, his esotropia decreased to 6 prism diopters and the abduction improved to 45°. Case 2: A 5-year-old girl had Duane syndrome type I OS with a marked facial turn. She had esotropia of 40 prism diopters in primary position, and the left eye could not abduct to midline. Postoperatively, the facial turn resolved, the esotropia decreased to 8 prism diopters, and the abduction improved to 30°. This procedure improves large-angle esotropia and abduction deficits in unilateral Duane syndrome type I.

Results of bilateral medial rectus muscle recession in unilateral esotropic Duane syndrome

To report the results of bilateral medial rectus muscle recession in improvement of the ocular alignment and motility of patients with unilateral esotropic Duane syndrome. All medical files of patients with Duane syndrome seen by the first author between 1997 and 2006 were reviewed. Pre- and postsurgical deviation, angle of abnormal head position, severity of limitation in abduction, severity of globe retraction, and upshoots and downshoots were compared. Twenty-five patients with unilateral esotropic Duane syndrome (type 1) underwent recession of both medial rectus muscles. Mean age of patients was 9.7 years: male-to-female ratio was 0.67 (10:15). The left eye was involved in 23 of the patients. Mean esotropia decreased from 24.3(Delta) (range, 12(Delta)-50(Delta)) to 1.3(Delta) (range, 0(Delta)-10(Delta)). Mean abnormal head position decreased from 21.4 degrees (range, 15 degrees-35 degrees) to 1 degrees (range, 0 degree-5 degrees). Esotropia and abnormal head position disappeared in 80% of the patients and improved in the remaining. None of the patients developed exotropia. Mean limitation in abduction decreased from -3.8 to -3.3. Globe retraction was eliminated in 14 of the patients and improved in the others. Mild upshoots and downshoots, which were observed in 5 patients, disappeared in 2 and improved in 3 patients. Bilateral recession of medial rectus muscles has resulted in improvement of deviation, abnormal head position, and globe retraction in patients with unilateral esotropic Duane syndrome.

Vertical Rectus Muscle Transposition for Correcting Abduction Deficiency in Duane's Syndrome Type 1 and Sixth Nerve Palsy

To report the clinical outcome and complications of the Scott Foster procedure for treating abduction deficiency in patients with Duane's syndrome type 1 and sixth nerve palsy. A retrospective, interventional case series included 62 consecutive patients (62 eyes: 38 eyes with Duane's syndrome and 24 eyes with sixth nerve palsy) who underwent the Scott Foster procedure for treatment of abduction deficiency. The main outcome measures were deviation, face turn, and abduction deficiency. In patients with sixth nerve palsy, mean distance deviation improved from 44.7+/-7.2 prism diopters (PD) before surgery to 12.5+/-4.0 PD after surgery (P<.05), and in patients with Duane's syndrome type 1, mean distance deviation improved from 31.5+/-4.3 PD preoperatively to 9.2+/-3.1 PD. Mean near deviation improved from 27.1+/-4.5 PD to 6.2+/-2.5 PD in patients with Duane's syndrome and from 40.3+/-6.2 PD to 13.7+/-3.4 PD in patients with sixth nerve palsy. Mean abduction deficiency improved from -4 to -2 (P<.05) in both groups. Face turn improved significantly after surgery. No patient had an overcorrection. No recurrence was detected. The Scott Foster procedure is effective for improving deviation, abduction deficiency, and face turn in patients with Duane's syndrome type 1 and especially in those with sixth nerve palsy.

