A novel classification and its clinical significance in Chiari I malformation with syringomyelia based on high-resolution MRI

Guan, Jian; Yuan, Chenghua; Zhang, Can; Ma, Longbing; Yao, Qingyu; Cheng, Lei; Liu, Zhenlei; Wang, Kai; Duan, Wanru; Wang, Xingwen; Wang, Zuowei; Wu, Hao; Chen, Zan; Jian, Fengzeng

doi:10.1007/s00586-021-06746-y

Cited by 16 publications

(15 citation statements)

References 27 publications

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“…The most common clinical manifestation of syringomyelia is dilatation of the central canal of the spinal cord. It is often associated with Chiari malformation (CM), basilar invagination or atlantoaxial dislocation, arachnoid adhesion, other cerebrospinal fluid circulation disorders (1)(2)(3)(4)(5)(6), and other spinal deformities. Thus, it is a type of chronic central spinal cord injury (SCI) (7).…”

Section: Introductionmentioning

confidence: 99%

Spinal Obstruction-Related vs. Craniocervical Junction-Related Syringomyelia: A Comparative Study

Yuan

Guan

et al. 2022

Front. Neurol.

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BackgroundNo prior reports have focused on spinal cord injury (SCI) characteristics or inflammation after destruction of the blood–spinal cord barrier by syringomyelia. This study aimed to determine the differences in syringomyelia-related central SCI between craniocervical junction (CCJ) syringomyelia and post-traumatic syringomyelia (PTS) before and after decompression.MethodsIn all, 106 CCJ, 26 CCJ revision and 15 PTS patients (mean history of symptoms, 71.5 ± 94.3, 88.9 ± 85.5, and 32.3 ± 48.9 months) between 2015 and 2019 were included. The symptom course was analyzed with the American Spinal Injury Association ASIA and Klekamp–Samii scoring systems, and neurological changes were analyzed by the Kaplan–Meier statistics. The mean follow-up was 20.7 ± 6.2, 21.7 ± 8.8, and 34.8 ± 19.4 months.ResultsThe interval after injury was longer in the PTS group, but the natural history of syringomyelia was shorter (p = 0.0004 and 0.0173, respectively). The initial symptom was usually paraesthesia (p = 0.258), and the other main symptoms were hypoesthesia (p = 0.006) and abnormal muscle strength (p = 0.004), gait (p < 0.0001), and urination (p < 0.0001). SCI associated with PTS was more severe than that associated with the CCJ (p = 0.003). The cavities in the PTS group were primarily located at the thoracolumbar level, while those in the CCJ group were located at the cervical-thoracic segment at the CCJ. The syrinx/cord ratio of the PTS group was more than 75% (p = 0.009), and the intradural adhesions tended to be more severe (p < 0.0001). However, there were no significant differences in long-term clinical efficacy or peripheral blood inflammation markers (PBIMs) except for the red blood cell (RBC) count (p = 0.042).ConclusionPTS tends to progress faster than CCJ-related syringomyelia. Except for the RBC count, PBIMs showed no value in distinguishing the two forms of syringomyelia. The predictive value of the neutrophil-to-lymphocyte ratio for syringomyelia-related inflammation was negative except in the acute phase.

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Section: Introductionmentioning

confidence: 99%

Spinal Obstruction-Related vs. Craniocervical Junction-Related Syringomyelia: A Comparative Study

Yuan

Guan

et al. 2022

Front. Neurol.

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“…The indications for secondary surgery included: (1) new, recurrent, or progressive neurological symptoms (independent of syrinx size), (2) large or swollen syringomyelia with obvious spinal cord compression ( 10 ), (3) progression of syringomyelia over time despite no or few symptoms, or other obvious pathology. Patients were excluded if they had a history of shunt placement, infection, multiple decompression, or incomplete follow-up data.…”

Section: Methodsmentioning

confidence: 99%

“…Patients were categorized as Group I if insufficient decompression ( Figure 2 ) was achieved. Clinical symptoms were attributed to the brainstem, cerebellum, and lower cranial nerves, or if the upper segment of the syrinx was at the foramen magnum level ( 10 , 12 ). “Good decompression” was defined as effective spinal cord decompression where the original obstruction to CSF flow was caused by the ectopic cerebellar tonsil, as observed on MR images, and reappearance of the subarachnoid cisterns ( Figure 2 ) of the posterior fossa between the brainstem and cerebellar tonsil.…”

Section: Methodsmentioning

confidence: 99%

Neurological deterioration after posterior fossa decompression for adult syringomyelia: Proposal for a summarized treatment algorithm

Yuan

Guan

et al. 2022

Front. Surg.

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“…According to Yamazaki 55 and Marino 36 , syrinx formation is related to the blocking of the CSF pathway at the CVJ in CMIs. However, the serious blockage of CSF flow at the CVJ due to severe compression might inversely affect syrinx development, thereby misleading neurosurgeons to underestimate brainstem compressions 56 .…”

Section: Relationship Between the Cvjcsi And Syrinxmentioning

confidence: 99%

A Novel Craniocervical Junction Compression Severity Index-Based Grading System for Multidirectional Quantification of the Biomechanics at Foramen Magnum of Chiari Malformation Type I

Liu

Zhang

et al. 2022

J Neurol Surg B Skull Base

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Objective This study aimed to establish a novel grading system, based on the craniovertebral junction compression severity index (CVJCSI) for multidirectional quantification at the foramen magnum plane for Chiari malformation type I (CMI). Methods The CVJCSI grading system was established to stratify patients based on the ventral (modified clivoaxial angle < 138°), dorsal (tonsil herniation), and central (brainstem herniation) CVJ compression, the CVJCSI grading system was established to stratify patients. The optimal surgical method for each grade was recommended by intragroup comparisons regarding the efficacy of the three operations. Finally, according to the CVJCSI grading system, a prospective validation trial was performed and surgically treated for internal validation. Results Based on the retrospective study (n = 310), the CVJCSI included six grades: I- syrinx alone without compression; II- dorsal compression; III- dorsal, and central compression; IV- ventral compression; V- dorsal, and ventral compression; and VI– ventral, dorsal, and central compression. Among all available variables, only the CVJCSI and surgical methods significantly affected the CCOS. The CCOS scores, overall and for each CVJCSI grade, in the prospective cohort (n = 42) than in the retrospective analysis. Conclusions The CVJCSI can be used to stratify CMI patients. The higher the CVJCSI grade, the more severer the CVJ compression and the worse posterior fossa deformity. Meanwhile, the CVJCSI was negatively correlated with the CCOS. The lower the CVJCSI grade, the better the response to surgery, and the less-invasive surgical procedure were warranted. Finally, the prospective cohort study validated the proposed CVJCSI-based surgical protocols.

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A novel classification and its clinical significance in Chiari I malformation with syringomyelia based on high-resolution MRI

Cited by 16 publications

References 27 publications

Spinal Obstruction-Related vs. Craniocervical Junction-Related Syringomyelia: A Comparative Study

Spinal Obstruction-Related vs. Craniocervical Junction-Related Syringomyelia: A Comparative Study

Neurological deterioration after posterior fossa decompression for adult syringomyelia: Proposal for a summarized treatment algorithm

A Novel Craniocervical Junction Compression Severity Index-Based Grading System for Multidirectional Quantification of the Biomechanics at Foramen Magnum of Chiari Malformation Type I

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