Platybasia

  • 文章类型: Journal Article
    目的:研究(1)基底动脉内陷(BI)合并寰枢关节脱位(AAD)患者和健康个体的寰枢关节外侧形态及其与脱位严重程度的关系;(2)LAA形态对术后复位度(RD)的影响。
    方法:在这项回顾性倾向评分匹配的病例对照研究中,收集2011年至2022年62例BI和AAD患者的影像学和基线资料.无枕颈交界畸形的六十三名参与者作为对照。采用Logistic回归和受试者工作特征(ROC)曲线进行分析。
    结果:年龄,倾向评分匹配后,两组的BMI和性别没有显着差异。矢状倾斜角(SSA)和冠状倾斜角(CSA)较小和较大,分别,患者组高于对照组。负SSA值通常表示前转换的LAA。回归分析显示,SSA与脱位严重程度之间存在显着负相关。然而,未发现CSA与脱位严重程度之间的关系。多变量逻辑回归分析显示,最小SSA是令人满意的降低(RD≥90%)的独立预测因子。ROC曲线显示曲线下面积为0.844,截止值设定为-40.2。
    结论:患者组的SSA明显小于对照组,并且更加不对称。脱位严重程度与SSA有关,但与CSA无关。最小SSA可作为术后水平RD的预测指标。
    OBJECTIVE: To investigate (1) lateral atlantoaxial articulation (LAA) morphology in patients with basilar invagination (BI) with atlantoaxial dislocation (AAD) and healthy individuals and its relationship with the severity of dislocation and (2) the effect of the LAA morphology on reduction degree (RD) after surgery.
    METHODS: In this retrospective propensity score matching case-control study, imaging and baseline data of 62 patients with BI and AAD from 2011 to 2022 were collected. Six hundred thirteen  participants without occipitocervical junctional deformity served as controls. Logistic regression and receiver operating characteristic (ROC) curve were used for analysis.
    RESULTS: The age, BMI and sex did not differ significantly between the two groups after propensity score matching. Sagittal slope angle (SSA) and coronal slope angle (CSA) was lower and greater, respectively, in the patient group than in the control group. A negative SSA value usually indicates anteverted LAA. Regression analysis revealed a significant negative correlation between SSA and severity of dislocation. However, no relationship was found between CSA and the severity of dislocation. The multivariate logistic regression analysis revealed that minimum-SSA emerged as an independent predictor of satisfactory reduction (RD ≥ 90%). The ROC curve demonstrated an area under the curve of 0.844, with a cut-off value set at -40.2.
    CONCLUSIONS: SSA in patients group was significantly smaller and more asymmetric than that in the control group. Dislocation severity was related to SSA but not to CSA. Minimum-SSA can be used as a predictor of horizontal RD after surgery.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
    目的:基底动脉压迫(BI)是一种罕见但令人衰弱的颅骨交界处(CVJ)异常,已知会导致危及生命的延髓脑干压迫。我们的研究分析了BI的手术方法和相关结果。
    方法:使用PubMed进行了系统评价,谷歌学者,根据系统审查和荟萃分析(PRISMA)指南的首选报告项目和WebofScience电子数据库,以严格评估检查BI的主要文章。
    结果:我们分析了65篇文献中的87例患者,以女性为主(55.17%),平均年龄46.31±17.94岁,通常表现为运动缺陷(59.77%)和感觉缺陷(55.17%)。常用的手术包括枕颈后路融合术(24.14%),前路减压(20.69%),联合前路减压后路融合(21.84%)。经前路入路的患者年龄较大(55.38±17.67vs.45.49±18.78岁,p<0.05),并且从症状发作到手术的持续时间更长(57.39±64.33vs.26.02±29.60个月,p<0.05)与后入路相比。我们的分析显示,从症状发作到手术的持续时间较长与进行齿状突切除术和减压的可能性增加之间存在显着关联(OR:1.02,95%CI:1.00-1.03,p<0.05)。此外,在调整所有其他协变量后,类风湿关节炎病史和使用后路手术与术后并发症风险升高显著相关(p<0.05).
    结论:复杂CVJ疾病的治疗方法应根据外科医生的经验和压迫性病理的性质进行调整。
    OBJECTIVE: Basilar impression (BI) is a rare yet debilitating abnormality of the craniovertebral junction, known to cause life-threatening medullary brainstem compression. Our study analyzes surgical approaches for BI and related outcomes.
    METHODS: A systematic review was conducted using PubMed, Google Scholar, and Web of Science electronic databases according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines to critically assess primary articles examining BI.
