颅颈交界区畸形的影像学分析及诊治策略

发布时间：2018-06-03 02:55

本文选题：单纯Chiari + Ⅰ型畸形　；参考：《浙江大学》2017年博士论文

【摘要】：第一部分单纯小脑扁桃体下疝畸形Ⅰ型(Chiari Ⅰ)的影像分析及诊治策略背景和目的单纯Chiari畸形的病理生理学基础为因为胚胎阶段中胚层体节枕骨部发育状况不佳,造成枕骨未能发育完全、后颅窝窄小,但小脑发育不受影响,所以后颅窝空间狭小,造成扁桃体疝入至椎管中。目前临床治疗Chiar Ⅰ型畸形的主要手术方式有:(1)后颅窝骨性减压术;(2)后颅窝膜性减压术;(3)后颅窝减压+脊髓空洞造瘘或分流术;(4)后颅窝减压+下疝小脑扁桃体切除+枕大池成形术。最近,有文献报道Chiari畸形与寰枢椎不稳定相关,采用后路的复位、固定术,获得满意的临床治愈效果和随访结果。为此,我们评估了单纯Chiari Ⅰ型畸形的后颅窝容积情况和寰枢椎稳定性。方法回顾检索我科在2010年8月至2016年10月期间,诊断为单纯小脑扁桃体下疝畸形Ⅰ型(ChiariⅠ型)的临床病例共65例。满足纳入标准,结合排除标准,共60例纳入研究。取正常人60例作为对照组。病例组和对照组均进行颅颈交界区的CT(Philip,256-slice)和 MRI(General Electric,3.0-tesla)扫描。所有研究对象的影像数据输入PACS影像浏览系统(这一系统具有特殊的数字测绘功能)进行测量,测量参数包括:小脑扁桃体下疝超过枕骨大孔的距离(E),延髓颈髓角(CMA),后颅窝脑容积(PFBV),后颅窝容积(PFCV),后颅窝容积相对比(RR=PFBV/PFCV),矢状位关节面倾斜角(SJI),冠状位关节面倾斜角(CJI),颅颈倾斜角(CT)。结果(1)小脑扁桃体下疝程度比较(E):两组之间存在显著性差异。(2)延髓颈髓角(CMA):两组之间无显著性差异。(3)后颅窝脑容积(PFBV):两组之间无显著性差异。(4)后颅窝容积(PFCV):两组之间无显著性差异。(5)后颅窝容积相对比(RR=PFBV/PFCV):两组之间存在显著性差异。(6)矢状位关节面倾斜角(SJI):两组之间无显著性差异。(7)冠状位关节面倾斜角(CJI):两组之间无显著性差异。(8)颅颈倾斜角(CT):两组之间无显著性差异。(9)在单纯Chiari Ⅰ型畸形组,小脑扁桃体下疝程度(E)与后颅窝容积相对比(RR=PFBV/PFCV)之间进行相关性分析,存在显著正相关性。结论在单纯ChiariⅠ型畸形中,枕骨轻度发育不良导致后颅窝相对狭小,后颅窝容积相对比(RR)增大,与小脑扁桃体下疝(E)严重程度显著正相关;寰枢椎稳定性良好,未见不稳定因素,与单纯Chiari Ⅰ型畸形的发生发展无显著相关性。临床诊治该类疾病应以扩大后颅窝容积和局部减压为重点。第二部分颅底凹陷合并寰枢椎脱位(GroupA型BI)的影像分析及诊治策略背景和目的颅底凹陷(BI)属于一类临床上广泛存在的颅颈交界区(CVJ)畸形。Goel与Laheri[1]根据是不是合并寰枢椎脱位(AAD)把颅底凹陷分成A、B两种类型。其病理生理学基础是因为机体发育阶段内颅颈相接处各类不正常发育导致颅底扁平、内翻,内陷等,进而对脑干、脊髓等神经造成压迫,最终形成各类神经性的神经脊髓综合征。颅底凹陷合并寰枢椎脱位(BIand AAD,Group A型BI)是临床诊治的重点和难点。目前国内外治疗颅底凹陷合并寰枢椎脱位采取的主要术式包括经后方入路的枕下减压+枕颈融合内固定术,寰枢椎复位+融合内固定术,经口腔齿状突切除+复位固定术,经口腔寰枢椎脱位松解+复位固定术,经枕颈侧方的远外侧入路或枕下后外侧入路齿状突切除术等[2,3]。归纳起来,其手术方式以前路/后路的减压+复位+固定和复位+固定为主。文献报道的颅底凹陷合并寰枢椎脱位主要与寰枢椎不稳定相关,而后颅窝发育情况如何,是否存在后颅窝容积相对狭窄?为此,我们评估了颅底凹陷合并寰枢椎脱位的后颅窝容积情况和寰枢椎稳定性。方法回顾检索我科在2010年8月至2016年10月期间,诊断为颅底凹陷合并寰枢椎脱位(Group A型BI)的临床病例共22例。满足纳入标准,结合排除标准,共20例纳入研究。取正常人20例作为对照组。病例组和对照组均进行颅颈交界区的CT(Philip,256-slice)和 MRI(General Electric,3.0-tesla)扫描。所有研究对象的影像数据输入PACS影像浏览系统(这一系统具有特殊的数字测绘功能)。测量参数包括:Chamberlain's line(CL),寰齿间距(ADI),颅底角(CBA),斜坡长度(DB),Boogard角,延髓颈髓角(CMA),后颅窝脑容积(PFBV),后颅窝容积(PFCV),后颅窝容积相对比(RR=PFBV/PFCV),矢状位关节面倾斜角(SJI),冠状位关节面倾斜角(CJI),颅颈倾斜角(CT)。结果(1)Chamberlain's line(CL):两组之间存在显著性差异。(2)寰齿间距(ADI):两组之间存在显著性差异。(3)颅底角(CBA):两组之间无显著性差异。(4)斜坡长度(DB):两组之间存在显著性差异。(5)Boogard角:两组之间存在显著性差异。(6)延髓颈髓角(CMA):两组之间存在显著性差异。(7)后颅窝脑容积(PFBV):两组之间无显著性差异。(8)后颅窝容积(PFCV):两组之间存在显著性差异。(9)后颅窝容积相对比(RR=PFBV/PFCV):两组之间存在显著性差异。(10)矢状位关节面倾斜角(SJI):两组之间存在显著性差异。(11)冠状位关节面倾斜角(CJI):两组之间无显著性差异。(12)颅颈倾斜角(CT):两组之间存在显著性差异。(13)在颅底凹陷合并寰枢椎脱位组,Chamberlain'sline(CL)与CMA之间存在显著负相关性;CL与RR之间存在显著正相关性。(14)在颅底凹陷合并寰枢椎脱位组,Chamberlain's line(CL)与矢状位关节面倾斜角(SJI)之间存在显著正相关性;Chamberlain's line(CL)与颅颈倾斜角(CT)之间无显著相关性。结论在颅底凹陷合并寰枢椎脱位中,枕骨重度发育不良导致后颅窝相对狭小,后颅窝容积相对比(RR)增大。寰枢椎发育不良导致寰枢椎侧方关节面倾斜角增大,并脱位失稳,与齿状突内陷(CL)严重程度显著正相关。临床诊治该类疾病应以复位寰枢椎脱位,并固定稳定寰枢椎为重点,建议同期行后颅窝减压扩大容积。第三部分颅底凹陷合并扁平颅底(Group B型BI)的影像分析及诊治策略背景和目的颅底凹陷(BI)属于一类临床上广泛存在的颅颈交界区(CVJ)畸形。Goel与Laheri[1]根据是不是合并寰枢椎脱位(AAD)把颅底凹陷分成A、B两种类型。其病理生理学基础是因为机体发育阶段内颅颈相接处各类不正常发育导致颅底扁平、内翻,内陷等,进而对脑干、脊髓等神经造成压迫,最终形成各类神经性的神经脊髓综合征。颅底凹陷合并扁平颅底(BI and platybasia,GroupB型BI)在临床研究及文献报道中较少提及。因其寰枢椎无脱位,相对稳定,所以目前国内外治疗颅底凹陷合并扁平颅底采取的主要术式包括经后方入路的枕下减压伴/不伴枕颈融合内固定术[4]。而最近Goel的临床研究认为[5],该类患者仍然存在寰枢椎不稳定,他仅予寰枢椎固定并重建稳定性,而不行后颅窝减压,同样取得了良好的临床疗效。那么,在颅底凹陷合并扁平颅底病例中,是否存在后颅窝发育不良导致后颅窝容积相对狭窄?