Cases reported "duane retraction syndrome"

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1/108. Significant immediate and long-term reduction of astigmatism after lateral rectus recession in divergent Duane's syndrome.

    Duane's syndrome is associated with anisometropia and amblyopia. We encountered 1 patient with right divergent Duane's syndrome (type 2 according to Huber's classification) with high astigmatism of the right eye and a head turn. In order to improve the head turn and thereby avoid eccentric gaze through the glasses, the right lateral rectus muscle was recessed by 7.75 mm. Postoperatively, the amount of astigmatism was reduced by 1.5 dpt with no change of the axis. This change of refraction remained stable over a follow-up time of 18 months. ( info)

2/108. Familial Duane's retraction syndrome.

    BACKGROUND: Duane's retraction syndrome (DRS) has been described as a clinical entity for more than a century. Although the majority of cases occur spontaneously, some cases of DRS are inherited. case reports: A young Romanian boy with a known left abduction deficit was determined to have DRS. On thorough examination of other family members, it was discovered that his younger brother and father also had the syndrome. Clinical findings of the affected family members are described. RESULTS: All three family members showed left esotropia in primary gaze, along with a compromised ability to turn the left eye outward. They also demonstrated an anomalous head turn to the left, and mild narrowing of the palpebral fissure and retraction of the globe on adduction. These are all classic signs of unilateral DRS. CONCLUSION: These findings provide further evidence of an autosomal dominant mode of inheritance in some cases of DRS. ( info)

3/108. Recession of both horizontal rectus muscles in Duane syndrome with globe retraction in primary position.

    INTRODUCTION: Duane syndrome is characterized by abduction deficiency, narrowing of the palpebral fissure on adduction, and globe retraction,which can be the most prominent aspect of the motility disorder. Recession of both horizontal rectus muscles was investigated for treatment of severe globe retraction. methods: Three patients with Duane syndrome were operated on for severe globe retraction. The medial rectus muscles were recessed from between 5.5 to 6.5 mm and the lateral rectus muscles 7.0 to 9.0 mm simultaneously. The recessions were asymmetric, as evidenced by amount of esotropia and face turn. Preoperative Hertel measurements were made in primary gaze, 30-degree left gaze, and 30-degree right gaze. The measurements were repeated at 6 months and 1 year after the operation. RESULTS: All three patients had improvement in globe retraction. The 6-month Hertel readings in primary position were improved by a mean of 3.0 mm (range 2.5 to 3.5 mm) measured in primary gaze. Hertel measurements were stable at 1 year after the operation. No complications were encountered. CONCLUSIONS: Recession of both horizontal rectus muscles is effective in the treatment of significant globe retraction in Duane syndrome. ( info)

4/108. A case of congenital inverse Duane's retraction syndrome.

    Inverse Duane's retraction syndrome is very uncommon. Congenital cases are even more unusual. A 6-year-old girl with convergent squint along with severe restriction on abduction is described. On attempted abduction, a narrowing of the palpebral fissure, upshoot and retraction of the eyeball were observed. brain and orbit MRI demonstrated no intracranial or intraorbital mass, fracture, or entrapment of the medial rectus. Forced duction test was strongly positive. The primary lesion was found to be a tight medial rectus with shortening and soft tissue contracture. Surgical tenotomy of the medial rectus led to successful postoperative motility, but some limitation at full adduction and abduction persisted. This is a case reported with congenital medial rectus shortening, suggesting that this condition may be one of the etiologies of the rare inverse Duane's retraction syndrome. ( info)

5/108. duane retraction syndrome, a new feature in 22q11 deletion syndrome?

    A female child with duane retraction syndrome is described. A microdeletion on chromosome 22(q11) was discovered using FISH analysis. It is postulated that duane retraction syndrome might be a new feature in 22q11 deletion syndrome. ( info)

6/108. A patient with de Morsier and Duane syndromes.

    de Morsier syndrome, or septo-optic dysplasia, is a developmental malformation complex characterized by optic nerve hypoplasia, dysgenesis of the septum pellucidum, and hypothalamic-pituitary dysfunction. (1,2) In duane retraction syndrome, there is absence of the sixth nerve nucleus with congenital retraction of the globe and narrowing of the lid fissure in adduction, frequent abduction deficiency, and variable limitation to adduction of the affected eye. (3) The purpose of this report is to present a patient with the uncommon and previously unreported concurrence of both of these congenital malformation complexes, presumably because of a common disturbance of neuronal development. ( info)

7/108. Lateral rectus split in the management of Duane's Retraction Syndrome.

