Duane retraction syndrome, egyptian study medcrave online. About 10% of affected individuals appear to have other affected family members. Pdf duane retraction or cocontraction syndrome is a congenital restrictive strabismus which can occur either as an isolated entity or in. Information for patients duanes retraction syndrome this leaflet describes duane s retraction syndrome, its causes and possible treatment. Information for patients duanes retraction syndrome. Both autosomal dominant most commonly and autosomal recessive forms of duane syndrome have been documented.
Duane syndrome is an uncommon congenital disorder of ocular motility with the following characteristics. The evaluation of patients of duane retraction syndrome regarding the clinical and anatomical aspects, putting them in consideration for the surgical plan of management. Duane retraction syndrome is a congenital strabismus syndrome occurring in isolated or syndromic forms. It is characterized by cocontraction of horizontal recti on attempted adduction causing globe retraction along with variable amounts of. In most people with isolated duane retraction syndrome, the cause of the condition is unknown. The aim was to study the clinical characteristics of duanes retraction syndrome drs in nepalese patients. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. Management of duane retraction syndrome with prismatic glasses ebru demet aygit,1 murat kocamaz,1 asli inal,1 korhan fazil,1 osman bulut ocak,1 serpil akar,2 birsen gokyigit1 1strabismus department, beyoglu eye training and research hospital, istanbul, turkey. The syndrome is classified in three types based on the presence of defect in adduction, abduction or both. While duane retraction syndrome drs is relatively common, surgical management of the associated strabismus can be challenging because of. Duane retraction syndrome drs is a form of congenital strabismus related to impairment of extraocular muscle innervation. Duane retraction syndrome associated with a small x. Duane retraction syndrome durs is a congenital disorder characterized by restricted horizontal eye movement with globe retraction and palpebral fissure narrowing on attempted adduction.
View enhanced pdf access article on wiley online library html view download pdf for offline viewing. In 70 percent of ds cases, this is the only disorder the individual has. Pdf duanes retraction syndrome clinical presentation. Surgery was aimed to eliminate the up shoot or down shoot, face turn with a horizontal deviation, and the globe retraction. Duane s retraction syndrome or dr syndrome, eye retraction syndrome, retraction syndrome, congenital retraction syndrome and stillingturk duane syndrome. Duane s retraction syndrome drs is a congenital eye movement disorder characterised by globe retraction and narrowing of the palpebral fissure on attempted adduction associated with restriction of abduction, adduction, or both. Pdf properties of saccades in duane retraction syndrome.
A few pathologic examinations revealed that the abducens nucleus and nerve corresponding to the side with the abduction deficit is absent or hypoplastic from the brain stem. Drs is a congenital form of strabismus with an incidence of 0. However, it was later made popular by alexander duane who published a series of 54 patients having this condition. Open access case report duane retraction syndrome a. Described by stilling in 1887 and turk in 1896 hence in european literature referred to as the stillingturk duane syndrome after its early describers. Duane s retraction syndrome motility disorder featuring. Duane retraction syndrome is a spectrum of eye motility disorders whose common feature is retraction of the affected eye on adduction. Purpose surgical treatment in duane retraction syndrome drs can be very challenging even for the strabismus specialists because of a wide spectrum of diversity in clinical manifestation. A congenital form of strabismus characterized by limited horizontal eye movement accompanied by globe retraction and palpebral fissure narrowing on attempted. It is characterised by restriction of outward andor inward movement of. In this article, you will learn about the condition, its impact on daily living, and when it requires treatment. The incidence of duane s syndrome is 1% to 4% of strabismus patients. To improve understanding of the binocular control of saccades by making highresolution eye movement recordings in patients with unilateral duane retraction syndrome drs type i marked by congenital absence of the sixth cranial nerve.
Duane s retraction syndrome is a congenital form of strabismus characterized by horizonatal eye movement limitation, globe retraction and palpebral fissure narrowing in attempted adduction. Duane retraction syndrome drs, also known as stillingturk duane syndrome, is a congenital eye movement anomaly characterized by variable horizontal duction deficits, with narrowing of the palpebral fissure and globe retraction on attempted adduction, occasionally accompanied by upshoot or downshoot. However, researchers have identified mutations in one gene, chn1, that cause the disorder in a small number of families. Duane s syndrome aka duane s retraction syndrome a congenital eye movement disorder due to misdirection of the nerve fibre on eye muscle causing some eye muscle to contract when they shouldnt, vice versa can be. Duane retraction syndrome drs is a congenital complex strabismus. Duane retraction syndrome drs is a congenital eye movement anomaly characterized by variable horizontal duction deficits, with. Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outwards. Pdf surgical treatment of duane retraction syndrome.