    RESULTS: We analyzed 87 patients from 65 articles, mostly female (55.17%) with a mean age of 46.31 ± 17.94 years, commonly presenting with motor (59.77%) and sensory deficits (55.17%). Commonly employed procedures included posterior occipitocervical fusion (24.14%), anterior decompression (20.69%), and combined anterior decompression with posterior fusion (21.84%). Patients who underwent anterior approaches were found to be older (55.38 ± 17.67 vs. 45.49 ± 18.78 years, P < 0.05) and had a longer duration from symptom onset to surgery (57.39 ± 64.33 vs. 26.02 ± 29.60 months, P < 0.05) compared to posterior approaches. Our analysis revealed a significant association between a longer duration from symptom onset to surgery and an increased likelihood of undergoing odontoidectomy and decompression (odds ratio: 1.02, 95% confidence interval: 1.00-1.03, P < 0.05). Furthermore, after adjusting for all other covariates, a history of rheumatoid arthritis and the use of a posterior approach were significantly associated with an elevated risk of postoperative complications (P < 0.05).
    CONCLUSIONS: The treatment approach to complex craniovertebral junction disease should be tailored to the surgeon\'s experience and the nature of the compressive pathology.
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  • 文章类型: Journal Article
    目的:比较正常人和诊断为颅骨交界处异常的颈静脉孔(JF)的直径,例如Chiari畸形I型(CMI)和/或基底内陷(BI)。
    方法:这项横断面分析研究测量了患者磁共振成像中右侧和左侧JF的直径,这些患者分为四组:68例合并CMI和BI(CMIBI),42与孤立的BI,45与分离的CMI,和102个对照病例。t检验确定性别差异,而单变量方差分析与Tukey的事后检验评估了组间的JF差异。
    结果:考虑到所有侧向性和性别的组合,CMI+BI的JF比它们各自的对照更窄。JF在BI方面比对照的两侧更窄,只有女性。不管横向,男性CMI的JF比CMI+BI宽,它与控件没有区别。在CMI的女性中,正确的JF与控件没有区别,它比CMI+BI宽;而左JF比对照窄,它与CMI+BI没有区别。
    结论:当在同一患者中发现CMI和BI合并时,JF狭窄与CMI和BI相关。当分别检测到CMI或BI时,它们并不意味着JF形态测量的明显变化。预计这些发现可能会带来经验性的基础,以支持声称颅内压紊乱可能作为CMI触发因素的理论。
    OBJECTIVE: We compared the diameter of the jugular foramen (JF) between normal individuals and those with a diagnosis of craniovertebral junction abnormalities, such as Chiari malformation type I (CMI) and/or basilar invagination (BI).
    METHODS: This cross-sectional analytical study measured the diameter of the right and left JF on magnetic resonance imaging scans of patients divided into 4 groups: 68 with combined CMI and BI (CMI+BI), 42 with isolated BI, 45 with isolated CMI, and 102 control cases. The t test determined sex differences, and univariate analysis of variance with Tukey\'s post hoc test assessed JF differences between groups.
    RESULTS: Considering all combinations of laterality and sex, the CMI+BI group had a JF narrower than their respective control groups. The JF was bilaterally narrower in the BI group than in the control group only in the women. Regardless of laterality, the CMI group had a wider JF than did the CMI+BI group in men and did not differ from that of the control group. In the female patients with CMI, the right JF did not differ from that of the controls and was wider than that in the CMI+BI group. Also, the left JF was narrower than that in the control group and did not differ from that in the CMI+BI group.
    CONCLUSIONS: JF narrowing is associated with CMI and BI when combined in the same patient. When CMI or BI are detected separately, they do not imply evident changes in JF morphometry. It is expected that these findings could bring empiric substrate to support theories that claim a possible role of intracranial pressure disturbances as a triggering factor for CMI.
