McClure Philip, Booy David, Katarincic Julia, Eberson Craig
Warren Alpert Medical School, Brown University, Providence, RI 02905, USA.
Pediatric Hand Surgery, Rhode Island Hospital, Providence, RI 02905, USA.
Int J Pediatr. 2016;2016:9736723. doi: 10.1155/2016/9736723. Epub 2016 Feb 8.
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.
背景。莫比乌斯综合征是一种罕见疾病，由双侧先天性第七神经麻痹所定义。我们专注于报告该人群中骨科疾病的患病率。方法。23名患有莫比乌斯综合征的个体接受了骨科体格检查，另外96名患者填写了一份关于自我报告的骨科诊断的调查问卷。结果。在体格检查系列中，60%的个体存在马蹄内翻足，在调查问卷中这一比例为42%。脊柱侧弯的比例分别为26%和28%。波兰综合征的比例分别为17%和30%。除了这些发现外，27%的患者报告存在麻醉困难，包括插管困难和气道问题。结论。在莫比乌斯综合征患者中，脊柱侧弯、马蹄内翻足、肢体横断性缺损和波兰综合征的患病率有所增加。在存在多种通常需要手术矫正的畸形情况下，注意到麻醉困难的发生率较高，在手术规划期间应与患者及其他医疗人员进行讨论。