Type i with marked limitation of abduction, type ii with. In duane retraction syndrome, extraocular findings including deafness, asymmetrical face, cleft palate, outer ear anomalies, preauricular tag, vertebral anomalies fusion of the second and third. Volpe, in volpes neurology of the newborn sixth edition, 2018. A simplified approach to the treatment of duanes syndrome. In 1905, duane emphasized that the retraction of the globe is an essential clinical feature of this syndrome, thus the term duane s retraction syndrome is deeply entrenched. Prospective study of 198 patients having duane retraction syndrome.
The clinical observations made on 20 patients 24 eyes of duane s retraction syndrome are presented. All types show globe retraction on adduction and lid fissure narrowing on adduction. Ananda andy kalevar, md michael flanders, md mcgill. Duane radial ray syndrome is a disorder that affects the eyes and causes abnormalities of bones in the arms and hands. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the. Article 4 vision therapy for convergence insufficiency co. Drs is characterized by restriction of horizontal outward eye movements abduction, with eyeball retraction upon adduction. Duane s retraction syndrome duane s syndrome is a rare eye condition where one or sometimes both eyes do not move to the left or right fully and the eyelids may open and close as the eyes try to turn. In rare forms of duane s syndrome there can also be other problems, for. This condition was first described by stilling in 1887.
Duane retraction syndrome associated with a small x chromosome deletion volume 43 issue 3 khaled k. Reoperation in esotropic duane retraction syndrome. Duane syndrome genetic and rare diseases information. This abnormality results from the improper development of certain nerves that control eye movement. However, other conditions and syndromes have been found in association with ds. If you need this document in an alternative format, for example, large print, braille or a language other than english, please contact the communications office by. Duane retraction syndrome an overview sciencedirect topics. Duane syndrome affects the eye muscles so that the ability to move the eye inward, outward, or in both directions is limited. The chn1 gene provides instructions for making a protein that is involved in the early development of the nervous system. Duane retraction syndrome drs is a special strabismus with variable presentation. A mutation in transcription factor mafb causes focal. It involves a congenital abnormality of the horizontally acting intraocular muscles, most commonly the lateral rectus, and is not. Surgical and functional outcomes in bilateral exotropic.
In some families with dominant duane syndrome, it has appeared. This is the situation that huber calls duane s syndrome. Duane s retraction syndrome drs is a congenital complex strabismus characterized by variable limitation of abduction, variable limitation of adduction, and globe retraction with narrowing of the palpebral fissure on attempted adduction. Duane retraction syndrome drs is a congenital eye movement anomaly characterized by variable horizontal duction deficits, with narrowing. Duane syndrome may be associated with goldenhar syndrome and prenatal thalidomide exposure, a. A modification of hubers classification of the syndrome is suggested in.
Duane retraction syndrome is a congenital cranial dysinnervation disorder characterized by uni or bilateral abduction deficit, narrowing of the palpebral fissure on adduction, and globe retraction with occasional upshoot or downshoot in adduction box 57. In duane retraction syndrome, extraocular findings including deafness, asymmetrical face, cleft palate, outer ear anomalies, preauricular tag. Duaneradial ray syndrome genetics home reference nih. As the eye moves inward, the eyelids partially close and the eyeball pulls back retracts into its socket. The authors present a case with a yet unreported association between duane retraction. The majority of cases of duane syndrome are sporadic occurring in individuals with no history of the condition in the family. Orthoptic department information sheet duane s retraction. Duane s retraction syndrome drs consists of a congenital abduction deficit accompanied by retraction of the globe on attempted adduction and by upshoots or downshoots of the affected eye on adduction. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. Study of duanes retraction syndrome ahluwalia 1988. Clinical analysis, management and outcome of duane s.
In the primary position, the most common type of deviation is esotropy less than 30 ptd, it will manifest if the abduction limitation is. Orthoptic department information sheet duane s retraction syndrome we are committed to making our publications as accessible as possible. Full text management of duane retraction syndrome with. Duane iii shows variable horizontal deviation on primary gaze and has poor adduction and abduction. Duane syndrome presents in several ways, all of which are characterized by anomalous cocontraction of the medial and lateral rectus muscles, producing retraction of the globe in actual or attempted adduction. Duane s retraction syndrome is a congenital deficiency of abduction of the eye associated with variable impairment of adduction, retraction of the globe, narrowing of the. Duane retraction syndrome type 1 with usher syndrome type. This condition prevents outward movement of the eye toward the ear, and in some cases may also limit inward eye movement toward the nose. Surgical management of strabismus in duane retraction syndrome. Results of surgical treatment in patients with different types of duane syndrome duane type meanpd n sd pvalue 1 preoperative 22. Duane retraction syndrome drs, representing duane s retraction syndrome.
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