The syndrome was first described by ophthalmologists Jakob Stilling (1887) and Siegmund Türk (1896), and subsequently named after Alexander Duane, who discussed the disorder in more detail in 1905.. Other names for this condition include: Duane's retraction syndrome . Duane syndrome 1 described here follows an autosomal dominant pattern. The syndrome was first described by ophthalmologists Jakob Stilling (1887) and Siegmund Türk (1896), and subsequently named after Alexander Duane, who discussed the disorder in more detail in 1905. Agenesis of the sixth nerve The 2022 edition of ICD-10-CM H50.811 became effective on October 1, 2021. It was first described by Heuck in 1879 1 and subsequently by several other . Identifier: 1-11 Title: Duane's Syndrome Type 2: Aberrant Regeneration of the Third and Sixth Nerves Subject: Aberrant Regeneration of the Third and Sixth Nerves; Duane's Syndrome Description: Example of a patient with Type 2 Duane's Syndrome. 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 adductionThe condition is caused by aberrant innervation of the lateral rectus by fibers of the oculomotor nerve. Type 2 Only 7% of DS cases are Type 2. This is esotropia Duane's syndrome type 1 (limited abduction, intact adduction). Type A Abduction and adduction are both defective but abduction is more defective than adduction. The aberrant nerve pathway is shown in red, and the dotted lines represent nerve hypoplasia or agenesis. Silly Mommy. Individuals having Duane Retraction Syndrome 2 (DURS2) ( 604356) are often found in autosomal dominant pedigrees also. These symptoms are referred to as Duane's syndrome type I and account for 70-80% of cases. Type 3: Ability to move the eye, or eyes, in both directions (abduction and adduction) is limited. 28. Course: The patient's symptoms and motility examination are consistent with Type III Duane retraction syndrome. Type I is the most common form of Duane syndrome, and affected patients will characteristically have a head turn towards the involved side, and will appear esotropic (crossed-eye) in straight ahead gaze. What are the symptoms. Contralateral face-turn; Exotropia in primary position The incidence of Duane retraction syndrome is approximately 1% of the total cases of strabismus. Duane's Syndrome Type 3 - Exotropia (XT) Clinical Features. The eye opening narrows and the eyeball pulls in when looking inward . Code History Duane's Syndrome Type III: OS limited adduction and abduction retraction in adduction G.Vicente 12. The eye opening narrows and the eyeball pulls in when looking inward toward the nose, however the reverse occurs . Duane Syndrome (or Retraction Syndrome, Stilling-Turk-Duane syndrome) is a rare eye deviation most commonly characterized by the inability of the eye to move outwards. Title: Duane's Syndrome Type I Author: Kathleen B. Digre, M.D., Professor of Ophthalmology and Neurology, Director of Neuro-Ophthalmology, Moran Eye Center, University of Utah School of Medicine Date: 03/03/2015 Keywords / Main Subjects: Duane's Syndrome type 1; Globe retraction; Limited abduction Diagnosis: Duane's Syndrome type 1 Demonstrates limitation of adduction in left eye with normal abduction. Duane's Syndrome Type 2. Type 1 About 78% of all DS cases are Type I. This is the most common type of Duane syndrome. These cases are very rare (if they exist at all), and most cases are actually Duane's Type 3. The eye-opening (palpebral fissure) narrows and the eyeball retracts into the orbit when looking inward toward the nose (adduction). Example of patients with Duane's Retraction Syndrome, Type 1. This unusual congenital form of strabismus was first described by Duane (1905), who collected reports of 54 cases.The condition is bilateral in 20% of cases. The disorder is . 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 . Duane's retraction syndrome (DRS) is a congenital eye movement disorder characterized by abduction deficiency, adduction limitation, globe retraction, and palpebral fissure narrowing on attempted adduction. Duane's Syndrome questions. Synergistic divergence is a rare type of Duane's syndrome with abduction on attempted adduction. Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311 Examples of Hess screen chart result among Duane's Syndrome Type 1. Duane retraction syndrome (DRS) is a unique restrictive variety of strabismus that is present from birth. [1,2,3,4,5,6] DRS is a congenital, incomitant ocular motility disorder characterized by abnormal function of the lateral rectus muscle in the affected eye, together with retraction of the globe and narrowing of the palpebral . Patient had LE Duane's retraction Type C syndrome Type A. Clinically, Duane syndrome is often subdivided into three types, each with associated symptoms. In Duane syndrome type 1, eye movement outward is limited. Diagrammatic representation of misdirection of nerve fibers in Duane's syndrome. Do Duane Syndrome patients have other eye problems? Note the left globe retraction especially on attempted right gaze. Duane syndrome Type 1: The ability to move the affected eye (s) outward toward the ear (abduction) is limited, but the ability to move the affected eye (s) inward toward the nose (adduction) is normal or nearly so. As the eye moves inward, the eyelids partially close and the eyeball pulls back (retracts) into its socket. Isolated Duane retraction syndrome is a disorder of eye movement. Duane syndrome Type 1: The ability to move the affected eye(s) outward towards the ear (abduction) is limited, but the ability to move the affected eye(s) inward towards the nose (adduction) is normal or nearly so.The eye opening (palpebral fissure) narrows and the eyeball retracts into the orbit when looking inward towards the nose (adduction). Type 1 cases were the most common (73%), and bilateral cases often were of different types. Also shows side-view of globe retraction in abduction. 1982; 100(9):1468-72 (ISSN: 0003-9950) Miller NR; Kiel SM; Green WR; Clark AW. EPIDEMIOLOGY Prevalence of about 1/1000 in general population Females (60%) > Males (40%) Accounts for up to 4% of all strabismus cases Most common type of congenital aberrant ocular innervation 80% of cases occur unilaterally, with a LE . It accounts for about 1-5% of total cases of strabismus and affects approximately 1 in 1,000 people throughout the . Description of components of Duane's Syndrome: limitation of abduction, variable limitation of . People with Duane syndrome may also have unusual head movements. PDF | Purpose: The purpose of this study is to create awareness about the face turns in Type 1 Duane's retraction syndrome (DRS). A mild downshoot of the left eye is also evident in right gaze. [2] [4] Duane syndrome has 3 types which vary depending on which type of eye movement is most restricted. The patient had excellent visual acuity with no . Visual acuity, dry refraction, cyclo-refraction, ocular motility, and amount of deviation were measured. Slowed hypometric abduction was found. 2015;52:e26-e29.] I also have duane syndrome I have type 1 in one eye and type 2 in the other and I calculated the odds of this happening to me and came out to be a 0.0000324% of ending up with this severity but it did and my mom didn't even travel during her pregnancy. Diagnosis: Duane's Syndrome Brief Description: This is a video showing the eye movements characteristic of Duane's Syndrome type 1 and demonstrating the examination techniques for diagnosing Duane's Syndrome. Duane's Type 2 is identified as good abduction with poor adduction. Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outward. I have developed lazy loss of hearing in one ear and I am slowly losing vision in my right eye. About 80% to 90% of DS cases involve only one eye — more often the left eye, but sometimes the right. Research Molecular Genetics test for Duane retraction syndrome and CHN1, CHN1, using Linkage analysis, Next-Generation (NGS)/Massively parallel sequencing (MPS) offered by Engle Laboratory. Akbari M, Shomali S, Mirmohammadsadeghi A and Fard M (2018) Augmented superior rectus transposition procedure in Duane retraction syndrome compared with sixth nerve palsy, Graefe's Archive for Clinical and Experimental Ophthalmology, 10.1007/s00417-017-3885-5, 256:5, (983-987), Online publication date: 1-May-2018. For language access assistance, contact the NCATS Public Information Officer. Duane retraction syndrome (DRS) is best thought of as a special type of ocular dysmotility caused by improper innervation, or dysinnervation, of one or more extraocular muscles. Read article at publisher's site (DOI): 10.3928/01913913-20100308-07. In 1974, with the support of electromyography (EMG) and following the suggestions of Lyle and Malbran, Huber classified Duane syndrome into the 3 types: Duane 1, Duane 2, and Duane 3. Shows limitation of abduction in both eyes and adduction in the left eye. [J Pediatr Ophthalmol Strabismus. There is information about participation criteria, enrollment status and contact information for the study. Contralateral face-turn; Exotropia in primary position | Find, read and cite all the research . Full text links . • Eye movements of two patients with Duane's retraction syndrome (type 1) were recorded using high-resolution infrared oculography. As discussed by Al-Baradie et al. Your ability to move your eye toward your ear is limited. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The syndrome was of type I in 87.0%, II in 6.5%, and III in 5.7%; 7.2% were Introduction Duane's retraction syndrome (DRS) is an unusual F. Anvari E. Hatef S.-F. Mohammadi A. Eskandari congenital form of strabismus; its frequency in the Eye Research Center, Farabi Eye Hospital, Tehran general population of strabismus patients is 1-5% . Type 1: The affected eye, or eyes, has limited ability to move outward toward the ear, but the ability to move inward toward the nose is normal or nearly so. Duane syndrome type 1. A classification scheme for DRS is established in the literature; however, variability of dysinnervation results in a spectrum of clinical presentation. Discussion of limited pathological cases. Type 1 Duane Retraction Syndrome Type 2 Duane Retraction Syndrome Type 3 Duane Retraction Syndrome See Also Ophthalmoplegia Public MeSH Note 2000; see DUANE'S SYNDROME 1991-1999, see OPHTHALMOPLEGIA 1979-1990, see OCULOMOTOR PARALYSIS 1966-1978 History Note . Left eye preponderance (67%) was observed and seven (10%) were bilateral. Defective abduction only, adduction is normal. Types of Duane syndrome Duane syndrome is commonly split into three categories, each with its own set of symptoms. First patient is shown gazing left and right to demonstrate these components. Other characteristic features can be for example the failure to move the eye inwards, poor convergence and/or retraction of the eyeball. The botulinum toxin was injected in the medial rectus of all patients. (A) type I: poor abduction and good adduction. Unilateral Duane's retraction syndrome (Type 1) Duane's retraction syndrome (DRS) is a congenital eye movement disorder characterized by adduction deficiency, abduction limitation, globe retraction, and palpebral fissure narrowing on attempted adduction. Clip of patient with Duane's Syndrome Type I. Duane Syndrome Type 1 is the most common type of Duane syndrome, an eye movement disorder that is present at birth People with Duane Syndrome have restricted ability to move the affected eye(s) outward toward the ear (abduction) and/or inward toward the nose (adduction). Duane emphasized thatretraction of the adducted involved globe is an essential clinical feature of this syndrome, and because this feature is so diagnostic, common usage of . Often, the affected eye will remain still or move upward when a person attempts to look toward the nose with the unaffected eye. Duane syndrome type 1: The ability to move the affected eye (s) outward toward the ear (abduction) is limited, but the ability to move the affected eye (s) inward toward the nose (adduction) is normal or nearly so. Clinically, Duane syndrome is often subdivided into three types, each with associated symptoms. Duane's Retraction Syndrome (DRS) was first described by Stilling in 1887, Sinclair in 1895, Bahr in 1896, Turk in 1899, Wolff in 1900 and Alexander Duane in 1905. and 2) What limitations do people with Duane's experience? The eye opening (palpebral fissure) narrows and the eyeball retracts into the orbit when looking inward toward the nose (adduction). There are three types of Duane syndrome, with type 1 being the most common: Type 1: Abduction is limited, but adduction is relatively normal. Where most forms of strabismus leave side-to-side eye movements intact, in Duane syndrome the "wiring" of the eye muscles gets jumbled, and movement of eye (s) is limited in certain directions. There were 25 (36%) affected males and 45 (64%) females. Duane's Type 2 is identified as good abduction with poor adduction. In a case of unilateral DRS, the right side of the brainstem, cavernous sinus, and orbit were completely normal. Research Molecular Genetics test for Duane's syndrome and CHN1, CHN1, using Mutation scanning of the entire coding region, Next-Generation (NGS)/Massively parallel sequencing (MPS) offered by Engle Laboratory. Prior to publication of Alexander Duane's description in 1905 1 this syndrome had been described by Stilling 2 in 1887 and Turk 3 in 1896 and, consequently, should more correctly be called the Stilling-Turk-Duane syndrome. A case is reported of an eight year old girl with a left Duane's retraction syndrome type 1, bilateral dissociated vertical deviation (DVD) and an infantile esotropia. Duane's retraction syndrome (DRS) is a congenital eye movement disorder characterized by adduction deficiency, abduction limitation, globe retraction, and palpebral fissure narrowing on attempted . Type 2: Adduction is limited, but abduction is relatively normal. H50.811 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. Example of patients with Duane's Retraction Syndrome, Type 1. Duane syndrome type 1: Recession of the medial rectus (no effect on abduction), posterior fixation suturing on the normal medial rectus (may diminish adduction innervation slightly to the involved eye, decreasing the esodeviation), or resection involving the lateral rectus (but may turn type 1 into a severe type 2). These cases are very rare (if they exist at all), and most cases are actually Duane's Type 3. This condition prevents outward movement of the eye (toward the ear), and in some cases may also limit inward eye movement (toward the nose). 