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阿佩尔综合征:关于阿佩尔手部畸形治疗的共识。

Apert syndrome: A consensus on the management of Apert hands.

作者信息

Pettitt David A, Arshad Zeeshaan, Mishra Anuj, McArthur Paul

机构信息

Mersey Regional Plastic Surgery Unit, Whiston Hospital, Warrington Rd, Prescot, Merseyside, L35 5DR, UK.

Mersey Regional Plastic Surgery Unit, Whiston Hospital, Warrington Rd, Prescot, Merseyside, L35 5DR, UK.

出版信息

J Craniomaxillofac Surg. 2017 Feb;45(2):223-231. doi: 10.1016/j.jcms.2016.11.018. Epub 2016 Dec 10.

DOI:10.1016/j.jcms.2016.11.018
PMID:28087285
Abstract

INTRODUCTION

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.

CONCLUSION

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.

摘要

引言

Apert综合征是一种先天性疾病,其特征为原发性颅缝早闭、面中部畸形以及复杂的手足对称性畸形。手部表现出先天性上肢畸形中最复杂的类型之一,这给小儿手外科医生带来了巨大挑战。本研究调查了英国四个专科颅面外科单位关于Apert手部畸形治疗的现有文献和当前实践,以便为制定指南提供依据。

方法

回顾当前文献。向英国四个专科颅面外科单位发放调查问卷并分析回复。

结果

Apert手部畸形的治疗很大程度上取决于畸形的程度。尽管所有单位都旨在实现五指手,但手术时机、手术方案和康复策略存在差异。

结论

本研究结果提供了英国四个颅面外科单位当前对Apert手部畸形治疗情况的有趣概况。英国这四个单位在Apert手部畸形治疗的大多数方面保持一致,尽管存在一些小的单位间差异。多学科治疗方法对于优化功能恢复和获得美观的手部仍然至关重要。

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