High cervical C3-4 'disc' compression associated with basilar invagination

A 20-year-old male had torticollis and short neck since birth. He presented with symptom of progressive quadriparesis over a two-year period. Investigations revealed basilar invagination with marked rotation in the craniovertebral region and relatively large C3-4 region osteophytes. Serial MRI over two years showed persistent signal opposite C3-4 disc space suggestive of cord compression. Although the cord was humped over the odontoid process, there was no clear radiological evidence that the cord was compromised at this level. During surgery, instability was identified only at the craniovertebral region and not at the level of C3-4. Distraction of the lateral masses of atlas and axis and fixation using interarticular spacers and bone graft and direct screw implantation in the lateral mass of the atlas and pars of the axis resulted in reduction of the basilar invagination and of atlantoaxial dislocation. The patient had marked clinical recovery, despite the fact that no direct procedure was done for C3-4 disc decompression. The case suggests that C3-4 disc changes could be secondary to primary instability at the craniovertebral junction.     

复杂颅底凹陷畸形的外科治疗

目的分析总结复杂颅底凹陷畸形的临床表现及治疗方法。方法根据临床表现和影像学特征,对我院近9年经手术治疗的30例复杂颅底凹陷畸形病例分为Ⅰ组、Ⅱ组,Ⅰ组行后方入路枕下减压术和／或枕颈植骨融合内固定术,Ⅱ组行前路经口齿状突切除术和枕颈植骨融合内固定术。结果Ⅰ组17例有效率85％,Ⅱ组13例有效率83％。结论对于不同类型的复杂颅底凹陷畸形患者,选择不同的手术方式,对于提高术后疗效有重要作用。     

Visualization of basilar invagination by computerized tomography.

Computerized tomography was used to visualize bony masses in the posterior fossa in a patient with basilar invagination associated with symptoms of vertebrobasilar insufficiency.     

Goel技术治疗颅底凹陷及寰枢椎脱位

目的 探讨应用寰椎侧块螺钉、枢椎椎弓峡部螺钉棒内固定术治疗颅底凹陷合并寰枢椎脱位的可行性及临床疗效.方法 回顾分析76 例颅底凹陷合并寰枢椎关节脱位患者临床诊断与治疗经过,其中合并寰椎枕骨化畸形的寰枢椎脱位50 例,未合并寰椎枕骨化畸形的寰枢椎失稳和脱位26 例(齿状突不连性或发育不良性寰枢椎脱位14 例、未合并脱位的颅底凹陷经前路切除齿状突减压所致医源性寰枢椎不稳12 例).全部病例均采用寰椎侧块螺钉和枢椎椎弓峡部螺钉棒或下关节突螺钉棒系统进行复位固定,髂后上嵴松质骨颗粒植骨.结果 74 例获得满意临床治疗效果,出院时日本骨科协会(JOA)评分(17 分法)由术前的9.43 ± 3.16 提高至13.80 ± 2.07(t = 4.063,P = 0.037),Odom 评级优19 例、良49 例、可7 例、差1 例.未合并寰枕融合者经后路固定手术均获得解剖学复位;50 例伴寰枕融合患者中15 例完全复位、35 例部分复位;其中26 例经口腔入路施行减压.共50 例获3 个月以上随访,JOA 评分由术前的8.90 ± 1.22 提高至14.72 ± 1.57(t = 4.914,P = 0.015),Odom 评级优18 例、良30 例、可2 例、差0 例.随访期间未出现断钉、断板现象,内固定稳固、植骨完全融合.1 例术后清醒拔管12 h 突发呼吸、心跳停止,复苏成功后深度昏迷,家属放弃治疗出院;1 例术后第6 天发生全身凝血机制障碍,随后出现四肢完全瘫痪,目前仍然在康复治疗中,肌力恢复至3 级;2 例术后发生呼吸衰竭;2 例出现切口延迟愈合.无一例发生感染和后组脑神经损伤并发症.结论 应用Goel 内固定技术行寰枢椎关节复位、固定及植骨融合治疗畸形寰枢椎脱位安全可行,疗效满意.     

