【作者】 张谦生；

【导师】 宋来君；

【作者基本信息】 郑州大学 ， 外科学， 2011， 硕士

【摘要】 背景和目的：颅颈交界区畸形也称枕骨大孔区畸形,主要是指枕骨及寰、枢椎发育异常,包括扁平颅底、颅底凹陷、寰枕融合、寰枢椎脱位、颈椎分节不全(Klippel-Feil综合征)、小脑扁桃体下疝畸形(Arnold-Chiari畸形)。这几种畸形可单独或几种同时出现。上述骨骼发育异常导致延髓、颈髓、小脑及后组颅神经(Ⅸ-Ⅻ)受压迫、牵拉,出现相应的神经症状和体征。下疝的小脑扁桃体继发脑脊液循环障碍,可能是脑积水及延脊髓空洞形成的重要原因。此区的软组织如筋膜发育异常、粘连增生、钙化更可进一步加重神经系统受压和脑脊液循环障碍。颅颈畸形的治疗目的是解除畸形骨骼及异常软组织对脑干、颈髓及神经纤维的压迫,恢复枕颈部骨结构的排列关系,疏通脑脊液循环,并重建枕颈部稳定性。单纯的保守治疗,如颅骨牵引、Halo-vest支架、头颈胸石膏外固定等,疗效欠佳,个别患者还可能导致病情加重。国内外现有的手术方式较多,效果各异,本研究通过对我科手术方式及疗效的回顾性分析,以期寻求治疗颅颈交界区畸形及其并发症的较好手术方式。材料与方法：选取我科自2007年1月至2011年3月共手术治疗颅颈畸形病人141例,影像学资料及临床资料完整,均行MRI、X线平片,部分行SCT检查。根据不同的临床表现和影像学检查征分为A、B、C组三组,A组单纯行后方入路枕下减压术,B组行枕下减压术、并一期行枕颈植骨融合内固定术,C组行后方入路枕下减压加经口口因入路齿状突切除术和枕颈植骨融合内固定术,术后随访患者的临床表现及影像学表现,分组计算有效率。结果：本组随访病例中影像学检查小脑扁桃体复位良好、无脊髓空洞扩大及继发脑积水者,三组患者术后早期或半年随访时原临床表现改善比率分别为88.4%、82.6%、80.3%,疗效明显,针对不同畸形类型选取的相应的手术方式是有效的。结论：1.颅颈交界区畸形的手术治疗中减压和稳定性重建应该并重；2.根据患者颅颈交界区畸形的具体类型、临床表现和影像学显示的神经组织受压情况,个性化的选择适宜的手术减压及固定方式；3.对于合并脑积水及脊髓空洞的治疗应着重解除第四脑室下端的脊髓中央管开口处(即闩部),可能存在的假膜,使脑脊液流出通畅；4.颅颈交界区畸形的稳定性重建主要靠植骨后3-6个月的骨性融合,而钛缆内固定不失为一种简便、有效、经济的固定技术。更多还原

【Abstract】 Background and Objective:Craniocervical junction malformation mainly refers to the occipital and atlas, atlas and vertebral developmental anomalies, including Basilar invagination, Atlantooccipital fusion, Atlantoaxial subluxation, Klippel-Feil syndrome, Arnold-Chiari malformation.These malformations may occur independently or several simultaneously.The structural stability of occipital bone, atlas and axis maintain the stability of the craniocervical junction together with pertaining(?)muscles, ligaments, fascias as well as joint capsules. The clinical manifestations of the instability of the craniocervical junction appear when the function and structure of atlanto-occipital joint and antalto-axial joint are destroyed, and the cause or excess movement in physiological state. When the cerebrospinal fluid is also obstructed at the foramen magnum, syringomyelia is often acomplicating factor.ChiariⅠmalformation is a complication occurring readily in the presence of such bony anomalies. Among the many malformations of the craniocervical junction, Chiari typeⅠsyndrome and syringomyelia are worthy of reseasch because of their prevalence and the seriousness of their symptom. Patients who frequently present with neurologic symptoms and deficits need warrant surgical treatment to prevent progression. Untreated craniocervical instability is associated with a high morbidity and a significant mortality.A variety of surgical interventions have been recommended for patients with craniocervical junction malformation, while their results are also different. So far, there has not been an ideal treatment method for the disease.Methods:141 adult cases with craniocervical junction malformation selected from January 2007 to March 2011 were divided into A, B and C group. According to different clinical and radiographic examination. They were underwent suboccipital craniectomy or transoral-transpharyngeal odontoidectomy as well as autogenous bone grafting and occipitocervical fusion. All operations were performed by a single surgeon at a single institution. The clinical outcome was assessed by evaluation of postoperative signs and symptoms and magnetic resonance imaging of the craniocervical junction.Results:Bone fusion and improved hydrocephalus were clear on images 1 year after surgery, and the postoperative course has been good. The effectiveness of three groups are 88.4%、82.6%、80.3% respectively. According to different type selection of corresponding deformity operation mode is effective.Conclusion:1. Decompression and stability reconstitution should be paid equal attention to pressure in the surgical treatment.2. A proper surgical decompression and fixed mode should be defined from the clinical features of the spinal cord and the cranial nerves. Both static and dynamic MRI scans must be performed; in this way identification of the neural abnormalities (hydromyelia, Chiari, etc.) and of the osseous compression is possible.3. The treatment of hydrocephalus and syringomyelia should also be surgical treated fundamentally.The tonsillectomy,the cutting of the pseduomembrane and the dura closing are the ideal treatment for Chiari malformation with syringomyelia.4. The stability of the craniovertebral junction abnormalities reconstruction mainly depend on the osseous fusion 3 to 6 months after the operation, and titanium cable internal fixation may be a simple, efficient and economic fixed technology.更多还原