是否存在寰枢椎稳定性问题?为此,我们评估了颅底凹陷合并扁平颅底的后颅窝容积情况和寰枢椎稳定性情况。方法回顾检索我科在2010年8月至2016年10月期间,诊断为颅底凹陷合并扁平颅底(Group B型BI)的临床病例共10例。满足纳入标准,结合排除标准,共10例纳入研究。取正常人10例作为对照组。病例组和对照组均进行颅颈交界区的CT(Philip,256-slice)和 MRI(General Electric,3.0-tesla)扫描。所有研究对象的影像数据输入PACS影像浏览系统(这一系统具有特殊的数字测绘功能)。测量参数包括:Chamberlain's line(CL),寰齿间距(ADI),颅底角(CBA),斜坡长度(DB),Boogard角,延髓颈髓角(CMA),后颅窝脑容积(PFBV),后颅窝容积(PFCV),后颅窝容积相对比(RR=PFBV/PFCV),矢状位关节面倾斜角(SJI),冠状位关节面倾斜角(CJI),颅颈倾斜角(CT)。结果(1)Chamberlain's line(CL):两组之间存在显著性差异。(2)寰齿间距(ADI):两组之间无显著性差异。(3)颅底角(CBA):两组之间存在显著性差异。(4)斜坡长度(DB):两组之间存在显著性差异。(5)Boogard角:两组之间存在显著性差异。(6)延髓颈髓角(CMA):两组之间存在显著性差异。(7)后颅窝脑容积(PFBV):两组之间无显著性差异。(8)后颅窝容积(PFCV):两组之间存在显著性差异。(9)后颅窝容积相对比(RR=PFBV/PFCV):两组之间存在显著性差异。(10)矢状位关节面倾斜角(SJI):两组之间无显著性差异。(11)冠状位关节面倾斜角(CJI):两组之间无显著性差异。(12)颅颈倾斜角(CT):两组之间存在显著性差异。(13)在颅底凹陷合并扁平颅底组,Chamberlain's line(CL)与延髓颈髓角(CMA)之间存在显著负相关性。Chamberlain's line(CL)与后颅窝容积相对比(RR)之间存在显著正相关性。(14)在颅底凹陷合并扁平颅底组,Chamberlain'sline(CL)与矢状位关节面倾斜角(SJI)之间无显著相关性。Chamberlain's line(CL)与颅颈倾斜角(CT)之间存在显著正相关性。结论在颅底凹陷合并扁平颅底中,枕骨重度发育不良导致后颅窝相对狭小,后颅窝容积相对比(RR)增大。扁平颅底导致斜坡内陷,颅颈倾斜角增大,与齿状突内陷(CL)严重程度显著正相关,未见明显寰枢椎失稳依据。临床诊治该类疾病应以后颅窝减压扩大容积为重点,前路减压内陷压迫的斜坡齿状突有待进一步商榷。
[Abstract]:The first part is the imaging analysis of type I (Chiari I) of the simple cerebellar tonsillar hernia type I (Chiari I), the background and objective of the diagnosis and treatment strategy. The basis of pathophysiology of simple Chiari malformation is that the development of the occipital part of the mesoderm is not good, the occipital bone is not developed completely and the posterior fossa is narrow, but the development of the cerebellum is not affected. After that, the development of the cerebellum is not affected. The space of the cranial fossa is small and causes the tonsillar hernia into the spinal canal. The main surgical methods for the treatment of Chiar type I deformities are: (1) posterior fossa osseous decompression; (2) posterior fossa membrane decompression; (3) posterior fossa decompression plus spinal cavities or shunt; (4) posterior cranial fossa decompression plus cerebellar tonsillectomy plus occipital large cistern plasty. The report of Chiari malformation was associated with atlantoaxial instability, using posterior reduction and fixation to obtain satisfactory clinical curative effects and follow-up results. To this end, we assessed the volume of posterior fossa and atlantoaxial stability in simple Chiari type I malformation. Methods a retrospective retrieval of our department from August 2010 to October 2016 was diagnosed as simple. 65 cases of cerebellar tonsillar hernia malformation (type Chiari type I) were found in a total of 65 cases. A total of 60 cases were included in the study. 