单纯经后路手术治疗先天性颅底陷入症合并的寰枢椎脱位

目的 探讨单纯经后路手术治疗先天性颅底陷入症合并寰枢椎脱位的效果.方法 2008年4月至2012年2月,对25例先天性颅底陷入症合并寰枢椎脱位患者,采用单纯经后路手术,术中行C2椎弓根和C1侧块或枕骨螺钉内固定及螺钉间撑开技术,对手术效果进行分析总结.结果 所有患者术前术后均未行经口齿状突切除.随访6～40个月,无患者出现症状恶化,22例患者症状有不同程度改善.术后影像学检查显示,所有患者均复位满意,减压充分,24例患者形成良好骨性融合.结论 单纯后路复位及固定结合术中螺钉间撑开技术,可以安全有效地治疗先天性颅底陷入症.     

Atlantoaxial joint distraction for treatment of basilar invagination secondary to rheumatoid arthritis

We present our experience of treating two cases of rheumatoid arthritis involving the craniovertebral junction and having marked basilar invagination by an alternative treatment method. In both the cases, the facets were osteoporotic and were not suitable for screw implantation. The patients were 66 and 72 years of age and both patients were females. Both the patients presented with complaints of progressively increasing spastic quadriparesis. Surgery involved attempts to reduce the basilar invagination and restore the height of the 'collapsed' lateral mass by manual distraction of the facets of the atlas and axis and forced impaction of titanium spacers in the joint in addition to bone graft harvested from the iliac crest. The procedure also provided stabilization of the region. No other fixation procedure involving wires, screws, plate and rods was carried out simultaneously. Following surgery both the patients showed symptomatic improvement and partial restoration of craniovertebral alignments. Follow-up is of 2 and 24 months. Distraction of the facets of atlas and axis and impaction of metal implant and bone graft in the facet joint can assist in reduction of basilar invagination and fixation of the region in selected cases of rheumatoid arthritis involving the craniovertebral junction.     

后路枕颈固定复位减压术治疗颅底凹陷症的疗效分析

目的 探讨后路枕颈固定复位减压术治疗颅底凹陷症的效果。方法 回顾性分析2015年6月至2020年1月采用单一后路枕颈固定复位减压术治疗的70例颅底凹陷的临床资料。记录病人报告的日本骨科学会（PRO-JOA）评分,用ΔPRO-JOA判断恢复程度,ΔPRO-JOA=（术后PRO-JOA评分-术前PRO-JOA评分）/（17-术前PRO-JOA评分）×100%,其中≥60%为手术效果较好,<60%为手术效果一般。结果 70例中,A型颅底凹陷[有寰枢椎脱位,寰椎齿状突间距（ADI）≥3 mm]28例,B型颅底凹陷症（无寰枢椎脱位,ADI<3 mm）42例。70例顺利完成手术,无手术死亡病例。70例术后随访12～60个月,平均（36.2±10.3）个月。A型末次随访,24例（85.71%）手术效果较好,4例手术效果一般。B型末次随访,33例（78.57%）手术效果较好,9例手术效果一般。结论 后路固定复位减压术治疗颅底凹陷症,A型和B型都可获得良好的疗效。     

"Top of the basilar" syndrome]

Three cases of "Top of the basilar" syndrome are reported. The clinical manifestations included the consciousness disturbance, pupillary and oculomotor abnormalities. One of the 3 cases got cortical blindness. It is emphasized that the possibility of the TOB syndrome should be considered when unconsciousness arose suddenly, accompanied by lid, pupil and oculomotor disturbances without the motor and sensory disturbances. Usually, a bilateral symmetric butterfly hypodensity could be found in the thalamus on CT scan. In addition. It was valuable findings that the infarct lesions have been shown in the mesencephalon, pons, cerebellum, occipital and medial temporal lobe on CT scans.     

