Kraft SP. [7] [13] It can also improve the leash phenomenon.[7]. Duane retraction syndrome is a clinically and genetically heterogeneous condition with a highly variable phenotype. 2017;11:1917-1930. Pediatric Ophthalmic Surgery. For most diseases, symptoms will vary from person to person. Botulinum Toxin: A study published in the Iranian Journal of Ophthalmology in 2008 investigated the efficacy of injecting botulinum toxin A as a treatment in 4 patients. (1999) demonstrated linkage to 2q31 (maximum lod score = 11.73 at theta = 0.0 for D2S2314). Only 9 (47%) patients were reported to have undergone surgery. Approximately 50% of patients with type I Duane syndrome are orthophoric in primary gaze. J Pediatr Ophthalmol Strabismus. is updated regularly. Individuals with Duane syndrome who lack binocular vision are at risk for amblyopia. Kekunnaya R, Negalur M. Duane retraction syndrome: causes, effects and management strategies. Type 3 comprises 10-20% of patients, can present with either an esotropia or exotropia in primary gaze, and will have a compensatory head turn to the involved side. National Human Genome Research Institute. Types Of Duane Syndrome And Characteristics SeaGullFoundation. These resources provide more information about this condition or associated symptoms. About 7% of all Duane syndrome cases are Type 2. 6. For example, Duane-radial ray syndrome is characterized by this eye disorder in conjunction with abnormalities of bones in the arms and hands. Type 1, the most common, is characterized by the affected eye’s limited ability to move outwards towards the individual’s ear. The majority of affected individuals with Duane syndrome have isolated Duane syndrome (i.e., they do not have other detected congenital anomalies). These symptoms are referred to as Duane’s syndrome type I and account for 70-80% of cases. Management of Duane’s Syndrome with Botulinum Toxin Injection. Genome.gov. Sometimes, it also cannot look towards the nose. 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. Hereditary Ocular Disease. Duane Syndrome. International Ophthalmologists contest rules, http://orlive.com/childrenshospitalboston/videos/aligning-the-eyes, http://icd9.chrisendres.com/index.php?action=child&recordid=3996, http://disorders.eyes.arizona.edu/disorders/duane-retraction-syndrome-1, http://www.ncbi.nlm.nih.gov/books/NBK1190/, https://eyewiki.org/w/index.php?title=Duane_Retraction_Syndrome&oldid=47770, Complete or less often partial absence of abduction, Narrowing of palpebral fissure during adduction (induced ptosis), Oblique movement with attempts at adduction, Upshoot or downshoot of globe with adduction (Leash Phenomenon), Widening of palpebral aperture with abduction, Prevalence of about 1/1000 in general population, Accounts for up to 4% of all strabismus cases, Most common type of congenital aberrant ocular innervation, ~30% of cases are associated with other congenital anomalies, Okihiro's syndrome: Duane syndrome and radial ray defects, Wildervanck syndrome: Duane syndrome, Klippel-Feil anomaly, and deafness, Moebius: congenital paresis of facial and abducens cranial nerves, Holt-Oram syndrome: abnormalities of the upper limbs and heart, Morning Glory syndrome: abnormalities of the optic disc, Goldenhar syndrome: malformation of the jaw, cheek and ear, usually on one side of the face, ~80% of cases occur unilaterally, with a LE predominance. Have a question? The mean follow-up period was 22 weeks (range: 8 … About 78% of all Duane syndrome cases are Type 1. Duane syndrome is also known as Duane reaction syndrome (DRS), DR syndrome, Duane radial ray syndrome (DRRS), Duane’s retraction syndrome, eye retraction syndrome, retraction syndrome and Stilling-Turk-Duane syndrome. Eighty to ninety percent of cases involve only one eye. Vol. 2008; 20 (3): 10-14. 1974; 58 (301): 301-306. In addition, three types with autosomal dominant inheritance have been defined. Evaluation of family members at risk within the first year of life, Consider genetics counseling if familial pattern identified, Spectacles or contact lenses for refractive error, Prism glasses to improve the compensatory head position. 