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  • 文章类型: Case Reports
    目的和背景:提出一种治疗基底内陷患者的新技术。基底内陷(BI)是一种先天性疾病,可以压迫颈髓质交界处,导致神经功能缺损.严重病例需要手术干预,但是关于方法的选择存在争议。前路手术可以直接减压,但并发症发生率高,而后入路提供间接减压,稳定性好,并发症少。材料和方法:一名15岁男孩患有严重的脊髓病,颈部疼痛,双侧上肢肌肉无力,手麻持续4年。此外,在访问前三个月,他经历了麻木和步态障碍的增加。在检查中,他在上肢和下肢都表现出反射亢进,双侧上肢肌肉无力(MMT4),肘部以下和双腿的双侧感觉减退,轻度尿失禁,步态痉挛.射线照片显示严重的基底内陷(BI)。术前图像显示严重的BI,并且齿状突严重压迫了脊髓。结果:患者采用无C臂技术进行后路手术。包括枕骨螺钉在内的所有螺钉均在导航引导下插入适当的位置。颅骨旋转和牵引可实现复位。在一年的随访显示以下结果:手动肌肉测试结果和感觉功能测试显示几乎完全康复,双侧手臂恢复(MMT5)和平稳行走。患者的颈椎日本骨科协会评分从9/17提高到16/17。术后图像显示良好的脊髓减压,无重大或严重并发症发生。结论:基底内陷与Klippel-Feil综合征并存是一种相对罕见的疾病。使用后路入路用无C臂技术治疗可还原BI被证明是解决严重脊髓病的安全方法。这种新颖的导航技术对BI患者产生优异的结果。
    Objectives and Background: To present a novel technique of treatment for a patient with basilar invagination. Basilar invagination (BI) is a congenital condition that can compress the cervicomedullary junction, leading to neurological deficits. Severe cases require surgical intervention, but there is debate over the choice of approach. The anterior approach allows direct decompression but carries high complication rates, while the posterior approach provides indirect decompression and offers good stability with fewer complications. Materials and Methods: A 15-year-old boy with severe myelopathy presented to our hospital with neck pain, bilateral upper limb muscle weakness, and hand numbness persisting for 4 years. Additionally, he experienced increased numbness and gait disturbance three months before his visit. On examination, he exhibited hyperreflexia in both upper and lower limbs, muscle weakness in the bilateral upper limbs (MMT 4), bilateral hypoesthesia below the elbow and in both legs, mild urinary and bowel incontinence, and a spastic gait. Radiographs revealed severe basilar invagination (BI). Preoperative images showed severe BI and that the spinal cord was severely compressed with odontoid process. Results: The patient underwent posterior surgery with the C-arm free technique. All screws including occipital screws were inserted into the adequate position under navigation guidance. Reduction was achieved with skull rotation and distraction. A follow-up at one year showed the following results: Manual muscle testing results and sensory function tests showed almost full recovery, with bilateral arm recovery (MMT 5) and smooth walking. The cervical Japanese Orthopedic Association score of the patient improved from 9/17 to 16/17. Postoperative images showed excellent spinal cord decompression, and no major or severe complications had occurred. Conclusions: Basilar invagination alongside Klippel-Feil syndrome represents a relatively uncommon condition. Utilizing a posterior approach for treating reducible BI with a C-arm-free technique proved to be a safe method in addressing severe myelopathy. This novel navigation technique yields excellent outcomes for patients with BI.
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  • 文章类型: Case Reports
    ChiariI畸形是后颅窝经常遇到的异常,发生在相当大比例的人口中。它通常与其他各种颅骨交界处异常共存,尽管Klippel-Feil综合征的频率较低。有趣的是,大多数ChiariI畸形患者没有任何症状。我们介绍了一个罕见的25岁男性慢性颈部和枕骨疼痛的病例,伴随着四肢的进行性虚弱和感觉障碍,尿急,并抬高了左肩。检查揭示了痉挛,弱点,反应敏捷.关于广泛的放射学评估(X射线,CT,和MRI),发现揭示了颅骨交界处的各种异常,包括完全的寰枕骨同化,基底内陷,和铂金。此外,观察到指示Klippel-Feil综合征的宫颈分割异常,还有Sprengel的畸形.MRI证实ChiariI畸形伴扁桃体疝和骨髓软化,以及颈椎-延髓交界处的压迫。该患者接受了手术,包括经口齿状突切除术联合枕颈固定术,之后观察到良好的临床反应。它强调了放射学成像对于诊断Chiari和颅骨交界处其他相关异常的必要性。
    A Chiari I malformation is a frequently encountered anomaly of the posterior fossa, occurring in a notable percentage of the population. It often coexists with various other craniovertebral junction abnormalities, albeit less frequently with Klippel-Feil syndrome. Interestingly, the majority of individuals with Chiari I malformation do not exhibit any symptoms. We present a rare case of a 25-year-old male with chronic neck and occipital pain, along with progressive weakness and sensory disturbances in all four limbs, urinary urgency, and elevated left shoulder. Examination unveiled spasticity, weakness, and brisk reflexes. On extensive radiological evaluation (X-ray, CT, and MRI), findings revealed various anomalies in the craniovertebral junction, including complete atlanto-occipital assimilation, basilar invagination, and platybasia. Furthermore, cervical segmentation abnormalities indicative of Klippel-Feil syndrome were observed, along with Sprengel\'s deformity. MRI confirmed Chiari I malformation with tonsillar herniation and myelomalacia, as well as compression at the cervico-medullary junction. This patient underwent a surgical procedure that included transoral odontoidectomy combined with occipito-cervical fixation, after which a good clinical response was observed. It emphasizes the necessity of radiological imaging for the diagnosis of Chiari and other associated abnormalities in the craniovertebral junction.