I期术中复位经后路枕颈融合术治疗原发性颅底凹陷合并寰枢椎脱位

目的探讨原发性颅底凹陷合并寰枢椎脱位的临床特点、外科手术治疗方式及临床效果。方法回顾分析2008年1月-2011年12月住院治疗且经影像学检查明确诊断的89例原发性颅底凹陷合并寰枢椎脱位患者的临床资料,男性28例,女性61例;年龄10～69岁,平均45．42岁。经后正中人路I期施行复位器辅助寰枢关节复位,以两块AO钢板连接枕骨与第2,3颈椎侧块螺钉内固定,取自体髂骨行枕颈植骨融合,并随访观察手术效果。结果共随访6～48个月,大多数患者临床症状明显改善,日本骨科协会评分由术前的8．80±1．36增至术后的15．35±1．47,手术前后比较差异有统计学意义（t=17．225,P=0．001）;手术改善率达82．93％。手术前后影像学测量平均值比较,寰齿间距（9．22mm：3．72mm）和齿状突顶点至Chamberlain线垂直距离（10．41mm：3．23mm）减小,而延髓颈髓角（130°：1500）和脊髓可用空间（11．13mm：15．54mm）增加,4项指标均不同程度改善。结论术中I期复位辅助植骨融合内固定术治疗原发性颅底凹陷合并寰枢椎脱位操作步骤简单,安全性高,疗效确切,但远期手术疗效尚有待长期随访观察。     

分期前后方减压治疗合并颅底陷入的Chiari畸形

目的 合并颅底陷入的Chiari畸形患者常有前方脑干压迫,多数经前路减压即可使扁桃体上移,空洞缩小,但少数患者前路减压后Chiari畸形和脊髓空洞并不减轻,本文旨在探讨采用分期前后方减压治疗该类患者.方法 9例患者经口咽入路齿状突磨除,观察1-2个月后发现Chiari畸形和脊髓空洞未减轻,遂行后方枕大孔减压,小脑扁桃体切除,硬膜扩大修补,枕颈或C1～2固定融合术.随访24-60个月(平均32个月).结果 本组无死亡或严重并发症患者,所有患者症状改善,8例Chiari畸形和脊髓空洞改善,1例无变化.结论 合并颅底陷入的Chiari畸形患者应首先行经口咽入路齿状突磨除术,多数患者后方仅需固定即可,但有些患者颅底陷入症状缓解,而Chiari畸形依旧,需后方减压加固定手术.     

A case of Collet-Sicard syndrome associated with traumatic atlas fractures and congenital basilar invagination

An 18 year old man with congenital basilar invagination developed multiple lower cranial nerve (CN) palsies including CN IX to XII after a traffic accident. Computed tomography of his skull base revealed a two part atlas Jefferson fracture. Normally, lower cranial nerves (CN IX-XII) pass through a space between the styloid process and the atlas transverse process. Atlas burst fractures rarely cause neurological deficits because of a greater transverse and sagittal diameter of the spinal canal at the atlas, and a tendency of the lateral masses to slide away from the cord after injury. However, when associated with a rare condition-congenital basilar invagination-atlas fractures can compromise the space and make CN IX-XII more vulnerable to compression injury. This report discusses the correlation between the anatomical lesions and clinical features of this patient.     

显微镜下经口腔复位内固定术治疗颅底凹陷合并寰枢椎脱位

目的 探讨经口腔入路松解、复位内固定术在颅底凹陷合并寰枢椎脱位治疗中的应用价值,并评价其有效性及安全性.方法 对3 例不可复性寰枢椎脱位患者实施显微镜辅助下经口腔入路松解、复位内固定术,术中以经口腔复位内固定钛板作为前方固定,自体颗粒骨植骨.采用日本骨科协会(JOA)17 分评分系统进行手术前后颈脊髓神经功能评价,MRI 及X 线判断术后寰枢间距及延髓颈髓角改善程度.结果 3 例患者手术平均时间为130 min(110、130 和150 min);平均出血量为150 ml(100、200 和150 ml).术后CT 检查显示,3 例患者内固定钛板及12 枚椎弓根钛钉固定理想、无松动迹象,颅底凹陷及寰枢关节脱位得到不同程度纠正;颈脊髓神经功能明显改善,JOA评分改善率分别为75.00%、40.00%和56.25%,平均改善率为57.08%,平均JOA 评分改善率评级为良好.其中1 例患者术后并发颅内感染,经对症治疗痊愈.结论 显微镜辅助下经口腔入路松解、复位内固定术治疗颅底凹陷合并寰枢椎脱位具有一定临床应用价值.     

