Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, Av. Adolpho Lutz, 100, Caixa Postal 6028, Campinas, São Paulo, 13084-880, Brazil.
Division of Neurosurgery, University of Campinas (UNICAMP), São Paulo, Brazil.
Childs Nerv Syst. 2021 Jun;37(6):2001-2009. doi: 10.1007/s00381-021-05169-w. Epub 2021 Apr 18.
The most commonly occurring syndromic craniosynostoses are Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, and Saethre-Chotzen syndrome. There is insufficient data regarding postoperative syndrome-related outcomes following the posterior vault distraction osteogenesis (PVDO) procedure, as well as data addressing whether or not additional procedures will be subsequently necessary to comprehensively treat children who undergo PVDO. Thus, the objective of this study is to describe and compare syndrome-related potential complications and outcomes associated with the PVDO procedure.
An observational retrospective study was performed on consecutive patients (n=24) with Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, or Saethre-Chotzen syndrome, respectively, who underwent PVDO between 2012 and 2019. Demographic data (patient gender and age when the PVDO procedure was performed), diagnosis, surgery-related data, and outcome data (perioperative and midterm complications and need for additional surgery) were verified.
Total relative blood transfusion volumes per kilogram for the patients were as follows: 22.75 ± 9.30 ml for Apert syndrome, 10.73 ± 2.28 ml for Crouzon syndrome (Apert versus Crouzon, p<0.05), 18.53 ± 8.08 ml for Pfeiffer syndrome, and 19.74 ± 9.12 ml for Saethre-Chotzen syndrome. None of the patients required a secondary procedure to alleviate intracranial pressure except for a Saethre-Chotzen patient.
PVDO is an effective technique to address elevated intracranial pressure in SC patients that alleviates the need for secondary procedures at midterm follow-up. Apert syndrome patients presented relatively higher total blood transfusion rates than Crouzon syndrome patients who were operated on at a later age and weighed more.
最常见的综合征性颅缝早闭包括 Apert 综合征、Crouzon 综合征、 Pfeiffer 综合征和 Saethre-Chotzen 综合征。关于后路颅顶扩张性骨切开术（PVDO）后与综合征相关的术后结果，以及是否需要进一步手术来全面治疗接受 PVDO 的儿童，数据有限。因此，本研究的目的是描述和比较与 PVDO 手术相关的综合征相关潜在并发症和结果。
对 2012 年至 2019 年间分别接受 PVDO 的 Apert 综合征、Crouzon 综合征、 Pfeiffer 综合征或 Saethre-Chotzen 综合征患者（n=24）进行了一项观察性回顾性研究。验证了人口统计学数据（患者性别和接受 PVDO 手术时的年龄）、诊断、手术相关数据和结果数据（围手术期和中期并发症以及是否需要额外手术）。
每位患者的每公斤总相对输血量如下：Apert 综合征为 22.75±9.30ml，Crouzon 综合征为 10.73±2.28ml（Apert 与 Crouzon，p<0.05）， Pfeiffer 综合征为 18.53±8.08ml，Saethre-Chotzen 综合征为 19.74±9.12ml。除了一名 Saethre-Chotzen 患者需要缓解颅内压的二次手术外，没有其他患者需要进行二次手术。
PVDO 是一种治疗 SC 患者颅内压升高的有效技术，可在中期随访时减轻二次手术的需要。Apert 综合征患者的总输血率明显高于 Crouzon 综合征患者，后者接受手术的年龄较大，体重也较重。
