Apert Syndrome: A Consensus on the Management of Apert Hands

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 sign...

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Bibliographic Details
Published in:Journal of cranio-maxillo-facial surgery 2017-02, Vol.45 (2), p.223-231
Main Authors: Pettitt, David A., MBChB, Arshad, Zeeshaan, Mishra, Anuj, PhD, McArthur, Paul, PhD
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - surgery
Acrocephalosyndactylia - surgery
Apert hands
Apert syndrome
Congenital hand deformity
Craniofacial surgery
Dentistry
Hand
Hand Deformities, Congenital - surgery
Humans - surgery
Practice Patterns, Physicians
Surgery
Surveys and Questionnaires
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Summary: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 exists. Conclusion The results of this study provide an interesting snapshot of the current management of Apert hands across the UK’s specialist 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.
ISSN:1010-5182
1878-4119
DOI:10.1016/j.jcms.2016.11.018