60 cases of normal people were taken as the control group. Both the case group and the control group were scanned by CT (Philip, 256-slice) and MRI (General Electric, 3.0-tesla) in the craniofacial junction. Like data input PACS imaging browsing system (this system has special digital mapping function), the measurement parameters include: the distance from the cerebellar tonsil hernia over the occipital foramen (E), the medulla cervical angle (CMA), the volume of the posterior cranial fossa (PFBV), the volume of the posterior fossa (PFCV), the volume contrast of the posterior fossa (RR=PFBV/PFCV), and the tilted sagittal joint. Angle (SJI), coronal articular surface inclination (CJI) and craniofacial angle (CT). Results (1) the degree of hernia in the cerebellar tonsillar (E): there was a significant difference between the two groups. (2) the medulla cervical spinal cord angle (CMA): there was no significant difference between the two groups. (3) the posterior cranial fossa (PFBV): there was no significant difference between the two groups. (4) the posterior fossa volume (PFCV): there was no significant difference between the two groups. (5) volume contrast between the posterior fossa (RR=PFBV/PFCV): there were significant differences between the two groups. (6) the sagittal articular surface inclination (SJI): there was no significant difference between the two groups. (7) the coronal articular surface inclination (CJI): there was no significant difference between the two groups. (8) the craniofacial inclination (CT): there was no significant difference between the two groups. (9) in simple Chiari I There was a significant positive correlation between the degree of E of the cerebellar tonsillar and the volume contrast of the posterior fossa (RR=PFBV/PFCV), and there was a significant positive correlation. Conclusion in the simple Chiari type I malformation, mild hypoplasia of the occipital fossa resulted in a relatively small posterior fossa, the contrast of the posterior fossa volume (RR), and the severe course of the cerebellar tonsil hernia (E). There is no significant correlation between the atlantoaxial stability and the development of the Chiari type I deformity. The clinical diagnosis and treatment of this kind of disease should be focused on the expansion of the volume of the posterior fossa and local decompression. Second parts of the skull base depression combined with the atlantoaxial dislocation (GroupA type BI) and the background of the diagnosis and treatment strategy And objective skull base depression (BI) belongs to a class of clinically extensive craniofacial junction (CVJ) malformation (CVJ) malformation.Goel and Laheri[1] based on not merging atlantoaxial dislocation (AAD) to divide the skull base into A, B two types. Its pathophysiology is based on the abnormal development of the cranial and cervical joints in the body during the development of the body and causes the flat and varus of the skull base. Internal depression and so on, and then the brain stem, spinal cord and other nerves causing oppression, and eventually forming all kinds of nerve spinal cord syndrome. The skull base depression combined with the atlantoaxial dislocation (BIand AAD, Group A BI) is the key and difficult point in the clinical diagnosis and treatment. The main surgical methods for the treatment of the skull base depression with the atlantoaxial dislocation include the pillow of the posterior approach Hypobaric + occipital cervical fusion internal fixation, atlantoaxial reduction plus fusion internal fixation, oral odontoid