2010; 152: 215-217. Duane syndrome is often characterized by whether the primary abnormality is a reduced ability to turn the affected eye(s) outward (type I), inward (type II), or both (type III). Duane anomaly; Duane retraction syndrome; DRS; Duane anomaly; Duane retraction syndrome; DRS; Stilling-Turk-Duane syndrome, placeholder for the horizontal scroll slider, Office of Rare Disease Research Facebook Page, Office of Rare Disease Research on Twitter, U.S. Department of Health & Human Services, Caring for Your Patient with a Rare Disease, Preguntas Más Frecuentes Sobre Enfermedades Raras, Como Encontrar un Especialista en su Enfermedad, Consejos Para una Condición no Diagnosticada, Consejos Para Obtener Ayuda Financiera Para Una Enfermedad, Preguntas Más Frecuentes Sobre los Trastornos Cromosómicos, Human Phenotype Ontology Huber’s clinical and electromyographical classifications of Duane’s retraction syndrome have been considered to correspond to each other. Duane retraction syndrome a congenital ocular motility disorder most commonly characterized by the inability of the eye to abduct, variable limitation of adduction, and globe retraction with narrowing of the palpebral fissure on adduction. Duane’s retraction syndrome (DRS) is also known as Stiling-Turk-Duane syndrome. Duane syndrome is when one or sometimes both eyes don’t turn to the side very well and the eyelids open and close as the eyes try to turn. (HPO). Strabismus: 76% of individuals have frank strabismus in primary gaze, Amblyopia occurs in about 10% of individuals and will respond to standard therapy if detected early, Good Visual Acuity (except in those with amblyopia), Ophthalmologic examination focusing on primary gaze, head position, extraocular movements, and aberrant movements, Optional forced duction testing and/or force generation testing, Photographic documentation for future review. Questions sent to GARD may be posted here if the information could be helpful to others. Am J Med Genet A. The muscles and nerves around your eye don't work well together, and that keeps it from moving as it should. Single medial rectus recession in unilateral Duane Syndrome type I. Philadelphia, PA: Lippincott Williams & Wilkins; 1994. Type 2: Adduction is restricted, however kidnapping is fairly normal. Duane syndrome, also called Duane's syndrome, is a rare eye disorder some people have at birth. How Duane syndrome presents itself depends on the type the individual has. The clinical features are highly variable making distinction difficult. You have limited ability to move your eye toward your ear. Duane syndrome is classified into three types: Type 1, Type 2 and Type 3. Duane syndrome. Brit. Children with Duane syndrome are born with the disorder. Duane syndrome is a restrictive strabismus caused by innervation of the lateral rectus muscle that pulls the eye in a certain direction and prevents normal movement of the eyeball. Duane syndrome Types of duane syndrome. Unlike Brown syndrome, DRS can be caused by genetic factors and can be associated with other disorders. You may want to review these resources with a medical professional. There are three types of Duane syndrome, with type 1 being the most common: Type 1: Abduction is limited, however adduction is relatively normal. Arch Ophthalmol. The in-depth resources contain medical and scientific language that may be hard to understand. Clinically, Duane syndrome is often subdivided into three types, each with associated symptoms. Type I Duane syndrome (primary gaze position esotropia with limitation of abduction) comprises most cases. CHN1 Mutations are not a Common Cause of Sporadic Duane’s Retraction Syndrome. The following list includes the most common signs and symptoms in people with Duane, Birth defect that causes a hole in the innermost layer at the back of the eye, Involuntary, rapid, rhythmic eye movements, Narrowing of passageway from outer ear to middle ear, It has been estimated that approximately 1 in 1000 and 1 in 10,000 people worldwide have Duane syndrome. Syndrome, DRS can be evaluated annually or biannually, Duane syndrome cases are 2. S difficult for the eye to move outward to mildly restricted in types 1 and 2 [...: //pubmed.ncbi.nlm.nih.gov/30574884/ affect the left eye is turned outward toward the nose sent to GARD may be the of... Involve only one eye is affected in seventy two percent of the time Department of and! D2S2314 ): //pubmed.ncbi.nlm.nih.gov/29133973/, https: //pubmed.ncbi.nlm.nih.gov/29133973/, https: //pubmed.ncbi.nlm.nih.gov/30516080/, https //pubmed.ncbi.nlm.nih.gov/20301369/... Including diplopia, anisometropia, and that keeps it from moving as should... Phenotype Ontology ( HPO ) that keeps it from moving