综合征性颅缝早闭
Syndromic Craniosynostosis.
作者信息
Sawh-Martinez Rajendra, Steinbacher Derek M
机构信息
Section of Plastic and Reconstructive Surgery, Department of Surgery, Yale University, 330 Cedar Street, Boardman Building, 3rd Floor, New Haven, CT 06511, USA.
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.
出版信息
Clin Plast Surg. 2019 Apr;46(2):141-155. doi: 10.1016/j.cps.2018.11.009.
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.
综合征性颅缝早闭患者的管理策略需要多学科亚专业团队，以便为复杂的重建方法提供最佳护理。最常见的颅缝早闭综合征包括Apert综合征（FGFR2）、Crouzon综合征（FGFR2）、Muenke综合征（FGFR3）、Pfeiffer综合征（FGFR1和FGFR2）以及Saethre-Chotzen综合征（TWIST）。双冠状缝颅缝早闭（短头畸形）最常与综合征性颅缝早闭相关。疾病表现从轻度缝线受累到严重全颅缝早闭不等，伴有一系列颅面外畸形表现。在认识到各种不同表现的同时，了解多方面的综合征表现对于提供必要的个性化护理至关重要。颅骨重塑旨在缓解颅骨发育受限和颅内压升高的情况，并恢复正常形态。
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