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 muscles and nerves around your eye don't work well together, and that keeps it from moving as it should. Duane syndrome is a strabismus condition clinically characterized by congenital non-progressive limited horizontal eye movement accompanied by globe retraction which results in narrowing of the palpebral fissure. Type 1: The affected eye, or eyes, has limited ability to move outward toward the ear, but the ability to move inward toward the nose is normal or nearly so. Duane's syndrome, right eye. It is characterized by co-contraction of the medial and lateral rectus muscles due to anomalous innervation of the lateral rectus muscle during embryogenesis. Methods. Duane syndrome (DS) is a type of eye misalignment that affects the movement of one or both of a baby's eyes from the time they are born. Figure 1: Duane Globe Retraction Syndrome, Type III. Type 1 includes limited or absent abduction with normal adduction. Often, when the eye moves toward the . Duane syndrome type 1 comprises 75-80% of patients and the affected eye, or eyes, has limited ability to move outward toward the ear, but the ability to move inward toward the nose is normal or nearly so 28). Duane's Syndrome Type 2. [ 8 , 9 , 10 ] Type 1 is marked limitation of abduction (corresponds to Type B in Brown's classification) explicable by maximum innervation reaching the lateral . [1,2,3,4,5,6] DRS is a congenital, incomitant ocular motility disorder characterized by abnormal function of the lateral rectus muscle in the affected eye, together with retraction of the globe and narrowing of the palpebral . No specific mutant gene has been found but a locus has been identified at 8q13. Arch Ophthalmol. Nystagmus is a rare association with Duane's syndrome. 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. In a case of unilateral DRS, the right side of the brainstem, cavernous sinus, and orbit were … 1 Duane's syndrome represents 1 to 4% of the strabismic population, with left eye and female predisposition. My 2 yr. old son was just diagnosed with Duane's Syndrome, type 1, and I have a few questions: 1) I understand that surgery is needed when there is an 'unacceptable' head turn. Duane syndrome is a congenital (from birth) eye condition that affects an individual's ability to move and align their eyes. There is information about participation criteria, enrollment status and contact information for the study. Duane syndrome — which is also known as Duane's syndrome or Duane retraction syndrome — is a rare form of strabismus, or misalignment of the eye. It typically results in strabismus (abnormal alignment of the eyes) and can cause amblyopia (lazy eye). Duane's syndrome type 2, characterized as good abduction with poor adduction, is very rare (if it exists at all), and most cases are actually Duane's type 3. 1. Type Type Il Duane's Ill MR FIGURE 10-1 IA—D. Duane's syndrome (DS) is a congenital ocular motility disorder characterized by abduction deficiency with variable limitation of adduction and globe retraction on adduction that may be in association with oblique elevation or depression. The syndrome was first described by ophthalmologists Jakob Stilling (1887) and Siegmund Türk (1896), and subsequently named after Alexander Duane, who discussed the disorder in more detail in 1905. This is the third case report of a Duane's syndrome and DVD presenting . The patient also had congenital hydrocephalus which was treated with a vertriculoperitoneal shunt. Multiple mutations in CHN1 have been found among such individuals. Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outward. The clinical features of the 3 types are similar (as listed below) with the following exceptions: Type 1 comprises 75-80% of patients and presents with an esotropia in primary gaze with a compensatory head turn to the involved side. Unilateral Duane's retraction syndrome (Type 1). Nearly 80% of cases are unilateral and characterized by limited abduction (type 1 Duane's syndr Here, we have a case associated with type 1 Duane's syndrome with exotropia. Type IV Duane Syndrome 7 Type IV Duane syndrome has been called simultaneous abduction, synergistic divergence, the "splits", and perversion of the extraocular muscles.1,3,6,7 Type IV Duane syndrome has previously been described in the literature, but only case reports have been reported. Identifier: 1-12 Title: Duane\\'s Retraction Syndrome Type 3 Subject: Duane\\'s Retraction Syndrome Type 3; Lid retraction Description: Example of a patient with Type 3 Duane's Retraction Syndrome, as well as bilateral Duane's Syndrome. Type 1 Duane Retraction Syndrome (n.). There are 3 types of Duane Retraction Syndrome with multiple subgroups existing. The condition is caused by aberrant innervation of the lateral rectus by fibers of the oculomotor nerve. Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outward. The twin pair were recruited through the Australian . This is the American ICD-10-CM version of H50.811 - other international versions of ICD-10 H50.811 may differ. There is information about participation criteria, enrollment status and contact information for the study. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. Duane's Syndrome Type 3 - Exotropia (XT) Clinical Features. Presented at the Neurology Grand Rounds in Fall 2011 at the University of Utah.James Bell, MDMoran Eye Center N. Research Molecular Genetics test for Duane's syndrome and CHN1, CHN1, using Mutation scanning of the entire coding region, Next-Generation (NGS)/Massively parallel sequencing (MPS) offered by Engle Laboratory. Subjects and Methods:. Generally, with treatment, eye movement can be corrected. Sixteen patients with esotropic type 1 Duane syndrome without history of ocular surgery were selected for this interventional case series. There are three subtypes: type 1 (associated with loss . This report describes the clinical findings in 70 cases of Duane's retraction syndrome. Description of components of Duane's Syndrome: limitation of abduction, variable limitation of adduction, and palpebral fissure narrowing and globe retraction with attempted adduction. Duane syndrome type 1 (Concept Id: C0994516) Duane syndrome is a strabismus condition clinically characterized by congenital non-progressive limited horizontal eye movement accompanied by globe retraction which results in narrowing of the palpebral fissure. Duane's Retraction Syndrome (DRS) was first described by Stilling in 1887, Sinclair in 1895, Bahr in 1896, Turk in 1899, Wolff in 1900 and Alexander Duane in 1905. What makes a head turn 'unacceptable'? (2002), Duane syndrome type 1 refers to marked or complete limitation of abduction with minimal or no limitation of adduction.Duane syndrome type 2 refers to marked or complete limitation of adduction . Duane syndrome (DS) is a rare eye disorder some people are born with. People with Duane syndrome usually have difficulty moving one of eyes away from the nose. Duane's retraction syndrome (DRS) is a congenital, incomitant ocular motility disorder characterized by abnormal function of the lateral rectus muscle in the affected eye, together with retraction of the globe and narrowing of the palpebral fissure on attempted adduction. There is information about participation criteria, enrollment status and contact information for the study. The dynamics of adducting saccades in the affected hypometric eye were normal, suggesting an absence of functional cocontraction of the medial and lateral rectus muscles. 15.1 Introduction. Duane's Syndrome Type II Strabismus, Ocular Motility Disorders -> Ophthalmoplegic Hereditary Syndromes. It's typical or nearly normal for you to be able to move your eye toward your nose. If you have problems viewing PDF files, download the latest version of Adobe Reader. This video is part of the . Optometric impression Type B follows Lyle's classification. We report a single case study of concordant bilateral Duane's Retraction Syndrome (DRS) (type 1) in female monozygotic (MZ) twins aged 47 years. The eye opening narrows and the eyeball pulls in when looking inward toward the nose, however the reverse occurs when looking outward toward . Research Molecular Genetics test for Duane retraction syndrome and CHN1, CHN1, using Linkage analysis, Next-Generation (NGS)/Massively parallel sequencing (MPS) offered by Engle Laboratory. Duane's Syndrome (#1) Primary Position: Duane's Syndrome (#2) Left Gaze: Duane's Syndrome (#3) Right Gaze: thumbnails view: Duane's syndrome: [dwānz] Etymology: Alexander Duane, American ophthalmologist, 1858-1926 an autosomal-dominant syndrome in which the affected eye shows limitation or absence of abduction, restriction of adduction, retraction of the globe on adduction, narrowing of the palpebral fissure on adduction and widening on abduction, and deficient . 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