resection plus reduction fixation, oral atlantoaxial dislocation release and reduction fixation, distal lateral approach or occipital lateral odontoid resection through occipital cervical lateral approach, [2,3]., and the decompression of anterior / posterior approach + recovery Position + fixation and reduction + fixation. The literature reports that the skull base depression combined with atlantoaxial dislocation is mainly associated with atlantoaxial instability. How is the development of the posterior fossa and the relative stenosis of the posterior fossa? To this end, we evaluated the volume of posterior fossa and atlantoaxial stability in the skull base depression combined with atlantoaxial dislocation. From August 2010 to October 2016, 22 cases were diagnosed as the clinical cases of the skull base depression combined with the atlantoaxial dislocation (Group A type BI), which met the inclusion criteria, combined with the exclusion criteria, 20 cases were included in the study. 20 normal persons were taken as the control group. The case group and the control group were both CT (Philip, 256-slice) and MRI (Gen) in the craniofacial junction area. Eral Electric, 3.0-tesla) scan. The image data of all the subjects are entered into the PACS image browsing system (the system has a special digital mapping function). The measurement parameters include: Chamberlain's line (CL), atlantoodontoid space (ADI), the skull base angle (CBA), the slope length (DB), Boogard angle, the medulla cervix angle (CMA), the posterior cranial fossa, the posterior cranial fossa Volume (PFCV), posterior fossa volume contrast (RR=PFBV/PFCV), sagittal articular surface inclination (SJI), coronal articular surface tilt angle (CJI), craniofacial inclination (CT). Results (1) Chamberlain's line (CL): there were significant differences between the two groups. (2) there was a significant difference between the two groups. (3) the skull base angle (CBA): there was no significant difference between the two groups. (4) slope length (DB): there was significant difference between the two groups. (5) Boogard angle: there was a significant difference between the two groups. (6) the medulla cervical spinal cord angle (CMA): there was a significant difference between the two groups. (7) the posterior cranial fossa volume (PFBV): there was no significant difference between the two groups. (8) the volume of posterior fossa (PFCV): there was a significant difference between the two groups. (9) posterior cranium Volume contrast (RR=PFBV/PFCV): there were significant differences between the two groups. (10) sagittal articular surface inclination (SJI): there was a significant difference between the two groups. (11) the coronal articular surface inclination (CJI): there was no significant difference between the two groups. (12) the craniofacial inclination (CT): there was a significant difference between the two groups. (13) the skull base depression combined with the atlantoaxial. There was a significant negative correlation between Chamberlain'sline (CL) and CMA, and there was a significant positive correlation between