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Orthopedic Manifestations of Mobius Syndrome: Case Series and Survey Study

机译:Mobius综合征的骨科表现:病例系列和调查研究

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摘要

Background. Mobius Syndrome is a rare disease defined by bilateral congenital 7th nerve palsy. We focus on reporting the prevalence of orthopedic disease in this population. Methods. Twenty-three individuals with Mobius Syndrome underwent orthopedic physical examination, and additional 96 patients filled out a survey for self-reported orthopedic diagnoses. Results. Clubfoot was present in 60% of individuals in the physical exam series and 42% of those in the survey. Scoliosis was present in 26% and 28%, respectively. Poland's Syndrome was present in 17% and 30%. In addition to these findings, 27% of patients reported having difficulty with anesthesia, including difficulty in intubation and airway problems. Conclusion. An increased prevalence of scoliosis, clubfoot, transverse limb deficiencies, and Poland's Syndrome is identified in the setting of Mobius Syndrome. In the setting of several deformities often requiring surgical correction, a high incidence of anesthetic difficulty is noted and should be discussed with patients and other providers during surgical planning.
机译:背景。莫比乌斯综合症是一种罕见的疾病,由双侧先天性第七神经麻痹定义。我们专注于报告此人群中骨科疾病的患病率。方法。 23例莫比乌斯综合症患者接受了骨科体格检查,另外96名患者填写了一份自我报告的骨科诊断调查表。结果。在身体检查系列中有60%的人有马蹄内翻足,在调查中有42%的人有。脊柱侧弯分别占26%和28%。波兰综合症的发病率分别为17%和30%。除了这些发现之外,还有27%的患者报告麻醉困难,包括插管困难和气道问题。结论。在莫比乌斯综合症的背景下,发现脊柱侧凸,马蹄足,四肢横行缺陷和波兰综合症的患病率增加。在通常需要手术矫正的几种畸形的情况下,注意到麻醉困难的发生率很高,应在手术计划期间与患者和其他提供者进行讨论。

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