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

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A Correction to this article was published on 10 April 2021

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Abstract

Background

The existing classification in Chiari I malformation (CM-I) has limited significance for the selection of surgical methods.

Objective

The purpose of this study was to investigate the surgery of CM-I with syringomyelia based on the high-resolution MR imaging (HRMRI) findings.

Methods

Data from 115 patients were collected and retrospectively analyzed. For those with syringomyelia up to the level of C1, HRMRI was performed and according to the communication status between the fourth ventricle and the syringomyelia, patients can be divided into four types, namely Type A: classic communicating; Type B: partial communicating; Type C: non-communicating; Type D: atrophic. All operations were performed with Foramen magnum and Magendie dredging (FMMD), and all intradural factors that may have induced the obstruction of CSF circulation were recorded. The efficiency of operation on syringomyelia was evaluated by mJOA, imaging findings, and complications in the follow-up periods.

Results

The postoperative follow-up period was from 12 to 24 months, with an average of 14.3 months. At 1 year, the mJOA of 115 patients was significantly higher than that before the operations (before surgery 12.1 ± 2.3 vs. after surgery 14. 2 ± 1.4, P < 0.05). In addition, postoperative re-examination showed that the size of the syringomyelia was reduced or completely resolved in patients of Type A, 100% (2/2); Type B, 81% (9/11); Type C, 84% (81/97); and Type D, 20% (1/5).

Conclusions

According to our new classification based on HRMRI, FMMD is the key to surgical treatment, especially for Type A and Type B patients.

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References

  1. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg 92:920–926. https://doi.org/10.3171/jns.2000.92.6.0920

    Article  CAS  PubMed  Google Scholar 

  2. Chiari H (1987) Concerning alterations in the cerebellum resulting from cerebral hydrocephalus. 1891. Pediatr Neurosci 13:3–8. https://doi.org/10.1159/000120293

    Article  CAS  PubMed  Google Scholar 

  3. Chern JJ, Gordon AJ, Mortazavi MM, Tubbs RS, Oakes WJ (2011) Pediatric Chiari malformation Type 0: a 12-year institutional experience. J Neurosurg Pediatr 8:1–5. https://doi.org/10.3171/2011.4.PEDS10528

    Article  PubMed  Google Scholar 

  4. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15. https://doi.org/10.3171/jns.1994.80.1.0003

    Article  CAS  PubMed  Google Scholar 

  5. Heiss JD, Jarvis K, Smith RK, Eskioglu E, Gierthmuehlen M, Patronas NJ, Butman JA, Argersinger DP, Lonser RR, Oldfield EH (2019) Origin of syrinx fluid in syringomyelia: a physiological study. Neurosurgery 84:457–468. https://doi.org/10.1093/neuros/nyy072

    Article  PubMed  Google Scholar 

  6. Gardner WJ, Goodall RJ (1950) The surgical treatment of Arnold-Chiari malformation in adults; an explanation of its mechanism and importance of encephalography in diagnosis. J Neurosurg 7:199–206. https://doi.org/10.3171/jns.1950.7.3.0199

    Article  CAS  PubMed  Google Scholar 

  7. Williams B (1980) On the pathogenesis of syringomyelia: a review. J R Soc Med 73:798–806

    Article  CAS  Google Scholar 

  8. Talacchi A, Meneghelli P, Borghesi I, Locatelli F (2016) Surgical management of syringomyelia unrelated to Chiari malformation or spinal cord injury. Eur Spine J 25:1836–1846. https://doi.org/10.1007/s00586-015-4262-x

    Article  PubMed  Google Scholar 

  9. Dlouhy BJ, Dawson JD, Menezes AH (2017) Intradural pathology and pathophysiology associated with Chiari I malformation in children and adults with and without syringomyelia. J Neurosurg Pediatr 20:526–541. https://doi.org/10.3171/2017.7.PEDS17224

    Article  PubMed  Google Scholar 

  10. Muraoka S, Araki Y, Taoka T, Kawai H, Okamoto S, Uda K, Ota S, Naganawa S, Wakabayashi T (2018) Prediction of intracranial arterial stenosis progression in patients with moyamoya vasculopathy: contrast-enhanced high-resolution magnetic resonance vessel wall imaging. World Neurosurg 116:e1114–e1121. https://doi.org/10.1016/j.wneu.2018.05.181

    Article  PubMed  Google Scholar 

  11. Guan J, Yuan C, Zhang C, Ma L, Yao Q, Cheng L, Liu Z, Wang K, Duan W, Wang X, Wu H, Chen Z, Jian F (2020) Intradural pathology causing cerebrospinal fluid obstruction in syringomyelia and effectiveness of Foramen magnum and Foramen of magendie dredging treatment. World Neurosurg. https://doi.org/10.1016/j.wneu.2020.08.068

    Article  PubMed  PubMed Central  Google Scholar 

  12. Yuan C, Yao Q, Zhang C, Jian F (2019) Spontaneous resolution of syringomyelia with a 16-year serial magnetic resonance imaging follow-up: a case report and literature review. World Neurosurg 130:432–438. https://doi.org/10.1016/j.wneu.2019.07.138

    Article  PubMed  Google Scholar 

  13. Yuan C, Guan J, Jian F (2020) Rapid progression of acute cervical syringomyelia: a case report of delayed complications following spinal cord injury. J Spinal Cord Med. https://doi.org/10.1080/10790268.2020.1733336

    Article  PubMed  Google Scholar 

  14. Gardner WJ, Angel J (1958) The cause of syringomyelia and its surgical treatment. Clevel Clin Q 25:4–8. https://doi.org/10.3949/ccjm.25.1.4

