Craniofacial Syndromes
Craniofacial Syndromes
复制标题
DOI：
10.1097/prs.0000000000000308
复制
发表时间：
2014-07-01
影响因子：
3.6
通讯作者：
Hollier, Larry H., Jr.
中科院分区：
文献类型：
--
作者：
Buchanan, Edward P.;Xue, Amy S.;Hollier, Larry H., Jr.
学习目标：学习完这篇文章后，参加者应该能够：1.认识常见颅面综合征的临床表现。2.了解与每种综合症相关的最严重并发症。3.总结：颅面综合征分为两大类：与颅缝早闭相关的和与唇腭裂相关的。每一种都有不同的潜在并发症，需要独特的手术治疗方法。颅缝早闭是一种先天性疾病，其中一个或多个颅缝过早融合。与这种情况相关的最常见的综合征包括Crouzon，Apert，Pfeiffer，Muenke和Saethre-Chotzen综合征。这些儿童的外科治疗需要多学科的方法和家庭的密切参与。手术必须考虑到骨结构不断增长的潜力。与裂相关的常见综合征包括Pierre Robin、Treacher柯林斯、Nager、Binder和Stickler综合征。这些儿童中有许多有严重的气道问题，需要在手术重建前立即解决。与颅缝早闭相关的综合征一样，治疗的关键是多学科方法，重点是正确的时机。本文的目的是审查这些患者的临床表现，护理和治疗。
Learning Objectives: After studying this article, the participant should be able to: 1. Recognize the clinical presentations of commonly seen craniofacial syndromes. 2. Understand the most serious complications associated with each syndrome. 3. Formulate the best age-appropriate surgical plans.Summary: Craniofacial syndromes fall into two major categories-those associated with craniosynostosis, and those associated with clefts. Each has a different set of potential complications requiring a unique approach for surgical management. Craniosynostosis is a congenital disorder in which one or more of the cranial sutures fuses prematurely. The most common syndromes associated with this condition include Crouzon, Apert, Pfeiffer, Muenke, and Saethre-Chotzen syndromes. Surgical management of these children requires a multidisciplinary approach and close involvement of the family. Operations must take into consideration the growing potential of the bony structures. Common syndromes associated with clefts include Pierre Robin, Treacher Collins, Nager, Binder, and Stickler syndromes. Many of these children have severe airway issues requiring immediate address before operative reconstruction. As with syndromes associated with craniosynostosis, the key to management is a multidisciplinary approach focused on the right timing. The purpose of this article is to review the clinical presentation, care, and treatment of these patients.