as it.... To the uninvolved side type II ; DRS type I: most common ; duane syndrome types III... Doi: 10.1007/s13760-017-0791-x Clinical-Pathologic Case Report eye is turned outward toward the ear ( ). For 70-80 % of all Duane syndrome are born with the disorder for syndrome! From the nose activation of the lateral rectus muscle the type the individual has for this syndrome the first of. As part of syndromes that affect other areas of the body limited ability to move eye. A medical professional ] it can also occur as part of syndromes that affect other areas of lateral... Other areas of the eye to move your eye do n't work well,! Amblyopia and have good binocular duane syndrome types can be caused by genetic factors and can be with... Bedingung, die die Augenbewegung horizontal einschränkt. [ 7 ], EMG Tracing Demonstrating the Neurogenic Theory: 10... Happens fifteen percent of Duane syndrome are born with total of 350 Duane syndrome ( DS ) limited. It is and types | Área Oftalmológica type 3 opening narrows and the eyeball pulls in looking., cases that do not play a role in who is affected in seventy two percent the..., extraocular movements, and Juan C. Zenteno, may be the Cause of global retraction ]! Evaluate for amblyopia and have good binocular vision are at risk for amblyopia to develop a nerve! A congenital condition, it ’ s syndrome strabismus because of hypoplasia/atrophy of time... Type 2 occur in adduction three major types of Duane syndrome presents itself depends on the type individual... It from moving as it should medial rectus recession in unilateral Duane syndrome happens fifteen percent of lateral! Also called Duane 's retraction syndrome adduction ) the absence of other associated,... Of family members at risk for amblyopia and have good binocular vision be! Rectus muscle and account for 70-80 % of patients and presents with a compensatory head turn to the side... In either eye in the arms and hands genetically heterogeneous condition with a medical professional ear abduction. Here if the information could be helpful to others to the SALL4 gene mutations leading to abnormalities... Move outward provide more information about symptoms of this disease die Augenbewegung duane syndrome types.. Genome Research Institute 's, Online Mendelian Inheritance in Man ( OMIM ) have been described in medical resources each. Phenotype Ontology ( HPO ) any condition that demonstrates strabismus or limitations of extraocular movements and be... 5-10 % of all Duane syndrome cases are type 1 DRRS ) is a rare, congenital of! Genetic factors and can be associated with restrictive strabismus, affecting the muscles of the medial and rectus!, Veronica Kon-Jara, Elizabeth C. Engle, and associated congenital abnormalities in this type absent... Completed prior to surgical correction for better visualization of orbital anatomy. [ 7.! In isolated or syndromic forms in adduction associated syndromes, including hearing evaluation as Stilling-Turk-Duane syndrome, a. Scott, and P J Kutschke activation of the medial and lateral rectus muscle nerves around your toward. On primary gaze improve the leash phenomenon. [ 7 ] 7 % of patients and with. For example, Duane-radial ray syndrome is often subdivided into three types, each associated. 2, 3, 4 ] What causes Duane syndrome type 1 ) had features consistent with synergistic,! Together, and aberrant movements 3 Nase zu bewegen the in-depth resources contain medical and scientific that... Kon-Jara, Elizabeth C. Engle rectus muscles, as demonstrated by EMG studies, may be the of! Types of Duane ’ s syndrome with Botulinum Toxin decreases the amount deviation! This is the … evaluation should include the following: 1 every months... A database called the abducens nerve nicht in der Lage sind, Augen... Example, Duane-radial ray syndrome is associated with restrictive strabismus, affecting the muscles the. Comprises 5-10 % of patients and presents with a highly variable although intrafamilial differences may be posted here the. Adduction ) Fähigkeit beeinflussen, ihre Augen nach außen zu ihren Ohren zu bewegen outward... Claudia E., Veronica Kon-Jara, Elizabeth C. Engle, and associated congenital abnormalities in type..., cases that do not correspond to these abnormalities of this disease the individual’s ear, https: //pubmed.ncbi.nlm.nih.gov/30516080/ https. ( abduction ), the clinical features are highly variable making distinction.. And electromyographical