Local Deformation of Extraocular Muscles during Eye Movement

To study extraocular muscle (EOM) function, the local physiologic contraction and elongation (deformation) along human horizontal EOMs were quantified by using motion-encoded magnetic resonance imaging (MRI). Eleven subjects (healthy right eye) gazed at a target that moved horizontally in a sinusoidal fashion (period, 2 seconds; amplitude, +/-20 degrees), during MRI with an optimized protocol. In addition, EOM longitudinal deformation in two patients with Duane's syndrome type I was analyzed. The horizontal EOMs and the optic nerve were tracked through 15 time frames, and their local deformation was calculated. Eight segments were separated along the EOMs and left-to-right and right-to-left eye movements were compared. In healthy subjects, the maximum EOM deformation was situated at approximately two thirds of the muscle lengths from the scleral insertions. The EOM deformations were similar for the entire movement range as well as in both movement directions. In the two patients with Duane's syndrome type I, the abnormal innervation of lateral rectus muscle affected specific EOM segments only. The posterior muscle segments contracted and the anterior muscle segments relaxed during adduction. Motion-encoded MRI is a useful technique for advancing the understanding of the physiology and pathophysiology of EOMs in humans during eye movement.

Clinical variability in a three‐generation family with Duane’s syndrome

Abstract Purpose The majority of Duane syndrome cases are sporadic in origin with only 2‐5% patients showing a familial pattern. Only a few large families have been reported. The purpose of this study is to report the clinical presentations, abnormalities of ocular motility, and strabismus in 3 generations of a family with Duanes syndrome. Methods 22 members of a family of three generations (age range 6 years to 70 years) were examined. Detailed ophthalmologic assessment included assessment of vision, binocularity, and video recordings of ocular motility. Results Unilateral Duane syndrome type 1 was noted in the left eye of a lady in the first generation of the pedigree. In the second generation, of the 4 subjects examined, two females had esotropia with diplopia, one male had minimal bilateral abduction deficit with endpoint nystagmus and one sibling was normal. 12 subjects from the third generation were examined. Two of them had unilateral Duane’s syndrome. Two children had minimal abduction deficit with endpoint nystagmus. Three subjects had esotropia and diplopia requiring surgery. The mother of one of the affected subject had normal eye movements. Conclusion This large family with Duane syndrome appears to have a dominant form of inheritance with variable penetrance and expressivity. The skipped generation indicates unaffected carrier state. The variable phenotypic expression seen in this family suggests that classical Duane syndrome could be one end of a spectrum of eye movement disorders comprising esotropia, abduction deficits and globe retraction; the other end being normal ocular motility.

Tratamiento quirúrgico del síndrome de duane, tipo I mediante retroinserción del recto medial del lado afecto y faden del recto medial contralateral

Different surgical approaches have been described for the treatment of Duane's syndrome. The purpose of our study is to report the results of patients undergoing recession of the medial rectus (MR) muscle of the affected eye and placement of contralateral MR faden posterior fixation sutures. Retrospective study of 11 patients treated by a 4-7 mm recession of the MR of the affected eye and 13 mm faden posterior fixation suture of the contralateral MR in order to correct abnormal head position and esotropia in primary position. After surgery, there was no torticolis in 81.8% of patients, with less than 10 degrees of torticolis in the remainder. In all patients, postoperative esotropia was less than 5 prismatic dioptres. This is a safe and effective procedure in Duane's syndrome type I to treat moderate esotropia and torticolis.

Duane retraction syndrome: Type II with severe abducens nerve hypoplasia on magnetic resonance imaging

Duane retraction syndrome is a congenital oculomotor disorder characterized by horizontal eye movement limitations with palpebral fissure narrowing and globe retraction on attempted adduction. Recent reports showed the absence of the subarachnoid part of the abducens nerve on magnetic resonance imaging (MRI) in types I and III. We present a case of Duane syndrome type II, in which severe hypoplasia of the abducens nerve was revealed on high-resolution MRI.

