Abstract：
OBJECTIVE: Posterior vault distraction osteogenesis (PVDO) has been utilized during the past 15 years to treat a variety of clinical features commonly presented by patients with Apert syndrome. The objective of this study is to determine the efficacy of PVDO in addressing both elevated intracranial pressure (ICP) and ectopia of the cerebellar tonsils (ECT) in young Apert patients. In addition, we aimed to determine the prevalence of hydrocephalus in Apert syndrome patients who underwent PVDO.
METHODS: A retrospective study was made with a cohort of 40 consecutive patients with syndromic craniosynostosis (SC), previously diagnosed with Apert syndrome, who underwent PVDO between 2012 and 2022, and thereafter received at least 1 year of follow-up care. Demographic data and diagnosis, along with surgical and outcome data, were verified using medical records, clinical photographs, radiologic examination, and interviews with the parents of all cohort patients.
RESULTS: The average patient age when PVDO was performed was 12.91 ± 10 months. The average posterior advancement distance achieved per patient was 22.68 ± 5.26 mm. The average hospital stay per patient was 3.56 ± 2.44 days. The average absolute and relative blood transfusion volumes were 98.47 ml and 17.63 ml/kg, respectively. Although five patients (14%) presented ECT preoperatively, this condition was completely resolved by PVDO in three of these five patients. One of the three patients whose ECT had completely resolved presented syringomyelia postoperatively, requiring subsequent extra dural foramen magnum decompression. All of the remaining four patients were asymptomatic for ECT for at least 1 year of follow-up, and none of these four patients required any additional treatments to address ECT. Two patients presented hydrocephalus requiring ventriculoperitoneal shunt placement.
CONCLUSIONS: This study demonstrates that PVDO both reduces diagnosed elevated ICP symptoms and is partially effective in treating ECT in Apert syndrome patients. Hydrocephalus in Apert syndrome is an uncommon feature. The effectiveness of PVDO in addressing hydrocephalus is uncertain.
METHODS: A retrospective study was made with a cohort of 40 consecutive patients with syndromic craniosynostosis (SC), previously diagnosed with Apert syndrome, who underwent PVDO between 2012 and 2022, and thereafter received at least 1 year of follow-up care. Demographic data and diagnosis, along with surgical and outcome data, were verified using medical records, clinical photographs, radiologic examination, and interviews with the parents of all cohort patients.
RESULTS: The average patient age when PVDO was performed was 12.91 ± 10 months. The average posterior advancement distance achieved per patient was 22.68 ± 5.26 mm. The average hospital stay per patient was 3.56 ± 2.44 days. The average absolute and relative blood transfusion volumes were 98.47 ml and 17.63 ml/kg, respectively. Although five patients (14%) presented ECT preoperatively, this condition was completely resolved by PVDO in three of these five patients. One of the three patients whose ECT had completely resolved presented syringomyelia postoperatively, requiring subsequent extra dural foramen magnum decompression. All of the remaining four patients were asymptomatic for ECT for at least 1 year of follow-up, and none of these four patients required any additional treatments to address ECT. Two patients presented hydrocephalus requiring ventriculoperitoneal shunt placement.
CONCLUSIONS: This study demonstrates that PVDO both reduces diagnosed elevated ICP symptoms and is partially effective in treating ECT in Apert syndrome patients. Hydrocephalus in Apert syndrome is an uncommon feature. The effectiveness of PVDO in addressing hydrocephalus is uncertain.
摘要：
目的：在过去的15年中，后穹窿牵张成骨（PVDO）已被用于治疗Apert综合征患者常见的各种临床特征。这项研究的目的是确定PVDO在解决年轻Apert患者的颅内压升高（ICP）和小脑扁桃体外翻（ECT）方面的功效。此外,我们旨在确定接受PVDO的Apert综合征患者脑积水的患病率.
方法：对40名连续患有颅骨融合综合征（SC）的患者进行了一项回顾性研究，之前被诊断出患有Apert综合征,患者在2012年至2022年间接受了PVDO,此后接受了至少1年的随访治疗.人口数据和诊断，以及手术和结果数据，使用医疗记录进行了验证,临床照片，放射学检查,并对所有队列患者的父母进行访谈。
结果：进行PVDO的患者平均年龄为12.91±10个月。每位患者获得的平均后前进距离为22.68±5.26mm。每位患者平均住院时间为3.56±2.44天。平均绝对和相对输血量分别为98.47ml和17.63ml/kg,分别。虽然5例患者（14%）术前出现ECT，在这5例患者中,有3例通过PVDO完全缓解了这种情况.3例ECT完全消退的患者中有1例出现脊髓空洞症，需要随后的硬脑膜孔耳外减压。其余4例患者均无症状进行ECT至少1年的随访,这四名患者中没有一名需要任何额外的治疗来解决ECT。两名患者出现脑积水，需要进行脑室腹膜分流术。
结论：这项研究表明，PVDO既能减轻诊断的ICP升高症状，又能部分有效治疗Apert综合征患者的ECT。Apert综合征中的脑积水是一个罕见的特征。PVDO治疗脑积水的有效性尚不确定。
方法：对40名连续患有颅骨融合综合征（SC）的患者进行了一项回顾性研究，之前被诊断出患有Apert综合征,患者在2012年至2022年间接受了PVDO,此后接受了至少1年的随访治疗.人口数据和诊断，以及手术和结果数据，使用医疗记录进行了验证,临床照片，放射学检查,并对所有队列患者的父母进行访谈。
结果：进行PVDO的患者平均年龄为12.91±10个月。每位患者获得的平均后前进距离为22.68±5.26mm。每位患者平均住院时间为3.56±2.44天。平均绝对和相对输血量分别为98.47ml和17.63ml/kg,分别。虽然5例患者（14%）术前出现ECT，在这5例患者中,有3例通过PVDO完全缓解了这种情况.3例ECT完全消退的患者中有1例出现脊髓空洞症，需要随后的硬脑膜孔耳外减压。其余4例患者均无症状进行ECT至少1年的随访,这四名患者中没有一名需要任何额外的治疗来解决ECT。两名患者出现脑积水，需要进行脑室腹膜分流术。
结论：这项研究表明，PVDO既能减轻诊断的ICP升高症状，又能部分有效治疗Apert综合征患者的ECT。Apert综合征中的脑积水是一个罕见的特征。PVDO治疗脑积水的有效性尚不确定。