CL and RR. (14) there was a significant positive correlation between the CL (CL) and the sagittal oblique angle (SJI) in the skull base depression combined with the atlantoaxial dislocation group, and there was no significant difference between Chamberlain's line (CL) and the craniofacial inclination angle. Conclusion in the skull base depression with atlantoaxial dislocation, the posterior fossa of the posterior cranial fossa is relatively small and the volume contrast of the posterior fossa (RR) increases. Atlas and axis dysplasia leads to an increase in the oblique angle of the lateral articular surface of the atlantoaxial joint, and the dislocation of the dislocation and the severity of the odontoid inversion (CL). The disease should be based on the reduction of atlantoaxial dislocation and the fixation of the atlantoaxial compression, and the posterior cranial fossa decompression is recommended for the same period. Third parts of the skull base depression combined with flat skull base (Group B BI) image analysis and diagnosis and treatment strategy background and objective skull base depression (BI) belong to a class of clinically widespread craniofacial junction (CVJ) malformed.Goel and Lahe Ri[1] is based on not merging the atlantoaxial dislocation (AAD) into the two types of the skull base depression, which is divided into A and B types. Its pathophysiology basis is that the abnormal development of the cranial and cervical joints during the development of the body causes the flat, inverted, and internal depression of the skull base, and then oppresses the nerves of the brain stem and the spinal cord and eventually forms a variety of neurogenic neurospinal synthesis. Combination of the skull base depression with the flat skull base (BI and platybasia, GroupB BI) is rarely mentioned in clinical and literature reports. Because of its atlantoaxial without dislocation and relatively stable, the main surgical procedures for the treatment of the skull base depression with flat skull base include the posterior decompression of the occipital decompression with / without occipital cervical fusion internal fixation. The clinical study of [4]. and the recent clinical study of Goel believed that the patients of this class still had atlantoaxial instability, he only given the atlantoaxial fixation and rebuilt stability, but not the posterior fossa decompression, which also achieved good clinical efficacy. Then, in the case of cranial depression with flat skull base, there was a posterior fossa volume in the posterior fossa volume. Relatively narrow? Is there a problem of atlantoaxial stability? To this end, we evaluated the volume of the posterior cranial fossa and the stability of the atlantoaxial spine in the skull base depression with the flat skull base. Methods a total of 10 clinical cases diagnosed as the skull base depression with the flat skull base (Group B type BI) were retrieved in our department from August 2010 to October 2016. CT (Philip, 256-slice) and MRI (General Electric, 3.0-tesla) scan in the craniofacial junction were scanned in the case group and the control group. The