课件：先天畸形性寰枢椎脱位.ppt

枕骨-寰椎先天性融合 缺乏寰枕关节，致使寰枢关节活动加大，累积性劳损将使寰枢之间的韧带及关节囊松弛，从而发生寰枢关节的不稳，或在轻微外力后进行性加重。 颅底凹陷 颅底凹陷纵然有寰枢关节的存在，但是其活动范围会受到畸形骨质的限制，同时部分患者合并寰椎枕骨化。 齿状突发育不全 齿状突游离小骨 齿状突发育不良 齿状突缺如 Killipper-Feil综合征 又称短颈畸形，系指两个或两个以上的颈椎融合。 枢椎和其下相连的几个颈椎发生先天性融合伴有斜颈畸形，寰枢关节负荷增大。 THANK YOU SUCCESS * * 可编辑 Manifestations pyramidal signs (weakness and spasticity) appearence short neck ,low hairline ,facial asymmetry neck movement restriction and torticollis The history of transient unconsciousness or sudden neurological deterioration following minor trauma Imageology MRI helps to assess soft tissue anomalies, syringomyelia, the extent of cervicomedullary compression and cord changes. 3D-CT assessment of the bony configuration of the craniovertebral junction Chamberlain line extends between the posterior pole of the hard palate and the opisthion (posterior margin of the foramen magnum) Wackenhein clivus baseline a line along the clivus and extrapolating it inferiorly into the upper cervical spinal canal Ventral spinal cord compression may occur when the angle is less than 150° Diagnosis distance between the anterior arch of the atlas and the odontoid is greater than 3 mm in adults and 4.5 mm in children. Differential Diagnosis Traumatic AAD Spontaneous AAD Traumatic AAD 创伤引起的寰枢椎不稳或脱位比较常见，主要有以下几种类型： 1.寰椎椎弓骨折（Jefferson骨折）。 2.寰椎横韧带断裂 X线侧位片寰椎前脱位（ADI间隙5mm），而齿状突完整。 3.齿状突骨折。 Spontaneous AAD 非特异性炎症 颈部感染。如扁桃体炎、中耳炎、咽炎等均可致咽后壁淋巴结炎，从而使临近的寰枢关节产生反应性关节炎，引起关节积液、横韧带松弛。 类风湿关节炎 类风湿性关节炎最常见，炎症造成的齿状突周围骨、韧带结构的破坏在寰枢脱位的发生中起很大作用。 Treatment Preoperative management Anesthetic considerations Anterior decompression Posterior stabilization techniques Preoperative management Clinotherapy Cervical collar 枕颈区先天性畸形患者发生颈区疼痛，过伸过曲照片提示寰枢关节不稳，均应立刻给予颈托保护。 Skull traction or cervical traction 颅骨牵引复位后均应继之以融合手术，以保护脊髓。因为韧带结构的陈旧性损伤不可能完整修复而重建寰枢间的稳定性。 Anesthetic considerations During intubation, any sudden movements of the neck, especially flexion, should be st