斜坡-齿状突型颅底陷入10例临床分析

目的 探讨经口咽前路减压手术治疗斜坡-齿状突型颅底陷入症的疗效。方法 本组10例，MRI主要表现为斜坡内陷，齿状突突入颅内，延髓及颈段脊髓腹侧受压变形有，形中3例合并枕骨内陷，小脑扁桃体下疝及脊髓空洞。手术切除颅颈部腹侧骨性畸形和增生的结缔组织，解除延髓、脊髓腹侧的压迫。结果 痊愈7例，好转2例，无效1例。无死亡和脑脊液漏，术后软腭裂开2例，经再次修复后愈合。结论 颅底陷入尤其是斜坡—齿状突型是经口腔前路减压的主要适应证；早期诊断与充分减压是获得良好疗效的关键。     

颅颈部蛛网膜下腔改变对颅底陷入在外科治疗中的临床意义

目的探讨颅颈交界区蛛网膜下腔形态学改变对颅底陷入患者在外科治疗中的意义。方法回顾分析两种不同手术方法治疗20例颅底陷入患者的临床资料．单纯行后颅窝减压术患者（A组）与经口齿状突切除术合并后方植骨融合固定术患者（B组）进行蛛网膜下腔形态学改变的对比。结果影像学上：B组比A组蛛网膜下腔容积增加约12．58mL，枕大池亦明显扩大：临床上：A组2例患者症状明显改善、2例症状好转、6例无变化，B组8例症状明显改善、2例症状好转。结论蛛网膜下腔形态学改变对颅底陷入手术预后的评估有重要的参考价值。     

Ⅰ期术中复位经后路枕颈融合术治疗原发性颅底凹陷合并寰枢椎脱位

目的探讨原发性颅底凹陷合并寰枢椎脱位的临床特点、外科手术治疗方式及临床效果。方法回顾分析2008年1月-2011年12月住院治疗且经影像学检查明确诊断的89例原发性颅底凹陷合并寰枢椎脱位患者的临床资料,男性28例,女性61例;年龄10~69岁,平均45.42岁。经后正中入路Ⅰ期施行复位器辅助寰枢关节复位,以两块AO钢板连接枕骨与第2,3颈椎侧块螺钉内固定,取自体髂骨行枕颈植骨融合,并随访观察手术效果。结果共随访6~48个月,大多数患者临床症状明显改善,日本骨科协会评分由术前的8.80±1.36增至术后的15.35±1.47,手术前后比较差异有统计学意义(t=17.225,P=0.001);手术改善率达82.93%。手术前后影像学测量平均值比较,寰齿间距(9.22mm∶3.72mm)和齿状突顶点至Chamberlain线垂直距离(10.41mm∶3.23mm)减小,而延髓颈髓角(130°∶150°)和脊髓可用空间(11.13mm∶15.54mm)增加,4项指标均不同程度改善。结论术中Ⅰ期复位辅助植骨融合内固定术治疗原发性颅底凹陷合并寰枢椎脱位操作步骤简单,安全性高,疗效确切,但远期手术疗效尚有待长期随访观察。     

直接后路加压器械撑开复位枕颈融合术治疗颅底凹陷症合并寰枢椎脱位的疗效

目的 探讨直接后路加压器械撑开复位枕颈融合术治疗颅底凹陷症伴寰枢椎脱位的安全性和有效性。方法 回顾性分析2017年1月至2018年1月采用枕颈融合术治疗的19例颅底凹陷症伴寰枢椎脱位的临床资料。采用日本骨科协会（JOA）评分和Ranawat分级评估神经功能恢复情况,测量齿状突至钱氏线（CL）、颅底斜坡切线（WL）、枕孔线（ML）评估垂直脱位情况,测量寰齿间距（ADI）距离评估水平脱位情况,测量颈髓角（CMA）评估脊髓受压情况。19例平均随访（17.47±6.65）个月。结果 末次随访时,症状明显改善17例,有改善但不明显2例。至末次随访JOA评分[（15.63±1.12）分]明显高于术前[（11.00±2.40）分;P<0.05]。神经功能改善率平均76%,其中优14例（73.68%）,良5例（26.32%）。术前Ranawat分级为Ⅱ级7例、ⅢA级11例、ⅢB级1例;末次随访时Ⅰ级14例,Ⅱ级5例。19例末次随访时均获得50%以上的复位,其中18例CL完全复位,16例WL完全复位,15例ML完全复位,17例ADI完全复位,17例CMA恢复正常。结论 直接后路加压器械撑开复位枕颈融合术治疗颅底凹陷症伴寰枢椎脱位,不仅能够显著改善脊髓受压情况,而且能够达到良好复位     

