Duane retraction syndrome may occur as an isolated phenomenon or present as a syndrome. Isolated form: This comprises 90% of cases which are sporadic and unilateral. [aimu.us]

Case presentation A 6-month-old boy presented with esotropia which had been noticed in early infancy. He showed limited abduction, fissure narrowing on attempted adduction and a small upshoot OS. [bmcophthalmol.biomedcentral.com]

Various characteristics studied included sex, age at presentation, laterality, manifest primary position horizontal deviation, upshoot and downshoot, amblyopia, and associated ocular and systemic abnormalities. [ncbi.nlm.nih.gov]

• Short Stature

  RTS is a multisystem condition characterized by psychomotor developmental delay, broad thumbs and great toes, short stature, and characteristic facies with a beaked nose. [ncbi.nlm.nih.gov]

  Ruptured aneurysm of the sinus of Valsalva with Wildervanck syndrome (cervico-oculo-acoustic syndrome), blepharoptosis and short stature: case report. Circ J. 2007 Sep;71(9):1485-7. Tubbs RS, Oakes WJ, Blount JP. [rarediseases.org]

• Diplopia

  Paretic From Restrictive Etiologies of Diplopia Keywords / Main Subjects: Duane’s Syndrome; Duanes Syndrome type 1; Globe retraction; diplopia Diagnosis: Duane’s Syndrome Brief Description: This is a video showing the eye movements characteristic of Duane [morancore.utah.edu]

  SRT+MRc improved esotropia, head position, abduction limitation, and stereopsis without inducing torsional diplopia. [ncbi.nlm.nih.gov]

  She was managed conservatively since there was no diplopia in primary and reading position, and she did not have an abnormal head posture. [panafrican-med-journal.com]

  He had compensatory face turn to the right to eliminate diplopia (Figure 1). The patient had diplopia on left and right gaze. Ophthalmologic examination showed marked limited adduction and abduction in his left eye. [clinmedjournals.org]

• Lacrimation

  This comprehensive book includes significant sections on various structures of the visual system, covering anterior chamber, choroid, conjunctiva, cornea, globe, iris and ciliary body, lacrimal system, lens, optical nerve, orbit, sclera and vitreous. [books.google.de]

  (Lacrimal Apparatus Diseases) Arcus Senilis Arcus, Corneal (Arcus Senilis) Asthenopia Astigmatism Astrocytoma, Malignant, Optic Nerve (Optic Nerve Glioma) Atrophies, Gyrate (Gyrate Atrophy) Atrophies, Hereditary Optic (Optic Atrophies, Hereditary) Atrophy [provisu.ch]

  Anterior Chamber Brain Choroid Congenital Syndromes Conjunctiva Cornea Glaucomas Hypertensive Retinopathy iPod Beiträge Iris and Ciliary Body Lacrimal System Lens Lids Neuro-Ophthalmology Optic Nerve Orbit Retina Sclera Strabismus Cranial Nerve Palsies [atlasophthalmology.net]

• Blepharoptosis

  No additional anomalies such as fourth (trochlear) nerve palsy, blepharoptosis, or dense amblyopia, which have been reported in previous families with Duane syndrome, were observed. [ncbi.nlm.nih.gov]

  Association with blepharoptosis is seen here and has been frequently associated with Duane. [1] Also association of Duane retraction syndrome is seen with facial hypoplasia which has only once been described. [7] Vertical retraction is also seen to be [ojoonline.org]

  Ruptured aneurysm of the sinus of Valsalva with Wildervanck syndrome (cervico-oculo-acoustic syndrome), blepharoptosis and short stature: case report. Circ J. 2007 Sep;71(9):1485-7. Tubbs RS, Oakes WJ, Blount JP. [rarediseases.org]

• Hearing Impairment

  Hypoplasia of the abducens nerves and hearing impairment occurred in severely affected individuals. [ncbi.nlm.nih.gov]

  Wildervanck syndrome is also typically associated with hearing impairment at birth (congenital). [rarediseases.org]

  Not usually, however, some patients with Duane syndrome have other problems, such as hearing impairment, Goldenhar syndrome, spinal and vertebral abnormalities. [aapos.org]

