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Duane Retraction Syndrome (Duane Syndrome)

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A syndrome characterized by marked limitation of abduction of the eye, variable limitation of adduction and retraction of the globe, and narrowing of the palpebral fissure on attempted adduction. The condition is caused by aberrant innervation of the lateral rectus by fibers of the OCULOMOTOR NERVE.

Also Known As:

Duane Syndrome; Syndrome, Duane; Duanes Syndrome; Syndrome, Duane's; Duane Retraction Syndrome 1; Duane Retraction Syndrome 2; Duane Retraction Syndrome, Type 1; Duane Retraction Syndrome, Type 2; Duane Retraction Syndrome, Type 3; Duane's Syndrome; Okihiro Syndrome; Type 1 Duane Retraction Syndrome; Type 2 Duane Retraction Syndrome; Type 3 Duane Retraction Syndrome; Ocular Retraction Syndromes; Retraction Syndrome, Duane; Retraction Syndrome, Ocular; Retraction Syndromes, Ocular; Syndrome, Duane Retraction; Syndrome, Ocular Retraction; Syndrome, Okihiro; Syndromes, Ocular Retraction; Ocular Retraction Syndrome

Networked: 93 relevant articles (4 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Esotropia (Esophoria)
2.	Paralysis (Palsy)
3.	Torticollis (Wryneck)
4.	Deafness (Deaf Mutism)
5.	Hearing Loss (Hearing Impairment)

Experts

1.	Engle, Elizabeth C: 5 articles (04/2012 - 11/2002)
2.	Kohlhase, Jürgen: 5 articles (05/2007 - 11/2002)
3.	Khan, Arif O: 3 articles (06/2015 - 06/2006)
4.	Chan, Wai-Man: 3 articles (04/2012 - 11/2002)
5.	Demer, Joseph L: 3 articles (08/2011 - 11/2006)
6.	Demircan, A: 2 articles (06/2015 - 10/2013)
7.	Gokyigit, B: 2 articles (06/2015 - 10/2013)
8.	Oystreck, Darren T: 2 articles (06/2015 - 03/2014)
9.	Pekel, G: 2 articles (06/2015 - 10/2013)
10.	Abu-Amero, Khaled K: 2 articles (06/2015 - 03/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Duane Retraction Syndrome:

1.	Carbon MonoxideIBA 06/01/2015 - "Vertical rectus transposition in Duane's syndrome: does co-contraction worsen?" 06/01/2015 - "Reply: Vertical rectus transposition in Duane's syndrome: does co-contraction worsen?" 10/01/2004 - "In Duane syndrome, lateral rectus inactivation markedly reduced co-contraction and globe retraction. " 03/01/2002 - "This case also suggests the possibility that some part of the upshoot in adduction in Duane's syndrome is due to the co-contraction of the superior and medial rectus muscles due to this innervation anomaly." 08/01/2009 - "Patients with Duane syndrome had improved adduction, elimination of co-contraction, and decreased torticollis. "
2.	oxaceprol (AHP)IBA 10/01/2013 - "In Duane syndrome patients, the deviation was reduced by a mean of 95%, the AHP was eliminated in 86% of patients, the abduction was improved by 42%, and a useful BSVF of ≈ 67% of normal was achieved at 1 year post operation. " 01/01/2004 - "Unilateral or bilateral horizontal rectus muscle recession offers a simple and effective surgical option for eliminating AHP and is our treatment of choice in patients with Duane's syndrome." 10/01/2013 - "VRT surgery with posterior fixation is an effective treatment method for complete sixth nerve palsy and Duane syndrome with esotropia, AHP, and abduction deficiency. " 01/01/2004 - "Patients with Duane's syndrome undergoing strabismus surgery of a horizontal muscle recession procedure, medial rectus recession for an esodeviation or lateral rectus recession for an exodeviation, in order to correct an abnormal head position (AHP) and a significant tropia in primary position were identified. " 01/01/2011 - "There are ocular diseases which diagnosis is straightforward for general practitioner, pediatricians or pediatric surgeons (horizontal nystagmus, lateral rectus paralysis, ptosis, esotropia), but others less obvious (superior oblique paralysis, Duane syndrome, A and V-pattern strabismus, torsional nystagmus) because of the compensatory head posture, and these last disorders are predisposed to confusion with congenital AHP Interdisciplinary collaboration between ophthalmologist, pediatrician, pediatric surgeon, ENT specialist and neurologist is mandatory in establishing the etiology of AHP Every child with AHP must be examined by an ophthalmologist."
3.	Botulinum Toxins (Botulinum Toxin)IBA 03/01/2010 - "This is the first study to explore the diagnostic role of botulinum toxin in Duane syndrome. " 03/01/2013 - "To report the results of botulinum toxin (BOTOX®, Allergan) type A (BTA) bilateral injection in the treatment of Duane retraction syndrome (DRS) type 1 in patients aged up to 3 years. " 03/01/2013 - "Botulinum toxin treatment in patients up to 3 years of age who have esotropic Duane retraction syndrome." 03/01/2010 - "This article is a retrospective review of patients with Duane syndrome treated with botulinum toxin using the toxin clinic database between 1980 and 2007. " 03/01/2010 - "Diagnostic use of botulinum toxin in patients with Duane syndrome."
4.	Transcription Factors (Transcription Factor)IBA 12/01/2007 - "The authors used magnetic resonance imaging (MRI) to study extraocular muscles (EOMs) and nerves in Duane-radial ray (Okihiro) syndrome (DRRS) caused by mutations in the transcription factor SALL4. " 07/01/2003 - "We have recently shown that Okihiro syndrome results from mutation in the putative zinc finger transcription factor gene SALL4 on chromosome 20q13.13-13.2. "
5.	Wildervanck syndromeIBA 10/01/2014 - "Wildervanck syndrome: an uncommon cause of Duane syndrome." 04/01/2014 - "We describe a case with Wildervanck syndrome (cervico-oculo-acoustic syndrome) comprising Klippel-Feil anomaly, retractio bulbi (Duane syndrome), and congenital sensorineural deafness. " 03/01/2014 - "Wildervanck Syndrome (WS; cervico-oculo-acoustic syndrome) consists of Duane retraction syndrome (DRS), the Klippel-Feil anomaly, and congenital deafness. " 07/01/2010 - "We report a case of Wildervanck syndrome exhibiting Klippel-Feil anomaly, Duane retraction syndrome and deafness. " 12/01/1985 - "A 12-year-old boy with the cervico-oculo-acoustic syndrome, which comprises Duane retraction syndrome, Klippel-Feil anomaly (fused cervical vertebrae) and congenital hearing loss, is described. "
6.	Duane anomalyIBA 05/11/2007 - "Loss of function mutations in SALL4 cause Okihiro syndrome, an autosomal dominant disorder characterised by radial ray malformations associated with Duane anomaly. " 03/01/2006 - "Okihiro syndrome (OS) is defined by forelimb defects associated with the eye disorder Duane anomaly and results from mutations in the gene SALL4. " 02/01/2006 - "Mutations in SALL4 have been identified in patients with Okihiro syndrome, which is characterized by radial ray anomalies associated with a Duane anomaly. " 03/01/2003 - "[Familial congenital bilateral Duane's retraction syndrome (Stilling-Turk-Duane syndrome). " 11/01/2000 - "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. "
7.	Marcus Gunn phenomenonIBA 01/01/2010 - "The authors describe a 17-month-old boy with Duane retraction syndrome type I, Marcus Gunn Jaw-winking, and crocodile tears in the same eye and discuss the proposed mechanisms of these conditions." 01/01/2010 - "Duane retraction syndrome type I, Marcus Gunn jaw-winking and crocodile tears are all syndromes of congenital aberrant innervation. " 01/01/2010 - "Duane retraction syndrome type I, Marcus Gunn jaw-winking and crocodile tears in the same eye." 01/01/2001 - "The documented association of the jaw winking with Duane syndrome as well as the similar laterality and sex distribution data are an interesting phenomenon." 09/01/2014 - "A 5-year-old girl presented with bilateral familial vertical Duane retraction syndrome with alternating esotropia, elevation deficit, Marcus gunn phenomenon, and facial hypoplasia. "
8.	Thalidomide (Thalomid)FDA Link 09/01/2011 - "We found that Duane syndrome and its variants were prominent in individuals who were exposed to thalidomide early in the sensitive period (days 20 to 26±). " 08/01/2009 - "Although structural anomalies of the eye can occur from thalidomide exposure, the most frequent eye complication is secondary to damage to the cranial nuclei in the brain stem, resulting in aberrant neurologic connections causing a condition of abnormal ocular movement, Duane syndrome. " 04/01/2013 - "Thromboembolism and congenital malformations: from Duane syndrome to thalidomide embryopathy." 07/01/2003 - "Mutations at the SALL4 locus on chromosome 20 result in a range of clinically overlapping phenotypes, including Okihiro syndrome, Holt-Oram syndrome, acro-renal-ocular syndrome, and patients previously reported to represent thalidomide embryopathy."
9.	Retinaldehyde (Retinal)IBA 03/01/2013 - "Intracranial AVM might have affected the normal vascular development during the second month of gestation, causing involutional changes of the sixth nerve and resulted in Duane's syndrome by "steal phenomenon." Retinal AVM may point to concomitant intracranial AVMs, thus warranting neurological assessment. " 01/01/2012 - "Our analyses suggest that Okihiro Syndrome may affect retinal nerve fiber layer development and visual acuity." 04/01/2007 - "Left-sided Duane's syndrome and retinal coloboma associated with contralateral microphthalmia." 03/01/2009 - "Duane retraction syndrome, nystagmus, retinal pigment epitheliopathy and epiretinal membrane with micro- and pachygyria, developmental delay, hearing loss and craniopharyngioma." 01/01/2012 - "Two siblings diagnosed with Okihiro Syndrome (also named Duane Radial Ray Syndrome) associated with ophthalmic manifestation including Duane Syndrome and retinal nerve fiber layer (RNFL) hypoplasia were presented. "
10.	Schmid-Fraccaro syndromeIBA 02/01/2007 - "Duane syndrome associated with the Cat Eye syndrome: a case report." 10/01/1977 - "Using multiple chromosomal banding techniques, we studied a child with typical cat-eye syndrome and ocular retraction syndrome. " 11/01/2006 - "Duane's syndrome and 22 marker chromosome: a possible cat-eye syndrome." 01/01/1994 - "The Duane anomaly may occur in association with various partly overlapping syndromes that may be part of the same clinical spectrum, as the cervico-oculo-acoustic syndrome, the cat eye syndrome and syndromes inherited in an autosomal dominant fashion: the acro-reno-ocular syndrome and the Okihiro syndrome. "

