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Imaging of Spinal CSF Disorders: Syringomyelia

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Clinical Neuroradiology

Abstract

Syringomyelia is a multifactorial disorder comprising fluid-filled cavitations within the spinal cord. In addition to establishing the diagnosis, neuroradiological techniques may discern the etiology and pathophysiology and thus inform treatment. While multiplanar MRI with T1- and T2-weighted sequences is the gold standard for imaging, advanced techniques such as phase-contrast MRI and cine MRI are increasingly used both in diagnosis and follow-up. This chapter provides a practical approach to syringomyelia imaging, its interpretation, and pitfalls.

Syringomyelia is rarely a primary anomaly; beyond demonstrating the size and extent of syringomyelia, imaging techniques should be directed toward identifying an underlying cause. A disturbance to normal craniospinal CSF circulation is the most common predisposition to syringomyelia formation, as, for example, occurs in hydrocephalus or Chiari type I malformation. Local disturbances of spinal CSF circulation may also result in syringomyelia, for example, following spinal cord trauma, infection, or inflammation. Spinal cord neoplasms will often be associated with “cyst” formation resulting in an appearance of syringomyelia.

Syringomyelia should therefore be considered the beginning rather than the end of a diagnostic process.

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Abbreviations

CIM:

Chiari I malformation

CIIM:

Chiari II malformation

C:

Cervical

CSF:

Cerebrospinal fluid

CT:

Computed tomography

L:

Lumbar

MRI:

Magnetic resonance imaging

T:

Thoracic

V-P shunt:

Ventriculoperitoneal shunt

US:

Ultrasonography

References

  • Fischbein NJ, Dillon WP, Cobbs C, Weinstein PR. The “presyrinx” state: a reversible myelopathic condition that may precede syringomyelia. Am J Neuroradiol. 1999;20:7–20.

    CAS  PubMed  Google Scholar 

  • Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. 2006;29(4):251–64. https://doi.org/10.1007/s10143-006-0029-5.

    Article  PubMed  Google Scholar 

  • Hughes JA, De Bruyn R, Patel K, Thompson D. Evaluation of spinal ultrasound in spinal dysraphism. Clin Radiol. 2003;58(3):227–33.

    Article  CAS  Google Scholar 

  • McGirt MJ, Nimjee SM, Fuchs HE, George TM. Relationship of cine phase-contrastmagnetic resonance imaging with outcome after decompression for Chiari I malformations. Neurosurgery. 2006;59(1):140–6. https://doi.org/10.1227/01.NEU.0000219841.73999.B3.

    Article  PubMed  Google Scholar 

  • Milhorat TH. Classification of syringomyelia. Neurosurg Focus. 2000;8(3):Article 1.

    Article  Google Scholar 

  • Oakes J, Thompson D. Paediatric perspectives. In: Flint G, Rusbridge C, editors. Syringomyelia: a disorder of CSF circulation. Berlin/Heidelberg: Springer; 2014. p. 193–207.

    Chapter  Google Scholar 

  • Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg. 1994;80(1):3–15. https://doi.org/10.3171/jns.1994.80.1.0003.

    Article  CAS  PubMed  Google Scholar 

  • Poretti A, Ashmawy R, Garzon-Muvdi T, Jallo GI, Huisman TAGM, Raybaud C. Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 2016;47: 293–307.

    Article  Google Scholar 

  • Rossi A. Hydrosyringomyelia, cysts, and other disorders of the cerebrospinal fluid spaces in the spine. In: Pediatric neuroradiology. Berlin/Heidelberg: Springer; 2015. p. 1–18.

    Google Scholar 

  • Rossi A, Martinetti C, Morana G, Severino M, Tortora D. Diagnostic approach to pediatric spine disorders. Magn Reson Imaging Clin N Am. 2016;24:621–44.

    Article  Google Scholar 

  • Singhal A, Bowen-Roberts T, Steinbok P, Cochrane D, Byrne AT, Kerr JM. Natural history of untreated syringomyelia in pediatric patients. Neurosurg Focus. 2011;31(6):E13. https://doi.org/10.3171/2011.9.FOCUS11208.

    Article  PubMed  Google Scholar 

  • Stoodley MA, Jones NR, Yang L, Brown CJ. Mechanisms underlying the formation and enlargement of noncommunicating syringomyelia: experimental studies. Neurosurg Focus. 2000;8(3):E2.

    CAS  PubMed  Google Scholar 

  • Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJL, Maher CO. Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 2011;8(2):214–21. https://doi.org/10.3171/2011.5.PEDS1122.

    Article  PubMed  Google Scholar 

  • Thompson DNP. Chiari I malformation and associated syringomyelia. In: Di Rocco C, et al., editors. Textbook of pediatric neurosurgery. Springer International Publishing AG; 2017. p. 1–32. https://doi.org/10.1007/978-3-319-31512-6_125-1

    Google Scholar 

  • Wykes V, Desai D, Thompson DN. Asymptomatic lumbosacral lipomas – a natural history study. Childs Nerv Syst. 2012;28(10):1731–9.

    Article  Google Scholar 

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Correspondence to Julija Pavaine .

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Pavaine, J., Thompson, D. (2018). Imaging of Spinal CSF Disorders: Syringomyelia. In: Barkhof, F., Jager, R., Thurnher, M., Rovira Cañellas, A. (eds) Clinical Neuroradiology. Springer, Cham. https://doi.org/10.1007/978-3-319-61423-6_12-1

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  • DOI: https://doi.org/10.1007/978-3-319-61423-6_12-1

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  • Print ISBN: 978-3-319-61423-6

  • Online ISBN: 978-3-319-61423-6

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