EDITORIAL UANE SYNDROME IS A WELL-DEFINED CON- kiss et al. These studies indicate that, in Duane syn- genital ocular motor abnormality that, drome, there is absence or hypoplasia of the sixth cra- in its most usual form, is characterized nial nerve and innervation of the lateral rectus muscle by (1) a large duction deficit (usually ab- by a branch of the third cranial nerve. Magnetic reso- D duction) accompanied by (2) a dispro- nance imaging studies have subsequently confirmed the portionately small horizontal deviation in primary gaze absence or hypoplasia of the sixth cranial nerve in pa- 8,9 that is compensated for by (3) a slight face turn that acts tients with Duane syndrome. to ensure that (4) normal binocularity is maintained; (5) Yet to be answered is how this specific and stereo- amblyopia is uncommon and usually associated with an- typic miswiring comes about in Duane syndrome. De- 1,2 isometropia. Other variable features include a mild to spite the fact that the overwhelming majority of cases of moderate limitation of adduction, palpebral narrowing Duane syndrome are not familial, recent genetic studies on adduction, and vertical upshoots or downshoots in of some of the infrequent familial and syndromic cases
JAMA Ophthalmology – American Medical Association
Published: Apr 1, 2013
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