Albert Gregory W, Menezes Arnold H, Hansen Daniel R, Greenlee Jeremy D W, Weinstein Stuart L
Department of Neurosurgery, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, Iowa 52242, USA.
J Neurosurg Pediatr. 2010 Jun;5(6):554-61. doi: 10.3171/2010.3.PEDS09489.
The authors conducted a study to evaluate the unique presenting signs and symptoms of Chiari malformation Type I (CM-I) in children younger than 6 years of age and highlight the benefits of early surgical treatment in this patient population.
The authors reviewed the medical records of patients who presented to the neurosurgery department before their 6th birthday and subsequently underwent surgery for CM-I. They identified 39 patients who had been evaluated between 1984 and 2007 and examined the medical records for presentation, surgical intervention, and outcome.
Children aged 0-2 years commonly presented with oropharyngeal dysfunction (77.8%). Children aged 3-5 years more frequently presented with syringomyelia (85.7%), scoliosis (38.1%), and/or headache (57.1%). All patients underwent posterior fossa craniectomy. Additionally, in many patients cervical laminectomy and/or duraplasty was performed. A few patients required transoral decompression and occipitocervical fusion. In most cases, surgery led to resolution or dramatic improvement of initial symptoms.
Early recognition and surgical treatment of CM-I in young children leads to good outcomes in the majority of patients. Additional therapies for oropharyngeal dysfunction, syringomyelia, and scoliosis can frequently be avoided.
作者开展了一项研究，以评估6岁以下儿童I型Chiari畸形（CM-I）独特的临床表现，并强调早期手术治疗对该患者群体的益处。
作者回顾了6岁生日前到神经外科就诊并随后接受CM-I手术的患者的病历。他们确定了1984年至2007年间接受评估的39例患者，并检查了病历中的临床表现、手术干预及结果。
0至2岁的儿童常见口咽功能障碍（77.8%）。3至5岁的儿童更常出现脊髓空洞症（85.7%）、脊柱侧弯（38.1%）和/或头痛（57.1%）。所有患者均接受了后颅窝颅骨切除术。此外，许多患者还进行了颈椎椎板切除术和/或硬脑膜成形术。少数患者需要经口减压和枕颈融合术。在大多数情况下，手术使初始症状得到缓解或显著改善。
幼儿CM-I的早期识别和手术治疗可使大多数患者获得良好预后。口咽功能障碍、脊髓空洞症和脊柱侧弯的额外治疗通常可以避免。
