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1、颅颈交界畸形termCraniocervical junctionCraniovertebral junctionMalformationDeformityAnomalyabnormalities Craniocervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that decrease the space for the lower brain stem an
2、d cervical cord.颅颈交界畸形分类Congenital: Structural abnormalities :Os odontoideum (齿状突小骨)Atlas assimilation (寰枕融合)Congenital Klippel-Feil malformation (先天性颈椎融合畸形,短颈畸形)Atlas hypoplasia (寰椎发育不良)Chiari malformations (小脑扁桃体下疝畸形)General or systemic disorders :Achondroplasia (先天性软骨发育不全,引起骨骼畸形)Down syndrome(唐氏综
3、合征,成骨不全)Acquired:Injuries(外伤)RA (类风关或成骨病) 引起寰枕畸形,扁平颅底,颅骨陷入Metastatic tumors (转移瘤)(引起寰枢椎畸形)Slowly growing craniocervical junction tumors (缓慢生长疾病)Many patients have multiple abnormalities.Symptoms and SignsSymptoms and signs can occur after a minor neck injury or spontaneously and may vary in progress
4、ion. Presentation varies by degree of compression and by structures affected.Neck pain, often with headacheSymptoms and signs of spinal cord compressionSymptoms and Signs1.Neck pain often spreads to the arms and may be accompanied by headache it is attributed to compression of the C2 root and the gr
5、eater occipital nerve and to local musculoskeletal dysfunction. Neck pain and headache usually worsen with head movement and can be precipitated by coughing or bending forward. If patients with Chiari malformation have hydrocephalus, being upright may aggravate the hydrocephalus and result in headac
6、hes.Symptoms and Signs2.Spinal cord compressionspastic paresis in the arms, legs, or both, caused by compression of motor tracts. Joint position and vibration senses (posterior column function) are commonly impaired. Tingling down the back, often into the legs, with neck flexion may occur. Uuncommon
7、ly, pain and temperature senses are impaired3.Neck appearance, range of motion The neck may be short,or in an abnormal position (eg, torticollis in Klippel-Feil malformation). Range of motion may be limited4.Brain compression (eg, due to platybasia, basilar invagination, or craniocervical tumors) ma
8、y cause brain stem, cranial nerve, and cerebellar deficits. sleep apnea窒息, hoarseness嘶哑, dysarthria构音, and dysphagia吞咽. Cerebellar deficits usually impair coordination5.Vertebrobasilar ischemiacan be triggered by changing head position. Syncope晕厥, drop attacks, vertigo, confusion or altered consciou
9、sness, weakness, and visual disturbance. 6.Syringomyelia common in patients with Chiari malformation. Segmental weakness and atrophy, which first appear or are most severe in the distal upper extremities; pain and temperature senses may be lost but light touch is preservedDiagnosisMRI CTPlain x-rays
10、of the brain and upper spinal cordTreatmentReduction and immobilization(牵引,颈托)surgical decompression, fixation, or both.(手术,内固定)Others (Radiation therapy , drug.放疗,药物)颅颈交界畸形的主要手术方式单纯后弓切除减压+枕大孔扩大+后路枕颈融合术寰椎后弓切除减压+枕大孔扩大+小脑扁桃体切除+硬膜扩大修补术寰椎后弓切除减压+枕大孔扩大+小脑扁桃体切除+硬膜扩大修补术+枕颈融合术经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术经口咽入路
11、寰椎前弓及齿状突切除减压+II期后路枕颈融合术单纯后路寰枢椎融合术Case 1Chiari 畸形Case 2寰枕畸形寰椎后弓切除减压+枕大孔扩大+小脑扁桃体切除+硬膜扩大修补术+枕颈融合术寰椎后弓切除减压+枕大孔扩大+小脑扁桃体切除+硬膜扩大修补术+枕颈融合术寰椎后弓切除减压+枕大孔扩大+小脑扁桃体切除+硬膜扩大修补术+枕颈融合术Case 3寰枕畸形经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术经口咽入路寰椎前弓及齿状突切除减压+I期后路枕颈融合术
外科学教学课件：颅颈交界畸形的外科处理策略