Clinical Characteristics of Bilateral Duane Syndrome

To describe the clinical characteristics of bilateral Duane syndrome. Retrospective medical record review (1982 to 2003) for patients with a diagnosis of Duane syndrome (examined by a pediatric ophthalmologist) who were bilaterally affected and had no prior ocular surgery. Data collected included type of Duane syndrome, gender, family history for strabismus, abnormal head position, versions, strabismus measurements, associated ocular and/or nonocular abnormalities, and amblyopia status. Of 270 patients with the diagnosis of Duane syndrome, 37 (14%) were bilaterally affected. None had ocular surgery prior to referral. Twenty-two (59%) were male, 35 (95%) had the same Duane syndrome type in both eyes, 29 (78%) had strabismus in primary position, 9 (24%) had ocular and nonocular congenital abnormalities, 6 (16%) had amblyopia, and 8 (22%) had a recorded strabismus family history. Unlike unilateral Duane syndrome, bilateral Duane syndrome may be more common in males and associated with a higher prevalence of strabismus in primary gaze position. The prevalences of amblyopia, positive strabismus family history, and associated congenital abnormalities in this series of bilateral cases is similar to the reported prevalence.

Full Vertical Rectus Muscle Transposition Combined with Medial Posterior Fixation Sutures for Patients with Adduction Deficiency

To compare the surgical and functional results of full tendon rectus muscle transposition to the medial rectus muscle augmented with posterior medial fixation sutures in patients with adduction deficiency (ADD) and Duane's syndrome type 2. Retrospective nonconcurrent interventional comparative case series. Twelve consecutive patients with ADD and Duane's syndrome type 2. Six patients treated by the full tendon rectus muscle transposition procedure alone were compared with 6 patients treated by the full tendon rectus muscle transposition combined with posterior medial fixation sutures. Postoperative ocular alignment, duction improvement, binocular functions, and reoperation. In the augmented surgery group, mean distance and near deviation decreased by 74.5% and 74%, respectively, versus 56% and 59%, respectively, in the control group (P = 0.007 and 0.02, between-group comparison for distance and near deviation, respectively). Mean duction improved in 73% of the study group compared with 52% of controls (P = 0.025). No postoperative complications or duction anomalies were observed during follow-up in the entire cohort; reoperation was needed in 1 patient in the study group and in all 6 patients in the control group. The augmented full vertical rectus muscle procedure is a beneficial surgical approach for patients with ADD and Duane's retraction syndrome type 2, reducing the need for multiple extraocular muscle surgery.

Familial ptotic lid elevation during ipsilateral abduction

To report and discuss the clinical findings of a 17-member family with 2 siblings who exhibit ptosis and abnormal synkinetic lid elevation associated with ipsilateral abduction. Sixteen members of the 17-member immediate family underwent ophthalmic examination. Two siblings exhibited ptosis and abnormal synkinetic lid elevation associated with ipsilateral abduction. One was bilaterally affected and the other had unilateral findings. A third sibling had isolated bilateral congenital ptosis. A fourth demonstrated classic Duane syndrome Type I in the right eye. Other family members did not have ophthalmic abnormalities. A unifying mechanism of congenital cranial dysinnervation may underlie these and similar phenotypes of oculomotor and/or abducens nerve abnormalities with or without abnormal synkinesis.

Multiple Orbital Dermoid Cysts Located Within the Lateral Rectus Muscle, Clinically Mimicking Duane Syndrome Type II

Multiple Orbital Dermoid Cysts Located Within the Lateral Rectus Muscle, Clinically Mimicking Duane Syndrome Type II

Possible new autosomal recessive syndrome of partial agenesis of the corpus callosum, pontine hypoplasia, focal white matter changes, hypotonia, mental retardation, and minor anomalies

We describe a brother and sister with severe developmental delay, hypotonia, partial agenesis of the corpus callosum, pontine hypoplasia, focal white matter degenerative abnormalities, macrocrania, frontal bossing, deep-set eyes, and hypertelorism. The brother also had Duane syndrome type II and an ectopic right ureter. The coexistence of these multiple physical and brain abnormalities in a brother and sister suggests a new autosomal recessive syndrome with a slowly progressive course.

Single Medial Rectus Recession in Unilateral Duane Syndrome Type I

Single Medial Rectus Recession in Unilateral Duane Syndrome Type I