  […] convergence 0000619 Impaired ocular abduction 0000634 Impaired ocular adduction 0000542 Palpebral fissure narrowing on adduction 0000661 Showing of 60 | Last updated: 5/1/2019 Management of Duane syndrome is mainly supportive. [rarediseases.info.nih.gov]

• Short Neck

  Showing of 60 | 80%-99% of people have these symptoms Oculomotor nerve palsy 0012246 Short palpebral fissure Short opening between the eyelids 0012745 Strabismus Cross-eyed Squint Squint eyes [ more ] 0000486 30%-79% of people have these symptoms Abnormal [rarediseases.info.nih.gov]

  Individuals with Wildervanck syndrome who are affected by KFS also tend to have an unusually short neck. In severe cases, the head may appear to be situated directly on the trunk. [rarediseases.org]

  DRS has also been associated with the Klippel Feil anomaly, and this is an excellent photograph that demonstrates short neck, usually you fusion of at least two vertebrae of the neck, Marcus Gunn jaw winking, among others. [cybersight.org]

• Small Face

  […] with small face turn and severe enophthalmos. [cybersight.org]

• Nystagmus

  Secondly, Ciancia syndrome is characterized by large angle esotropia, jerk nystagmus in abduction, latent nystagmus, torticollis and asymmetric optokinetic nystagmus [14]. [bmcophthalmol.biomedcentral.com]

  To report the association of Duane syndrome with nystagmus and a patterned hyperpigmentation of the retinal pigment epithelium, developmental delay, micro- and pachygyria and craniopharyngioma. [ncbi.nlm.nih.gov]

  All 60 oral presentations of the following sessions are described: screening, amblyopia, eye movements, vision, refraction, nystagmus, stereopsis, exotropia, vergence problems, refractive surgery, cataract and strabismus, strabismus surgery, botulinum [books.google.de]

  A few other anomalies have been reported in conjunction with DRS, including other ocular anomalies such as anisometropia, amblyopia (both in approximately 15% of DRS patients), anisocoria, ptosis, and nystagmus. [accessanesthesiology.mhmedical.com]

• Headache

  […] include: holding the head in an abnormal posture, turned to the right or left crossing or misalignment of the eyes closing one eye to see better Older children may be able to describe the problems they are experiencing, such as: double vision neck pain headaches [childrenshospital.org]

  There was no history of antecedent trauma, headache, or vomiting. Her pregnancy and delivery were uneventful; and her development was normal. There was no major illness in early childhood. She had no other systemic complaints. [panafrican-med-journal.com]

  Some people with DS also have double vision and headaches. You might also have neck pain as a result of tilting our head. Cause DS can be passed down in families, but this is rare. [webmd.com]

  Case report 1 A 7-year-old girl was referred because of increased headaches since 1 year due to abnormal head posture in relation to DRS. There was no family history of strabismus or congenital anomaly. [emvmsa1a.jouve-hdi.com]

• Hyperactivity

  Savasan et al observed that more than half of the patients with Evans syndrome had evidence of lymphoid hyperactivity. [9] Teachey et al demonstrated that more than half (58%) of patients with Evans syndrome might have autoimmune lymphoproliferative syndrome [emedicine.medscape.com]

  Hyperactive CHN1 mutations have been described in people with Duane syndrome with distinctive ocular motility patterns, that caused disaggregation with novel hyperactivating CHN1 mutations. [cybersight.org]

Duane's Syndrome (type I) It may be confused with a paralysis of the muscle which causes the eye to turn outwards (lateral rectus) resulting in an unnecessary neurological workup. [strabismus.org]

See Workup for more detail. Management Medical therapy is the mainstay of management. [emedicine.medscape.com]

Approach to the patient: an overview The above review emphasizes the need to extend patient evaluation in cases of DRS beyond the routine clinical examination by a strabismologist to genetic analysis, systemic workup and neuroimaging. [dovepress.com]

Unilateral rectus muscle recession in the treatment of Duane syndrome. J AAPOS 2012; 16:145–149. 12. Sukhija J, Kaur S, Singh U. [jeos.eg.net]

References Rao VB, Helveston EM, Sahare P (2003) Treatment of upshoot and downshoot in Duane syndrome by recession and Y-splitting of the lateral rectus muscle. J AAPOS 7: 389-395. Pressman SH, Scott WE (1986) Surgical treatment of Duane's syndrome. [omicsonline.org]