    Article  CAS  Google Scholar 

  15. Ma L, Yao Q, Zhang C, Li M, Cheng L, Jian F (2020) Chronic extradural compression of spinal cord leads to syringomyelia in rat model. Fluids Barriers CNS 17:50. https://doi.org/10.1186/s12987-020-00213-4

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Milhorat TH, Johnson WD, Miller JI, Bergland RM, Hollenberg-Sher J (1992) Surgical treatment of syringomyelia based on magnetic resonance imaging criteria. Neurosurgery 31:231–244 ((discussion 244–235))

    Article  CAS  Google Scholar 

  17. Giner J, Perez Lopez C, Hernandez B, Gomez de la Riva A, Isla A, Roda JM (2019) Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation. Neurologia 34:318–325. https://doi.org/10.1016/j.nrl.2016.09.010

    Article  CAS  PubMed  Google Scholar 

  18. Davidoff CL, Liu S, Wong JHY, Koustais S, Rogers JM, Stoodley MA (2017) Treatment of syringomyelia in patients with arachnoiditis at the craniocervical junction. World Neurosurg 107:565–573. https://doi.org/10.1016/j.wneu.2017.08.064

    Article  PubMed  Google Scholar 

  19. Fan T, Zhao H, Zhao X, Liang C, Wang Y, Gai Q (2017) Surgical management of Chiari I malformation based on different cerebrospinal fluid flow patterns at the cranial-vertebral junction. Neurosurg Rev 40:663–670. https://doi.org/10.1007/s10143-017-0824-1

    Article  PubMed  Google Scholar 

  20. Shamji MF, Ventureyra EC, Baronia B, Nzau M, Vassilyadi M (2010) Classification of symptomatic Chiari I malformation to guide surgical strategy. Can J Neurol Sci 37:482–487. https://doi.org/10.1017/s0317167100010507

    Article  PubMed  Google Scholar 

  21. Oldfield EH (2017) Pathogenesis of Chiari I—pathophysiology of syringomyelia: implications for therapy: a summary of 3 decades of clinical research. Neurosurgery 64:66–77. https://doi.org/10.1093/neuros/nyx377

    Article  PubMed  Google Scholar 

  22. Chang HS, Nagai A, Oya S, Matsui T (2014) Dorsal spinal arachnoid web diagnosed with the quantitative measurement of cerebrospinal fluid flow on magnetic resonance imaging. J Neurosurg Spine 20:227–233. https://doi.org/10.3171/2013.10.SPINE13395

    Article  PubMed  Google Scholar 

  23. Ya J, Zhou D, Ding J, Ding Y, Ji X, Yang Q, Meng R (2020) High-resolution combined arterial spin labeling MR for identifying cerebral arterial stenosis induced by moyamoya disease or atherosclerosis. Ann Transl Med 8:87. https://doi.org/10.21037/atm.2019.12.140

    Article  PubMed  PubMed Central  Google Scholar 

  24. Tubbs RS, Smyth MD, Wellons JC 3rd, Oakes WJ (2004) Arachnoid veils and the Chiari I malformation. J Neurosurg 100:465–467. https://doi.org/10.3171/ped.2004.100.5.0465

    Article  PubMed  Google Scholar 

  25. Ciappetta P, Signorelli F, Visocchi M (2019) The role of arachnoid veils in chiari malformation associated with syringomyelia. Acta Neurochir Suppl 125:97–99. https://doi.org/10.1007/978-3-319-62515-7_14

    Article  PubMed  Google Scholar 

  26. Giammattei L, Borsotti F, Parker F, Messerer M (2018) Chiari I malformation: surgical technique, indications and limits. Acta Neurochir (Wien) 160:213–217. https://doi.org/10.1007/s00701-017-3380-0

    Article  Google Scholar 

  27. Rehman L, Akbar H, Bokhari I, Babar AK, Hashin ASM, Arain SH (2015) Posterior fossa decompression with duraplasty in Chiari-1 malformations. J Coll Physicians Surg Pak 25:254–258

    PubMed  Google Scholar 

  28. Yuan C, Guan J, Du Y, Zhang C, Ma L, Yao Q, Cheng L, Liu Z, Wang K, Duan W, Wang X, Wu H, Chen Z, Jian F (2020) Repeat craniocervical decompression in patients with a persistent or worsening syrinx: a preliminary report and early results. World Neurosurg 138:e95–e105. https://doi.org/10.1016/j.wneu.2020.02.015

    Article  PubMed  Google Scholar 

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Acknowledgements

We thank Xu shenglong for English language editing.

Funding

This study was supported by “Yang fan” project of Beijing medical management center in 2021 (XMLX202138), Beijing Municipal Health Commission (Beijing Health Commission Independent Innovation Fund 2018-2-2014), Beijing Municipal Administration of Hospitals (Beijing Natural Science Foundation Grant, 7172091), Beijing Municipal Science and Technology Commission Grant numbers: Z191199996619048, and Beijing Municipal Commission of Education Grant numbers: KZ202010025043, 1192070315.

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Correspondence to Zan Chen or Fengzeng Jian.

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The study was approved by the institutional research ethics committee of Xuanwu Hospital.

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Guan, J., Yuan, C., Zhang, C. et al. A novel classification and its clinical significance in Chiari I malformation with syringomyelia based on high-resolution MRI. Eur Spine J 30, 1623–1634 (2021). https://doi.org/10.1007/s00586-021-06746-y

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