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Duane retraction syndrome —Case Report
Published in Jan-Tjeerd de Faber, 28th European Strabismological Association Meeting, 2020
Susanna Pelle, Agnes Sallai, Katalin Virágos Kis
Introduction: The Duane retraction syndrome (DRS)is an autosomal dominant hereditary disease, with a sporadical manifestation. The cause of pathological innervation of muscles of the bulbus is due to the congenital defect of intracranial centers. Instead of sixth cranial nerve, the lateral rec- tus muscles are innervated by the third cranial nerve. Main symptoms are: decreased abduction and adduction of the affected eye, narrowing of palpebral fissure and retraction of the bulbus oculi. There are three types by Huber according the clinical symptoms. We present a girl of 6 years suffering a bilateral form of DRS.
Eyelids
Published in Mostafa Khalil, Omar Kouli, The Duke Elder Exam of Ophthalmology, 2019
Omar Kouli, Mostafa Khalil, Stewart Gillan
A sporadic condition characterized by upper lid coloboma, microphthalmia, optic disc coloboma, maxillary and mandibular hypoplasia, and limbal dermoids (smooth yellow subconjunctival mass, typically at the inferotemporal limbus with hair protrusion). May be associated with Duane retraction syndrome.
Contralateral lateral rectus muscle recession in a patient with unilateral exotropic Duane retraction syndrome type II: A case report
Published in Strabismus, 2021
Keli Mao, Xiaohe Yan, Kun Ding, Lifei Chen, Xiaoming Lin
Duane retraction syndrome (DRS) is a rare congenital eye movement disorder caused by hypoplasia or absence of the sixth cranial nerve and nuclei combined with dysinnervation of one or two branches of the oculomotor nerve.1 It is characterized by horizontal ocular motility deficit, ocular deviation in the primary position, retraction of the globe, and narrowing of the palpebral fissure during attempted adduction, which are caused by the simultaneous contraction of the medial and lateral rectus muscles.2,3 Associated in many cases of DRS is an upshoot or downshoot: a “flipping” up or down movement which occurs when the eye moves above or below the horizontal plane in an adducted position. This abnormality is considered to be related to a sudden slippage of the tight lateral rectus muscle over the globe.4 Patients who showed esotropia or exotropia commonly adopt a face turn to maintain binocular single vision and make up for the duction deficit, depending on which duction is maximally affected.5,6 Huber categorized DRS into three types according to electrophysiology, among which type II (4–13%) is the least common presentation and presents with poor eye adduction and exotropia.1
Binocular Function in Subjects with Orthotropic Duane Retraction Syndrome
Published in Journal of Binocular Vision and Ocular Motility, 2019
Bhagya Lakshmi Marella, Ruby Moharana, Ramesh Kekunnaya
Duane retraction syndrome (DRS), also known as Stilling–Turk–Duane syndrome, is a rare form of congenital ocular motor disorder. Prevalence of DRS in the general population is 0.1% and it peaks to 1–4% of all strabismus cases.1,2 In the literature, various theories have been proposed to explain the pathogenesis of DRS. First, studies put forward myogenic theory. Studies advocating myogenic theory believed that fibrosis of the lateral rectus muscle leads to the dual insertion of the medial rectus, which causes movement restriction and the retraction of the globe on adduction.3 Further, electrophysiological studies revealed that absence of the abducens motor neurons on the ipsilateral side of the affected eye results in the aberrant innervation of the lateral rectus by the third cranial nerve.4,5 The concept of the congenital cranial dysinnervation syndrome (CCDS) has been evolved in last decade. Parsa et al. in 1998 showed the absence of sixth nerve in cases of unilateral Type 1 DRS which subsequently led to fibrosis of the extraocular muscles and the concept of CCDS.6,7
Duane Retraction Syndrome: Clinical Features and a Case Group-Specific Surgical Approach
Published in Seminars in Ophthalmology, 2019
Abuzer Gunduz, Ercan Ozsoy, Pamuk Betul Ulucan
Duane retraction syndrome (DRS) is a congenital ocular movement disorder characterized by moderate-to-severe limitation in abduction and/or adduction, as well as narrowing of the palpebral fissure, globe retraction, and vertical deviation of the affected eye on adduction.1 There is often a congenital anomaly of the sixth cranial nerve nuclei, with aberrant innervation supplied from the third cranial nerve.2 DRS is the most common form of congenital cranial dysinnervation disorders .3 It has an incidence of approximately 0.1% of the general population and accounts for 1–5% of all strabismus cases.4,5 DRS is primarily unilateral, although it presents bilaterally in 15% of all DRS patients.6 DRS type I is characterized by limited abduction, type II by limited adduction, and type III by limited abduction and adduction.7