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.
This publication is endorsed by: European Society of Neuroradiology (www.esnr.org)
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.
Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. 2006;29(4):251–64. https://doi.org/10.1007/s10143-006-0029-5.
Hughes JA, De Bruyn R, Patel K, Thompson D. Evaluation of spinal ultrasound in spinal dysraphism. Clin Radiol. 2003;58(3):227–33.
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.
Milhorat TH. Classification of syringomyelia. Neurosurg Focus. 2000;8(3):Article 1.
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.
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.
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.
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.
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.
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.
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.
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.
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
Wykes V, Desai D, Thompson DN. Asymptomatic lumbosacral lipomas – a natural history study. Childs Nerv Syst. 2012;28(10):1731–9.
Suggested Readings
Flint G, Rusbridge C. Syringomyelia: a disorder of CSF circulation. Berlin/Heidelberg: Springer Berlin Heidelberg . Imprint: Springer; 2014.
Kornienko VN, Pronin IN. Spine and spinal cord disorders: syringohydromyelia. Chapter 15: 15.5.6. In: Diagnostic neuroradiology. Berlin/Heidelberg: Springer; 2009. p. 1150–5. https://doi.org/10.1007/978-3-540-75653-8P.
Nogues MA. Syringomyelia. 2001. Last updated 16 Aug 2017. https://medicinainternaelsalvador.com/wp-content/uploads/2017/08/syringomyelia_20622.pdf
Tortori-Donati P, Rossi A. Hydromyelia and syringomyelia. Ch 39.7. In: Pediatric neuroradiology. Berlin/Heidelberg: Springer; 2005. p. 1603–6.
Vandertop WP. Syringomyelia. Neuropediatrics. 2014;45(1):3–9.
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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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