image data of all the subjects were entered into the PACS image browsing system (the system has special digital mapping). The measurement parameters include: Chamberlain's line (CL), ADI, CBA, DB, Boogard angle, cervical spinal cord angle (CMA), volume of posterior cranial fossa (PFBV), volume of posterior fossa (PFCV), volume contrast of posterior fossa (RR=PFBV/PFCV), sagittal oblique angle (SJI), oblique angle of coronal joint, craniofacial inclination angle (CT) results (1) Chamberlain's line (CL): there were significant differences between the two groups. (2) there was no significant difference between the two groups. (3) the skull base angle (CBA): there was a significant difference between the two groups. (4) the slope length (DB): (5) Boogard angle: there was a significant difference between the two groups. (6) the cervical medulla angle of the medulla (CMA) There was significant difference between the two groups. (7) the volume of the posterior cranial fossa (PFBV): there was no significant difference between the two groups. (8) the volume of posterior fossa (PFCV): there was a significant difference between the two groups. (9) the volume contrast between the posterior fossa (RR=PFBV/PFCV): there was a significant difference between the two groups. (10) the sagittal articular surface inclination (SJI): there was no significant difference between the two groups. 11) the oblique angle of the coronal joint (CJI): there was no significant difference between the two groups. (12) the craniofacial inclination (CT): there was a significant difference between the two groups. (13) there was a significant negative correlation between the Chamberlain's line (CL) and the medulla cervical medullary angle (CMA) between the skull base depression and the medullary cervical spinal cord (CMA), and there was a significant negative correlation between.Chamberlain's line (CL) and the volume of the posterior fossa (RR). There was a significant positive correlation between the Chamberlain'sline (CL) and the sagittal articular surface inclination (SJI) in the skull base depression with the flat skull base, and there was a significant positive correlation between the.Chamberlain's line (CL) and the craniocal inclination (CT). Conclusion in the skull base depression with the flat Skull base, the severe hypoplasia of the occipital bone led to the severe hypoplasia of the occipital bone. The posterior fossa is relatively small, and the volume contrast (RR) of the posterior fossa increases. The flat skull base leads to the incline subsidence, the craniofacial inclination increases, and the severity of the odontoid invagination (CL) is significantly positively correlated, and there is no evidence of the atlantoaxial instability. The clinical diagnosis and treatment of the disease should focus on the enlarged volume of decompression of the cranial fossa in the future, and the clivus teeth oppressed by anterior decompression of the decompression of the anterior fossa It needs further discussion.
【学位授予单位】：浙江大学
【学位级别】：博士
【学位授予年份】：2017
【分类号】：R651.1

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