Impaired vasoreactivity of the basilar artery system in patients with brainstem lacunar infarcts.

BACKGROUND AND PURPOSE: Diminished vasoreactivity (VR) has been evidenced in patients with hemispheric small vessel disease, however, there is no data regarding vertebrobasilar (VB) territory VR changes in patients with subcortical vascular encephalopathy located in the brainstem. Therefore, we compared the cerebral blood flow velocity (CBFV) responses of the VB system during different vasoregulatory challenges in healthy volunteers to those in patients with brainstem lacunar infarcts. METHODS: In 20 patients with brainstem lacunar infarcts and in 10 healthy volunteers the VR of the VB system was measured by analyzing the CBFV changes during different stimulation paradigms (ventilation, tilting and acetazolamide tests). During transcranial Doppler registration the systemic blood pressure and the expiratory partial CO2 pressure were monitored. RESULTS: During hypercapnia the VR was significantly higher in the control group than in the patient group (10.1 +/- 4.9 vs. 3.4 +/- 5.0 cm/s/kPa, p = 0.0025). In a subgroup of patients with mean baseline CBFV <25 cm/s the VR was 1.5 +/- 2.4 cm/ s/kPa, while patients with mean baseline CBFV >25 cm/s showed a significantly higher value (7.8 +/- 6.9 cm/s/kPa). Furthermore, in patients with mean baseline CBFV <25 cm/s the pulsatility index was significantly higher than in patients with mean baseline CBFV >25 cm/s (1.11 +/- 0.26 vs. 0.86 +/- 0.19, p = 0.0325), reflecting significantly higher vascular resistance in the former group. Although CBFV measurements during tilting and acetazolamide tests tended to support these findings, they showed no significant differences between patients and controls. CONCLUSION: Patients with cerebral microangiopathy involving the brainstem showed impaired VR in the VB flow territory in association with baseline CBFV.     

基底动脉发育不全患者脑干血流动力的变化及临床意义

目的探讨基底动脉发育不全(basilar artery hypoplasia,BAH)对脑干血流动力学变化的影响。方法 2011-01-2014-12对952例头晕或眩晕患者进行头颅CT、MRI和MRA检查筛查出符合研究条件BAH患者67例,符合标准的非BAH患者108例作为对照组。对2组患者记录血管危险因素,完成动态磁敏感增强灌注成像(PWI)检查、脑干听觉诱发电位(BAEP)、瞬目反射(BR)、经颅多普勒超声(TCD)。结果BAH组TCD基底动脉异常42例(62.7%),非BAH组异常14例(12.9%),差异有统计学意义(P0.001);主要为低血流速度和低搏动指数。BAH组脑桥出现低灌注26例(38.8%),非BAH组脑桥出现低灌注10例(9.3%),差异有统计学意义(P0.001),以平均通过时间(MTT)延长最多见。BAH组合并后循环梗死19例(28.4%),非BAH组后循环梗死6例(5.6%),差异有统计学意义(P=0.023)。BAH组BAEP异常48例(71.6%),非BAH组BAEP异常15例(13.8%),差异有统计学意义(P0.001);以Ⅲ~Ⅴ/Ⅰ~Ⅲ1最常见。BAH组BR异常51例(76.1%);非BAH组BR异常19例(17.6%),差异有统计学意义(P0.001);以R2波异常率最高(69.5%)。结论 BAH患者易出现脑桥部位的血流动力学改变,局部低灌注可能是BAH促发缺血性眩晕的原因之一。