Duane syndrome may be associated with goldenhar syndrome and prenatal thalidomide exposure, a. A simplified approach to the treatment of duanes syndrome. 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. 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. Both autosomal dominant most commonly and autosomal recessive forms of duane syndrome have been documented. However, researchers have identified mutations in one gene, chn1, that cause the disorder in a small number of families. Duane retraction syndrome drs is a form of congenital strabismus related to impairment of extraocular muscle innervation. In this article, you will learn about the condition, its impact on daily living, and when it requires treatment. Orthoptic department information sheet duane s retraction.
Surgical management of strabismus in duane retraction syndrome. Open access case report duane retraction syndrome a. 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. Drs is a congenital form of strabismus with an incidence of 0. The aim was to study the clinical characteristics of duanes retraction syndrome drs in nepalese patients. Volpe, in volpes neurology of the newborn sixth edition, 2018. The evaluation of patients of duane retraction syndrome regarding the clinical and anatomical aspects, putting them in consideration for the surgical plan of management. 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. In duane retraction syndrome, extraocular findings including deafness, asymmetrical face, cleft palate, outer ear anomalies, preauricular tag. The clinical observations made on 20 patients 24 eyes of duane s retraction syndrome are presented.
Horizontal eye movement can be limited to various degrees of severity in both abduction and adduction. Duane radial ray syndrome is a disorder that affects the eyes and causes abnormalities of bones in the arms and hands. Pdf surgical treatment of duane retraction syndrome. The syndrome is classified in three types based on the presence of defect in adduction, abduction or both. 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. Duane s retraction syndrome or dr syndrome, eye retraction syndrome, retraction syndrome, congenital retraction syndrome and stillingturk duane syndrome. In 70 percent of ds cases, this is the only disorder the individual has.
As the eye moves inward, the eyelids partially close and the eyeball pulls back retracts into its socket. 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 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. About 10% of affected individuals appear to have other affected family members. Duane s retraction syndrome motility disorder featuring. Ananda andy kalevar, md michael flanders, md mcgill. A mutation in transcription factor mafb causes focal. Reoperation in esotropic duane retraction syndrome. Duane retraction syndrome type 1 with usher syndrome type. 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 is a congenital eye movement anomaly characterized by variable horizontal duction deficits, with narrowing. Duane syndrome affects the eye muscles so that the ability to move the eye inward, outward, or in both directions is limited. All types show globe retraction on adduction and lid fissure narrowing on adduction. Duane retraction syndrome drs is a congenital eye movement anomaly characterized by variable horizontal duction deficits, with. Duane retraction syndrome drs is a congenital complex strabismus. However, it was later made popular by alexander duane who published a series of 54 patients having this condition. This is the situation that huber calls duane s syndrome. Duane retraction syndrome is a spectrum of eye motility disorders whose common feature is retraction of the affected eye on adduction. Surgical and functional outcomes in bilateral exotropic. 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. Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outwards. It is characterised by restriction of outward andor inward movement of.
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. Information for patients duanes retraction syndrome this leaflet describes duane s retraction syndrome, its causes and possible treatment. 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. A congenital form of strabismus characterized by limited horizontal eye movement accompanied by globe retraction and palpebral fissure narrowing on attempted. Prospective study of 198 patients having duane retraction syndrome. Duane syndrome genetic and rare diseases information. Pdf duane retraction or cocontraction syndrome is a congenital restrictive strabismus which can occur either as an isolated entity or in. Duane retraction syndrome is a congenital strabismus syndrome occurring in isolated or syndromic forms.
Duane syndrome is an uncommon congenital disorder of ocular motility with the following characteristics. It is characterized by cocontraction of horizontal recti on attempted adduction causing globe retraction along with variable amounts of. Full text management of duane retraction syndrome with. Pdf duanes retraction syndrome clinical presentation. Article 4 vision therapy for convergence insufficiency co. 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. Information for patients duanes 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 associated with a small x. It involves a congenital abnormality of the horizontally acting intraocular muscles, most commonly the lateral rectus, and is not. However, other conditions and syndromes have been found in association with ds.
Type i with marked limitation of abduction, type ii with. 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. The authors present a case with a yet unreported association between duane retraction. While duane retraction syndrome drs is relatively common, surgical management of the associated strabismus can be challenging because of. 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. Study of duanes retraction syndrome ahluwalia 1988.
Duane retraction syndrome associated with a small x chromosome deletion volume 43 issue 3 khaled k. This condition prevents outward movement of the eye toward the ear, and in some cases may also limit inward eye movement toward the nose. Specifically, the protein appears to be critical for the. In most people with isolated duane retraction syndrome, the cause of the condition is unknown. A modification of hubers classification of the syndrome is suggested in.
Duane iii shows variable horizontal deviation on primary gaze and has poor adduction and abduction. 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. 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. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the. The majority of cases of duane syndrome are sporadic occurring in individuals with no history of the condition in the family. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. 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 retraction syndrome an overview sciencedirect topics. The incidence of duane s syndrome is 1% to 4% of strabismus patients. Duane retraction syndrome, egyptian study medcrave online.
Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. This abnormality results from the improper development of certain nerves that control eye movement. Duane retraction syndrome drs is a special strabismus with variable presentation. In rare forms of duane s syndrome there can also be other problems, for. 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.
Pdf properties of saccades in duane retraction syndrome. This condition was first described by stilling in 1887. In the primary position, the most common type of deviation is esotropy less than 30 ptd, it will manifest if the abduction limitation is. In some families with dominant duane syndrome, it has appeared.
Orthoptic department information sheet duane s retraction syndrome we are committed to making our publications as accessible as possible. Duaneradial ray syndrome genetics home reference nih. The chn1 gene provides instructions for making a protein that is involved in the early development of the nervous system. Duane retraction syndrome durs is a congenital disorder characterized by restricted horizontal eye movement with globe retraction and palpebral fissure narrowing on attempted adduction. This condition is characterized by a particular problem with eye movement called duane anomaly also known as duane syndrome. Duane retraction syndrome drs, representing duane s retraction syndrome. Drs is characterized by restriction of horizontal outward eye movements abduction, with eyeball retraction upon adduction.
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