Spontaneous Resolution of Syringomyelia with a 16-Year Serial Magnetic Resonance Imaging Follow-Up: A Case Report and Literature Review

Yuan, Chenghua; Yao, Qingyu; Zhang, Can; Jian, Fengzeng

doi:10.1016/j.wneu.2019.07.138

Cited by 20 publications

(27 citation statements)

References 29 publications

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“…6 Twenty cases of spontaneous resolution of a syrinx in patients with CM1 have been previously described. [20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] In about half of them, an association with improvement of the tonsillar herniation is described, similar to our case. Recurrence after spontaneous resolution has been described in only 1 other case.…”

Section: Discussion Observationssupporting

confidence: 81%

“…An improvement of CSF flow at the foramen magnum due to spontaneous rupture of the arachnoid adhesions, typically encountered at the foramen magnum, has been suggested by 4 different authors. 25,27,33,35 Others have reported spontaneous improvement of CM1 due to brain atrophy in ageing patients. 37,38 Lifestyle changes, such as avoiding straining and breath holding and ceasing hard physical labor, have been described, leading to a resolution of syringomyelia due to reduced venous pressure.…”

Section: Discussion Observationsmentioning

confidence: 99%

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

Veken¹,

Harding²,

Hatami

et al. 2021

Journal of Neurosurgery: Case Lessons

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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.

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Section: Discussion Observationssupporting

confidence: 81%

Section: Discussion Observationsmentioning

confidence: 99%

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

Veken¹,

Harding²,

Hatami

et al. 2021

Journal of Neurosurgery: Case Lessons

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“…Spinal obstruction-related syringomyelia is similar to the compression syringomyelia model 13 . The long-term natural history of syringomyelia remains unclear 7,17 . The history of syringomyelia related to the craniocervical junction, especially in the revision group, was signi cantly longer than that in the PTS group.…”

Section: Discussionmentioning

confidence: 99%

Spinal obstruction-related versus craniocervical junction related syringomyelia: a comparative study of spinal cord injury

Yuan

Guan

et al. 2021

Preprint

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Background No prior reports have focused on spinal cord injury (SCI) characteristics or inflammation after destruction of the blood-spinal cord barrier by syringomyelia. To compare the difference of syringomyelia-related central SCI between craniocervical junction (CCJ) and post-traumatic syringomyelia (PTS) before and after decompression. Methods Between 2015 and 2019, 106 CCJ, 26 CCJ revision and 15 PTS patients (mean history of symptoms 71.5 ± 94.3, 88.9 ± 85.5 and 32.3 ± 48.9 months). The symptom courses were analysed with the ASIA, Klekamp and Samii scoring systems and Kaplan-Meier statistics for neurological changes. The mean follow-up was 20.7 ± 6.2, 21.7 ± 8.8 and 34.8 ± 19.4 months. Results Compared with the other group, the interval time after PTS was longer, but the natural history of syringomyelia was shorter (P=0.0004, 0.0173, respectively). The initial symptoms were usually paraesthesia (P=0.258), and the symptoms were mainly hypoesthesia (P=0.006), abnormal muscle strength (P=0.004), gait (P<0.0001) and abnormal urination (P<0.0001). SCI associated with PTS was more severe than that CCJ related (P=0.003). The cavities in the PTS group were primarily located at the thoracolumbar level, which was different from those in the cervical-thoracic segment at the CCJ. The rate of syrinx/cord was more than 75% (P=0.009), and the intradural adhesions tended to be more severe (P<0.0001). However, there were no significant differences in peripheral blood inflammation markers (PBIM) or long-term clinical efficacy except for the RBC (P=0.042). Conclusion The natural history of PTS tends to progress faster and is more severe than CCJ related. PBIM had no distinguishing effect on the difference in inflammation of syringomyelia except for the RBC. The predictive value of NLR for syringomyelia-related inflammation except in the acute phase was negative.

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“…Although surgical intervention is the widely accepted treatment modality, documented cases of spontaneous resolution of a syrinx associated with a Chiari malformation make the current treatment strategy controversial. [18][19][20][21][22][23][24][25][26] Some authors have demonstrated improvement or resolution of the Chiari I malformation itself, which leads to syringomyelia improvement. 20,21,23 To our knowledge, few documented cases exist of patients with spontaneous resolution of a syrinx associated with an unchanged Chiari malformation.…”

Section: Lessonsmentioning

confidence: 99%

“…20,21,23 To our knowledge, few documented cases exist of patients with spontaneous resolution of a syrinx associated with an unchanged Chiari malformation. 18,19,22,25,26 One explanation for spontaneous resolution in adults with an unchanged Chiari malformation involves the formation of a communication between the syringomyelia and the subarachnoid space through a tear caused by increased pressure from Valsalva maneuvers. 19,21,25,27 These types of communications have been demonstrated by MRI and neuropathological examinations.…”

Section: Lessonsmentioning

confidence: 99%

Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case

Gallo¹,

Rahmathulla²,

Rao

et al. 2021

Journal of Neurosurgery: Case Lessons

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BACKGROUIND Chiari malformations include a spectrum of congenital hindbrain herniation syndromes. In patients with the most common subtype, Chiari malformation Type I, 50% to 75% develop a syrinx. The pathogenesis of syringomyelia is not well understood, with multiple theories outlined in the literature. Although the presence of a syrinx in a patient with Chiari malformation is generally accepted as an indication for surgical intervention, there are documented cases of spontaneous resolution that support a more conservative approach to management. OBSERVATIONS The authors reported a case of spontaneous resolution of a cervical syrinx in an adult with an unchanged Chiari malformation. LESSONS Given the possibility of spontaneous resolution over time, the authors believe a more conservative approach of observation with periodic surveillance, magnetic resonance imaging, and neurological examination should be considered in the management of a patient with a Chiari malformation and associated syringomyelia.

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Spontaneous Resolution of Syringomyelia with a 16-Year Serial Magnetic Resonance Imaging Follow-Up: A Case Report and Literature Review

Cited by 20 publications

References 29 publications

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

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

Spinal obstruction-related versus craniocervical junction related syringomyelia: a comparative study of spinal cord injury

Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case

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