Management and treatment Management of DRS is mainly supportive and involves wearing spectacles or contact lenses to correct refractive errors; occlusion or penalization of the better-seeing eye for treatment of amblyopia or prisms to correct for abnormal [orpha.net]

The treatment options and realistic expectations should be discussed with the patient and the family in detail. [webeye.ophth.uiowa.edu]

Prognosis Isolated DRS is a benign disorder which, if managed appropriately, results in excellent long term prognosis for vision. In some cases, amblyopia or loss of binocular single vision might develop. [orpha.net]

With careful follow-up, the long-term prognosis for good vision is usually excellent. [aapos.org]

These can be differentiated from true DRS based on the history of systemic illness, diplopia, trauma, or surgery.[2] Prognosis Isolated DRS leads to an excellent long-term prognosis for vision if managed appropriately. [ncbi.nlm.nih.gov]

The prognosis of surgery is not good; therefore, it is best to avoid surgery if patients have some form of binocular vision. [emedicine.medscape.com]

Ophthalmology and Neurology, Director of Neuro-Ophthalmology, Moran Eye Center, University of Utah School of Medicine Date: 03/03/2015 Secondary CORE Categories: Neuro-ophthalmology / Evaluation of Diplopia / Differentiating Paretic From Restrictive Etiologies [morancore.utah.edu]

Numerous theories concerning the etiology and pathogenesis of Duane retraction syndrome and its relationship to the associated findings have been proposed, but the exact mechanism is still under investigation. [ncbi.nlm.nih.gov]

Etiology DRS results from failure of normal development of the pontine abducens nucleus or nerve resulting in failure of the normal innervation of the lateral rectus muscle on the affected side. [orpha.net]

PURPOSE: To determine the differences in various epidemiologic and clinical characteristics among types I, II, and III of unilateral Duane syndrome and between unilateral and bilateral Duane syndrome. [ncbi.nlm.nih.gov]

"[Epidemiological and clinical aspects of Stilling-Turk-Duane syndrome]". J. Fr. Ophtalmol. (in French). 19 (8–9): 533–42. PMID 8944136. Further reading[edit] Andrews, Caroline V.; Hunter, David G.; Engle, Elizabeth C. (1993). "Duane Syndrome". [en.wikipedia.org]

Summary Epidemiology The prevalence of DRS is about 1/10, 000, representing about 1-5% of all strabismus cases. Females are more affected than males. [orpha.net]

Friedman, CR, Neimann, J., Wegener, HC, and Tauxe, RV Epidemiology of Campylobacter jejuni infections in the United States and other industrialized nations. [books.google.es]

Considerations on pathophysiology and aetiology. Transactions of the Fifth International Orthoptic Congress, Cannes., pp.119-125. Journal Kim, J. H. and Hwang, J. [citethisforme.com]

As DRS type 3 partly share the same pathophysiology with type 1 and 2 DRS, the classification of DRS may have to be revised according to MRI findings. [journals.plos.org]

Pathophysiology The exact pathophysiology of Evans syndrome is unknown, although the mechanism generally seems to be related to the one associated with autoimmune hemolytic anemia and immune thrombocytopenia. [emedicine.medscape.com]

Click here to view Discussion Many factors stand behind the difficulty in the management of Duane retraction syndrome such as its mysterious pathophysiology together with its wide range of subtypes and diverse spectrum of the presenting signs. [jeos.eg.net]

Pathophysiology Imaging evidence of dysinnervation High-resolution MRI enables direct demonstration of the size and contractility of extraocular muscles, as well as their peripheral and subarachnoid motor innervation ( Figure 57.5A ). [entokey.com]

Engle Medicine Archives of ophthalmology 2007 An understanding of the genetic underpinnings of strabismus may help identify patients at risk early enough to prevent disability and may lead to new preventive or therapeutic approaches. [semanticscholar.org]

Surveillance is necessary to prevent or detect amblyopia. Vision therapy is advised for secondary convergence insufficiency. [orpha.net]

Prevention of secondary complications: Specialist examination early in life to detect refractive errors to prevent amblyopia and avoid compounding the motility problem; amblyopia therapy to prevent vision loss in the less preferred eye; surgery to prevent [ncbi.nlm.nih.gov]

Bilateral MR recession may also prevent contracture of the MR in the affected eye by creating fixation duress. [tnoajosr.com]