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Chiari malformation associated with craniosynostosis

机译：Chiari畸形与颅骨融合症相关

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Object Chiari malformation (CM) Type I is frequently associated with craniosynostosis. Optimal management of CM in patients with craniosynostosis is not well-established. The goal of this study was to report on a series of pediatric patients with both craniosynostosis and CM and discuss their management. Methods The authors searched the medical records of 383 consecutive patients treated for craniosynostosis at a single institution over a 15-year period to identify those with CM. They recorded demographic data as well as surgical treatment and outcomes for these patients. When MR imaging was performed, cerebellar tonsillar descent was recorded and any other associated findings, such as hydrocephalus or spinal syringes, were noted. Results A total of 29 patients with both CM and craniosynostosis were identified. Of these cases, 28% had associated occipital venous abnormalities, 45% were syndromic, and 52% also had hydrocephalus. Chiari malformation was more likely to be present in those patients with isolated lambdoid synostosis (55%), multisuture synostosis (35%), and pansynostosis (80%), compared with patients with coronal synostosis (6%) or sagittal synostosis (3%). All patients underwent surgical repair of craniosynostosis: 16 had craniosynostosis repair as well as CM decompression, and 13 patients did not undergo CM decompression. Of the 7 patients in whom craniosynostosis repair alone was performed, 5 had decreased tonsillar ectopia postoperatively and 5 had improved CSF flow studies postoperatively. Both patients with a spinal syrinx had imaging-documented syrinx regression after craniosynostosis repair. In 12 patients in whom CM was diagnosed after primary craniosynostosis repair, 5 had multiple cranial vault expansions and evidence of elevated intracranial pressure. In 5 cases, de novo CM development was documented following craniosynostosis repair at a mean of 3.5 years after surgery. Conclusions Chiari malformation is frequently seen in patients with both multi- and single-suture lambdoid craniosynostosis. Chiari malformation, and even a spinal cord syrinx, will occasionally resolve following craniofacial repair. De novo development of CM after craniosynostosis repair is not unusual.
机译：I型对象Chiari畸形（CM）经常与颅骨融合症相关。颅骨融合症患者对CM的最佳管理尚不完善。这项研究的目的是报告一系列同时患有颅骨融合症和CM的儿科患者，并讨论其治疗方法。方法作者在15年的时间里，在单一机构中检索了383例连续性颅骨融合症患者的病历，以鉴定患有CM的患者。他们记录了这些患者的人口统计学数据以及手术治疗和预后。进行MR成像时，记录了小脑扁桃体的下降情况，并记录了其他相关发现，例如脑积水或脊髓注射器。结果共鉴定出29例同时患有CM和颅骨前突的患者。在这些病例中，有28％伴有枕叶静脉异常，其中45％为综合征性，而52％也患有脑积水。与冠状动脉突触（6％）或矢状突触（3％）相比，孤立性lambdoid突触（55％），多缝合突突（35％）和胰腺突触（80％）的患者更容易出现Chiari畸形。 ）。所有患者均接受了颅骨融合术的手术修复：16例颅骨融合术修复以及CM减压，13例患者未进行CM减压。在仅进行颅突融合术修复的7例患者中，有5例术后扁桃体外翻减少，而5例术后CSF血流改善。两名患有脊髓性syrinx的患者在颅骨前突修复后均具有影像学证实的syrinx消退。在12例经颅脑前突修复后被诊断为CM的患者中，有5例具有多个颅穹顶扩张并显示颅内压升高。在5例病例中，有记录表明颅骨突触修复术后平均3.5年进行了从头CM的发展。结论多缝合和单缝合lambdoid颅突神经病患者均经常出现Chiari畸形。颅面部修复后，Chiari畸形，甚至是脊髓syrinx偶尔会消失。颅突修补术后从头开始发展CM并不罕见。

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