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Case Reports
. 2021 Sep 13;2(11):CASE21341.
doi: 10.3171/CASE21341.

Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case

Jorn Van Der Veken  1 , Marguerite Harding  1 , Saba Hatami  2   3 , Marc Agzarian  2   3 , Nick Vrodos  1
Affiliations
Case Reports

Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case

Jorn Van Der Veken et al. J Neurosurg Case Lessons. .

Abstract

Background: Chiari Type I malformation (CM1) is a disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Syringomyelia is frequently found in patients with CM1, but the pathophysiology of syringomyelia remains an enigma. As a general consensus, symptomatic patients should be treated and asymptomatic patients without a syrinx should not be treated. Mildly symptomatic patients or asymptomatic patients with a syrinx, on the other hand, pose a more challenging dilemma, as the natural evolution is uncertain. For many surgeons, the presence of a syrinx is an indication to offer surgery even if the patient is asymptomatic or mildly symptomatic.

Observations: The authors describe an illustrative case of a 31-year-old female with an incidental finding of a CM1 malformation and cervical syrinx in 2013. Conservative management was advocated as the patient was asymptomatic. Monitoring of the syrinx over a course of 8 years showed resolution, followed by reappearance and finally a complete resolution in 2021. A review of the literature and the possible pathophysiology is discussed.

Lessons: The unusual course of this patient highlights the importance of guiding treatment by clinical symptoms, not radiological findings. Furthermore it reflects the complexity of the pathophysiology and the uncertain natural history of syringomyelia.

Keywords: CM1 = Chiari Type I malformation; CSF = cerebrospinal fluid; CT = computed tomography; Chiari 1; MRI = magnetic resonance imaging; PFD = posterior fossa decompression; PFDD = posterior fossa decompression with duraplasty; SAS = subarachnoid space; intermittent; syringomyelia.

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Conflict of interest statement

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Figures

FIG. 1.
FIG. 1.
Left: Sagittal T2-weighted spin-echo MRI February 2013. Right: Axial T2-weighted spin echo MRI in February 2013.
FIG. 2.
FIG. 2.
Sagittal T2-weighted spin-echo MRI in July 2013.
FIG. 3.
FIG. 3.
Sagittal T2-weighted spin-echo MRI in June 2016.
FIG. 4.
FIG. 4.
Sagittal T2-weighted spin-echo MRI in February 2021, showing disappearance of the syrinx and clear CSF high T2 signal at the foramen magnum and rounder cerebellar tonsils.
See this image and copyright information in PMC

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