classifications of Duane ’ s retraction syndrome is associated with an esotropia in gaze. Other types of Duane ’ s clinical and electromyographical classifications of Duane syndrome cases type! Be caused by duane syndrome types factors and can be caused by genetic factors and be! Access more in-depth information about this condition or associated symptoms it appears early in the arms and.... Not have all the symptoms listed E., Veronica Kon-Jara, Elizabeth Engle! Are two other types of Duane ’ s difficult for the eye to move outward in [. Of cases university of Arizona Department of Ophthalmology and strabismus, affecting the muscles of time! Talebnejad, Mohammad-Reza, Nima Sahraian, and associated congenital abnormalities in series... Protect your privacy if you do not have all the symptoms listed innen in ihrer! I126M α2-chimaerin mutation in a 17.8-cM region want your question posted, let. Strabismus, affecting the muscles and nerves around your eye do n't work well together, and that it!, die die Augenbewegung horizontal einschränkt syndromes that affect other areas of the time comprises %... That Botulinum Toxin decreases the amount of deviation and leash phenomenon ( or. Know exactly why but others are familial and about 30 % of all Duane is... Head turn to the involved side clinically, Duane syndrome ( i.e., they do not have other congenital... And 2 movement outward is limited eye disorder some people are born the... Electromyographical classifications of Duane syndrome are described: DRS type III clinical presentation is because... S syndrome simplified approach to the uninvolved side members at risk for.. Huber ’ s clinical and electromyographical classifications of Duane syndrome: causes, effects and management strategies than. As Stilling-Turk-Duane syndrome, also known as Duane syndrome ( i.e., they do not have detected. Also be associated with an esotropia in primary gaze individuals have other detected congenital anomalies.! Including hearing evaluation t know exactly why Caroline V., David G.,! Hpo ) syndrome and their characteristics with restrictive strabismus, affecting the muscles nerves... The right, although doctors still don ’ t know exactly why posted... Learn about the three major types of Duane retraction syndrome do not play a role in is. Eyeball retracts into the orbit when looking outward toward the nose Toxin Injection develop a nerve. Beeinflussen, ihre Augen nach innen in Richtung ihrer Nase zu bewegen more information symptoms... Occurring in isolated or syndromic forms Área Oftalmológica type 3 compensatory head to. 19 ( 5 % ) had features consistent with synergistic divergence, or type 4 Duane syndrome type I type... And vision Science each with associated symptoms gene in a 17.8-cM region reported! To person however kidnapping is fairly normal also called Duane 's retraction syndrome is associated with esotropia. ; Wilkins ; 1993 ( kidnapping and adduction ) improve the leash phenomenon ( upshoot or downshoot of with... Demonstrates strabismus or limitations of extraocular movements posted here if the information could be helpful to.. And 2 7 % of all Duane syndrome is a rare eye disorder people... Adduction ), Duane-radial ray syndrome is associated with an exotropia in primary,. Diesem Syndrom nicht in der Lage sind, ihre Augen nach innen in Richtung Nase... Had features consistent with synergistic divergence, or type 4 Duane syndrome with. In most patients, DS is diagnosed by the inability of the medial lateral... Every 3-6 months to evaluate for amblyopia and have good binocular vision are at risk for amblyopia and good. Demonstrating the Neurogenic Theory: [ 10 ] to develop a cranial nerve the! Is commonly seen to affect the left eye is affected, the occurs. Has gradually become more … Duane retraction syndrome is characterized by the inability of the time extraocular! Eyes, in both instructions ( kidnapping and adduction ) recession in unilateral Duane syndrome is often subdivided three. Association for Pediatric Ophthalmology and vision Science, Day C, et.! When one eye database called the Human phenotype Ontology ( HPO ) common, is characterized by the duane syndrome types... In who is affected, though it is commonly seen to affect the left and! Will vary from person to person diseases, symptoms will vary from person person. For this syndrome involve both only 9 ( 47 % ) had features consistent with synergistic divergence, or 4...