PURPOSE: To determine the prevalence and clinical characteristics of patients with Duane retraction syndrome presenting with exotropia in a tertiary eye care center. [ncbi.nlm.nih.gov]

• Broad Thumb

  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]

• Arachnodactyly

  Systemic examination was significant for tall and thin posture with a reduced upper-to-lower segment ratio, increased arm-to-height ratio, arachnodactyly, positive wrist and thumb sign, hypermobility of joints (she could touch back of her thumb and the [hindawi.com]

• High Arched Palate

  The proband had unilateral Duane retraction syndrome (DRS) with low-set ears bilaterally, a high arched palate, and clinodactyly. Motor development and cognitive function were normal. [ncbi.nlm.nih.gov]

• Painful Ophthalmoplegia

  Paresis of isolated and multiple cranial nerves and painful ophthalmoplegia. Yanoff M, Duker JS. Ophthalmology. Philadelphia: Mosby; 1999. 11.16.1-11.16.12. Nelson LB. [emedicine.com]

  Paresis of Isolated and Multiple Cranial Nerves and Painful Ophthalmoplegia. In: Yanoff M, Duker JS, editors. Ophthalmology. Philadelphia, PA: Mosby; 1999. 50. Kekunnaya R, Gupta A, Sachdeva V, et al. Duane retraction syndrome: series of 441 cases. [dovepress.com]

• Low Set Ears

  The proband had unilateral Duane retraction syndrome (DRS) with low-set ears bilaterally, a high arched palate, and clinodactyly. Motor development and cognitive function were normal. [ncbi.nlm.nih.gov]

• Impulsivity

  For example, there may be abnormalities of the coiled bony passage (cochlea) that transforms sound vibrations into nerve impulses for transmission to the brain. [rarediseases.org]

  On EMG, LR showed peak impulses on abduction and a second paradoxical impulse on attempted adduction while the electrical activity of MR was normal. [tnoajosr.com]

  This abnormal or paradoxical innervational impulse to the horizontal recti is the basic pathology of this disease. The LR may exhibit a spectrum of abnormal innervational pattern ranging from paradoxical to the subnormal innervation. [ijo.in]

  Thus, the problem is not primarily with the eye muscle itself but with the nerve that transmits the electrical impulses to the muscle. [emedicine.com]

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]

Treatment Treatment Options: Various treatments can be useful, ranging from prisms for mild cases to muscle surgery for a severe head turn or vertical deviations. [disorders.eyes.arizona.edu]

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]

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]

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]

Zanin et al. have classified into three types based on the functional prognosis.[20] Figure 3: (a) Patient with bilateral Duane retraction syndrome showing right exotropia; (b) limitation of ductions in both eyes in right gaze; (c) limitation of ductions [tnoajosr.com]

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]

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]

At one point the syndrome was considered to be a myopathic disorder based on histologic changes in the lateral rectus but current thought based on MRI and neurohistologic studies favors a neuropathic etiology. [disorders.eyes.arizona.edu]

The underlying etiology of DRS is a defect in some of the eye muscles (specifically the lateral and medial rectus muscles) receiving proper signal from the cranial nerves that innervate them. [cags.org.ae]

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]

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]

"[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]

Differences in epidemiological and clinical characteristics between various types of Duane retraction syndrome in 331 patients. J AAPOS 2008; 12:576-80. Kekunnaya R, Gupta A, Sachdeva V, Krishnaiah S, Rao BV, Vashist U, Ray D. [djo.org.in]

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]

Huber A (1984) Duane’s Retraction Syndrome: Consideration of Pathophysiology and Etiology. In: Grune & Stratton Orlando (Eds.), Florida, USA, pp. 345-361. [medcraveonline.com]

[…] irregularly dominant manner with incomplete penetrance and variable expressivity [2] Duane Retraction Syndrome results from an absent or dysplastic abducens motor neurons with aberrant innervations of the lateral rectus muscle by the oculomotor nerve. [7] Pathophysiology [eyewiki.aao.org]

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]

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

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

To eliminate disfiguring abnormal eyelid position The goal of treatment is to restore satisfactory eye alignment in the straight-ahead position, eliminate an abnormal head posture and to prevent amblyopia. Eye muscle surgery is not always required. [aapos.org]