背景:Apert综合征是一种以原发性颅骨融合为特征的先天性疾病,中面部畸形和手和脚的复杂对称畸形。手展示了先天性上肢畸形最复杂的集合之一,对儿科手外科医生构成了重大挑战。这项研究考察了英国四个专业颅面单位关于Apert手管理的现有文献和当前实践,以便为指南制定提供基础。
方法:综述了现有文献。调查类型的问卷分发给了英国的四个颅面专家,并分析了答案。
结果:Apert手的管理很大程度上取决于畸形的存在程度。尽管所有单位的目标是实现五位数的手,手术时机的变化,存在操作协议和动员政策。
结论:这项研究的结果为英国四个颅面外科单位的Apert手的当前管理提供了一个有趣的快照。尽管存在一些较小的单位间差异,但英国的四个单位在Apert手管理周围的大多数地区都保持一致。多学科的管理方法对于优化功能和美学上可接受的手的恢复仍然至关重要。
BACKGROUND: Apert Syndrome is a congenital condition characterised by primary craniosynostosis, midfacial malformations and complex symmetrical malformations of the hands and feet. The hands demonstrate one of the most complex collections of congenital upper limb deformities, posing a significant challenge for the paediatric hand surgeon. This study examines the extant literature and current practice of the four UK specialist craniofacial units regarding the management of Apert hands in order to provide a basis for
guideline development.
METHODS: The current literature was reviewed. Survey-type questionnaires were distributed to the four UK specialist craniofacial units and responses analysed.
RESULTS: Management of the Apert hand is largely dictated by the degree of malformation present. Although all units aim to achieve a five digit hand, variation in the timing of surgery, operative protocols and mobilisation policies exist.
CONCLUSIONS: The results of this study provide an interesting snapshot of the current management of Apert hands across four UK craniofacial surgery units. The four UK units remain congruent on most areas surrounding the management of Apert hands although some minor inter-unit variation exists. A multidisciplinary approach to management remains fundamental in optimising the regain of function and aesthetically acceptable hands.