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  • 文章类型: Case Reports
    继发性基底内陷或基底印象是颅骨交界处的异常,其中齿状突脱出进入大孔,有可能压缩相邻结构并阻碍脑脊液(CSF)的正常流动。在一般人群中,发病率不到1%,主要发生在生命的前三十年,与神经轴畸形有关。在老年,主要病因是改变骨矿物质密度的疾病。临床过程通常是进行性的,最常见的症状是虚弱,颈椎疼痛和活动受限,还有发音障碍,呼吸困难和吞咽困难。这是一种进行性疾病,如果不及时治疗,会导致严重的神经损伤和死亡.我们报道了一个79岁的女性骨质疏松症和进行性吞咽困难导致严重营养不良的病例,由于围手术期风险高,决定不干预。
    Secondary basilar invagination or basilar impression is an anomaly at the craniovertebral junction where the odontoid process prolapses into the foramen magnum with the risk of compressing adjacent structures and obstructing the proper flow of cerebrospinal fluid (CSF). The incidence is less than 1% in the general population and occurs mainly in the first three decades of life when it is associated with malformations of the neuroaxis. In older age, the main aetiologies are diseases that alter bone mineral density. The clinical course is usually progressive and the most common symptoms are asthenia, cervical pain and restricted movement, but also dysphonia, dyspnoea and dysphagia. It is a progressive disease which, if left untreated, can cause severe neurological damage and death. We report the case of a 79-year-old woman with osteoporosis and progressive dysphagia leading to severe malnutrition, which conditioned the decision not to intervene due to the high perioperative risk.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    报告一例颅底发育异常(platybasia,基底内陷和C1-C2椎体融合);并在无法手术的情况下强调非手术治疗,以提高患者的生活质量。
    该病例报告为对一名17岁女性的描述性研究,该女性被送往Wardha的农村教学三级保健医院,马哈拉施特拉邦,印度;自10岁以来,主要抱怨四肢无力。
    白斑是由于发育异常导致的枕骨和上颈椎的发育缺陷。这种异常的机制尚不清楚;然而,最被接受的理论包括反常的基底-基底发育。压力效应可能会出现类似于进行性痉挛性瘫痪的症状,小脑症状,或者颅神经麻痹,除了肌肉骨骼症状.是的,因此,对于熟悉临床表现和放射学发现的医生和放射科医生来说至关重要。在以下颅底异常患者的情况下,鉴于运动症状逐渐恶化,建议进行手术干预,然而,由于涉及高风险,监护人拒绝了同样的选择。由于资金紧张,基因研究负担不起,缺乏对疾病的认识阻碍了监护人对疾病的明确管理做出决定。除了根治性神经外科手术,强化物理治疗对于显著改善患者的生活质量至关重要。
    UNASSIGNED: To report a case of anomalous development of base of skull (platybasia, Basilar invagination and C1-C2 vertebral fusion); and emphasize nonsurgical management in inoperable cases that can improve quality of life of the patient.
    UNASSIGNED: The case is reported as a descriptive study of a 17-year-old female who presented to a rural teaching tertiary care hospital in Wardha, Maharashtra, India; with chief complaints of weakness in all four limbs since 10 years of age.
    UNASSIGNED: Platybasia is a developmental defect of the occipital bone and upper cervical spine resulting from anomalous development. The mechanism of such anomalies is not known; however, the most accepted theory includes abnormal basi-occiput development. The pressure effects may present signs similar to progressive spastic paralysis, cerebellar symptoms, or cranial nerve palsy, in addition to musculoskeletal symptoms. It is, therefore, crucial for physicians and radiologists to be familiar with clinical manifestations and radiological findings. In the following case of a patient with base of skull anomalies, surgical intervention in view of progressive worsening of motor symptoms was advised, however, the guardians declined the same due to high risk involved. Due to financial constraints, genetic studies were unaffordable, and a lack of awareness regarding the disease hampered the guardians from making a decision on the definite management of the disease. Besides radical neurosurgery, intensive physiotherapy can prove vital in significantly improving the quality of life for the patient.
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