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

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.