    This case reports Type I Duane's Retraction Syndrome with marked upshoot of the globe in adduction. An 18-year-old patient presented with complaints of not being able to move his left eye outwards since childhood. A 'Y' split of the lateral rectus muscle was performed in the affected eye. There was marked cosmetic and functional improvement in the patient which was stable after 6 months of follow-up. Y splitting of the lateral rectus muscle is a simple technique that can be used effectively in the treatment of marked upshoot or downshoot in Duane's Retraction Syndrome. ( info)

8/108. Duane's retraction syndrome: literature review.

    BACKGROUND: Duane's retraction syndrome (DRS), also known as Stilling-Turk-Duane syndrome, is defined as a congenital miswiring of the lateral and medial recti muscles, resulting in an impaired ocular motility syndrome that includes palpebral fissure narrowing. The incidence of DRS is approximately 1% of the total cases of strabismus. Eighty percent of cases are unilateral and characterized by either limited abduction, limited adduction, or both. CASE REPORT: A 21-year-old man came to the clinic for a routine ocular examination without symptoms. A review of the history uncovered the presence of congenital, type I Duane's retraction syndrome. The examination demonstrated orthophoria in primary gaze, an abduction deficit O.S., and left globe retraction with palpebral fissure narrowing on right gaze O.S. MANAGEMENT: In most cases of DRS the eyes are straight in primary position and there is no amblyopia. amblyopia, when present, is usually the result of anisometropia and not strabismus. Because our patient had no symptoms of diplopia in primary gaze (orthophoria) or in attempted right gaze (due to suppression of the left eye with abduction), prismatic and/or surgical management were not indicated, since the patient was free from binocular and cosmetic abnormalities. CONCLUSION: DRS is characterized by abnormal development of the cells in the abducens nucleus (CN VI), resulting in restricted or absent abduction and erroneous innervation of the lateral rectus by branches emanating from oculomotor nuclei (CN III). Management may include orthoptics, surgery, or monitoring. ( info)

9/108. Duane's syndrome with compressive denervation of the lateral rectus muscle.

    PURPOSE: To describe an unusual case of Duane's syndrome. methods: Individual case report. RESULTS: A skull base meningioma was discovered in a woman with Duane's syndrome who presented with recurrent, large-angle esotropia and uncharacteristic atrophy of the lateral rectus muscle on magnetic resonance image (MRI) scan. CONCLUSION: neuroimaging may be useful in unusual cases of Duane's syndrome. ( info)

10/108. Oto-facio-cervical (OFC) syndrome is a contiguous gene deletion syndrome involving EYA1: molecular analysis confirms allelism with BOR syndrome and further narrows the Duane syndrome critical region to 1 cM.

    Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder involving hearing loss, branchial defects, ear pits and renal abnormalities. Oto-facio-cervical (OFC) syndrome is clinically similar to BOR syndrome, with clinical features in addition to those of BOR syndrome. Mutations in the EYA1 gene (localised to 8q13.3) account for nearly 70% of BOR syndrome cases exhibiting at least three of the major features. Small intragenic deletions of the 3' region of the gene have also been reported in patients with BOR syndrome. We have developed a fluorescent quantitative multiplex polymerase chain reaction for three 3' exons (7, 9 and 13) of the EYA1 gene. This dosage assay, combined with microsatellite marker analysis, has identified de novo deletions of the EYA1 gene and surrounding region in two patients with complex phenotypes involving features of BOR syndrome. One patient with OFC syndrome carried a large deletion of the EYA1 gene region, confirming that OFC syndrome is allelic with BOR syndrome. Microsatellite analysis has shown that comparison of the boundaries of this large deletion with other reported rearrangements of the region reduces the critical region for Duane syndrome (an eye movement disorder) to between markers D8S553 and D8S1797, a genetic distance of approximately 1 cM. ( info)
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