Therapies and Procedures

1.	Sutures (Suture) 01/01/2014 - "The Y-splitting procedure has been used both to treat up-shoots and down-shoots in Duane syndrome, and as a substitute for posterior fixation suture. " 05/01/2005 - "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. " 05/01/2005 - "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. " 04/01/2001 - "Subjects with Duane syndrome and esotropia in primary position who had undergone augmented transposition of the vertical rectus muscles obtained improved head position and better alignment in primary position and had a reduction in the incidence of reoperation for undercorrection when compared with similar patients who had undergone vertical rectus muscle transposition without posterior lateral augmentation sutures." 07/01/2002 - "Full tendon rectus muscle transposition surgery augmented with posterior fixation sutures has been shown to be effective in the treatment of abducens palsy and Duane's syndrome. "
2.	Denervation 01/01/2001 - "Duane's syndrome with compressive denervation of the lateral rectus muscle." 11/01/2006 - "The sparing of the LR in Duane syndrome from denervation atrophy despite absence of normal abducens innervation suggests existence of alternative LR innervation. "
3.	Tenotomy 01/01/2011 - "Surgical management techniques discussed include: 1) excyclotorsion of the muscle cones inducing elevation in adduction craniosynostosis, and surgery to stabilize the globe vertically in adduction in this situation; 2) loop myopexy to prevent prolapse of the elongated, highly myopic eye posteriorly between the superior and lateral recti; 3) superior oblique tenotomy and lengthening procedures to address inability to elevate the adducted eye in Brown syndrome; 4)Y-splitting of the lateral rectus to stabilize the adducted globe vertically in Duane retraction syndrome; and 5) re-recession of the anteriorized inferior oblique to a position posterior to the inferior rectus insertion in anti-elevation syndrome. "
4.	Injections 09/01/1991 - "Injections of botulinum toxin into the oculomotor muscles was used in incorrectible diplopia, ocular torticollis, Duane's syndrome and congenital nystagmus. "
5.	Eyeglasses (Spectacles) 12/01/2013 - "Occasionally, torticollis in Duane syndrome can be satisfactorily corrected with spectacles alone."