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Clin Plast Surg. 2019 Apr;46(2):141-155. doi: 10.1016/j.cps.2018.11.009.

Syndromic Craniosynostosis.

Author information

1
Section of Plastic and Reconstructive Surgery, Department of Surgery, Yale University, 330 Cedar Street, Boardman Building, 3rd Floor, New Haven, CT 06511, USA.
2
Section of Plastic and Reconstructive Surgery, Oral and Maxillofacial Surgery, Department of Surgery, Yale-New Haven Hospital, Yale University, 330 Cedar Street, Boardman Building, 3rd Floor, New Haven, CT 06511, USA. Electronic address: Derek.Steinbacher@yale.edu.

Abstract

Management strategies for syndromic craniosynostosis patients require multidisciplinary subspecialty teams to provide optimal care for complex reconstructive approaches. The most common craniosynostosis syndromes include Apert (FGFR2), Crouzon (FGFR2), Muenke (FGFR3), Pfeiffer (FGFR1 and FGFR2), and Saethre-Chotzen (TWIST). Bicoronal craniosynostosis (turribrachycephaly) is most commonly associated with syndromic craniosynostosis. Disease presentation varies from mild sutural involvement to severe pansynostoses, with a spectrum of extracraniofacial dysmorphic manifestations. Understanding the multifaceted syndromic presentations while appreciating the panoply of variable presentations is central to delivering necessary individualized care. Cranial vault remodeling aims to relieve restriction of cranial development and elevated intracranial pressure and restore normal morphology.

KEYWORDS:

Apert; Craniofacial syndromes; Craniosynostosis; Crouzon; FGFR; Muenke; Pfeiffer; Saethre-Chotzen

PMID:
30851747
DOI:
10.1016/j.cps.2018.11.009
[Indexed for MEDLINE]

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