Changes in temporal flow characteristics of CSF in Chiari malformation Type I with and without syringomyelia: implications for theory of syrinx development

Object The pathogenesis of syringomyelia in association with Chiari malformation Type I (CM-I) is unclear. Studies of patients with CM-I have shown alterations in the CSF velocity profile using cardiac-gated cine phase-contrast MRI, and computational simulations have demonstrated that temporal features of the CSF pulse could contribute to syrinx development or enlargement. Few studies have reported temporal characteristics of the CSF profile, and few studies have reported on CM-I patients with and without syringomyelia separately. This study was performed to determine whether specific temporal features of the CSF flow profile may underlie the development or enlargement of a syrinx in patients with CM-I. Methods Ten healthy volunteers and 18 patients with CM-I with (8 patients) and without (10 patients) syringomyelia were studied using cardiac-gated cine phase-contrast MRI, measuring the maximum CSF velocities in the cranial and caudal directions, the timing of these maximums relative to the cardiac cycle time, the timing of caudal flow onset, timing of cranial flow onset, and the duration of caudal flow. Results The caudal CSF flow was significantly faster (p ≤ 0.01) and earlier (p < 0.02) in patients without syringomyelia than in healthy volunteers and patients with syringomyelia. There were no significant differences in the CSF velocities between patients with syringomyelia and healthy volunteers. Patients with CM-I who had syringomyelia had a significantly later start of caudal CSF flow (p < 0.01) and earlier maximum cranial velocity (p = 0.03) than healthy volunteers, but the relative durations of caudal and cranial flow were not significantly different between any of the groups. Conclusions The significantly earlier arrival and earlier peak velocity of caudal CSF flow may underlie the development of syringomyelia in patients with CM-I, and after a syrinx develops the CSF flow profile appears to stabilize.

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Standard and cardiac-gated phase-contrast magnetic resonance imaging in the clinical course of patients with Chiari malformation Type I

Neurosurgical FOCUS ◽

10.3171/2011.7.focus11105 ◽

2011 ◽

Vol 31 (3) ◽

pp. E5 ◽

Cited By ~ 19

Author(s):

Uwe Max Mauer ◽

Andreas Gottschalk ◽

Carolin Mueller ◽

Linda Weselek ◽

Ulrich Kunz ◽

...

Keyword(s):

Mr Imaging ◽

Chiari Malformation ◽

Phase Contrast ◽

Foramen Magnum ◽

Central Canal ◽

Type I ◽

Csf Flow ◽

Chiari Malformation Type I ◽

Cerebrospinal Fluid Flow ◽

Contrast Magnetic Resonance

Object The causal treatment of Chiari malformation Type I (CM-I) consists of removing the obstruction of CSF flow at the level of the foramen magnum. Cerebrospinal fluid flow can be visualized using dynamic phase-contrast MR imaging. Because there is only a paucity of studies evaluating CSF dynamics in the region of the spinal canal on the basis of preoperative and postoperative measurements, the authors investigated the clinical usefulness of cardiacgated phase-contrast MR imaging in patients with CM-I. Methods Ninety patients with CM-I underwent preoperative MR imaging of CSF pulsation. Syringomyelia was present in 59 patients and absent in 31 patients. Phase-contrast MR imaging of the entire CNS was used to investigate 22 patients with CM-I before surgery and after a mean postoperative period of 12 months (median 12 months, range 3–33 months). In addition to the dynamic studies, absolute flow velocities, the extension of the syrinx, and tonsillar descent were also measured. Results The changes in pulsation were highly significant in the region of the (enlarged) cistern (p = 0.0005). Maximum and minimum velocities (the pulsation amplitude) increased considerably in the region where the syrinx was largest in diameter. The changes of pulsation in these patients were significant in the subarachnoid space in all spinal segments but not in the syrinx itself and in the central canal. Conclusions The demonstration of CSF flow pulsation can contribute to assessments of surgical outcomes. The results presented here, however, raise doubts about current theories on the pathogenesis of syringomyelia.

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Multimodal evaluation of CSF dynamics following extradural decompression for Chiari malformation Type I

Journal of Neurosurgery Spine ◽

10.3171/2014.10.spine1433 ◽

2015 ◽

Vol 22 (6) ◽

pp. 622-630 ◽

Cited By ~ 10

Author(s):

Jennifer L. Quon ◽

Ryan A. Grant ◽

Michael L. DiLuna

Keyword(s):

Chiari Malformation ◽

Phase Contrast ◽

Clinical Symptoms ◽

Type I ◽

Csf Flow ◽

Tonsillar Herniation ◽

Csf Dynamics ◽

Chiari Malformation Type I ◽

Cisterna Magna ◽

Flow Mri

OBJECT Extradural decompression is a minimally invasive technique for treating Chiari malformation Type I (CM-I) that avoids the complications of dural opening. While there is no agreement on which surgical method is optimal, mounting evidence demonstrates that extradural decompression effectively treats clinical symptoms, with a minimal reoperation rate. Neurological symptoms such as headache may be related to obstructed flow of CSF, and one aspect of successful extradural decompression is improved CSF dynamics. In this series, the authors report on their use of phase-contrast cine flow MRI to assess CSF flow as well as satisfactory decompression. METHODS The authors describe their first surgical series of 18 patients with CM-I undergoing extradural decompression and correlate clinical improvement with radiological changes. Patients were categorized as having complete, partial, or no resolution of their symptoms. Posterior fossa area, cisterna magna area, and tonsillar herniation were assessed on T2-weighted MRI, whereas improvement of CSF flow was evaluated with phase-contrast cine flow MRI. All patients received standard pre- and postoperative MRI studies; 8 (44.4%) patients had pre- and postoperative phase-contrast cine, while the rest underwent cine studies only postoperatively. RESULTS All 18 patients presented with symptomatic CM-I, with imaging studies demonstrating tonsillar herniation ≥ 5 mm, and 2 patients had associated syringomelia. All patients underwent suboccipital decompression and C-1 laminectomy with splitting of the dura. Patients with complete resolution of their symptoms had a greater relative increase in cisterna magna area compared with those with only partial improvement (p = 0.022). In addition, in those with complete improvement the preoperative cisterna magna area was smaller than in those who had either partial (0.020) or no (0.025) improvement. Ten (91%) of the 11 patients with improved flow also had improvement in their symptoms. There was 1 postoperative complication of dysphagia and dysphonia. None of the patients have required a second operation. CONCLUSIONS Extradural decompression has the potential to be the first-line treatment for CM-I but has been lacking an objective measure by which to assess surgical success as well as the need for reoperation. An increase in the CSF spaces and improved CSF dynamics may be associated with resolution of clinical symptoms. Including cine imaging as part of routine pre- and postoperative evaluation can help identify which patients are most likely to benefit from surgery.

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Cine phase-contrast MRI measurement of CSF flow in the cervical spine: a pilot study in patients with spinal cord injury

10.1117/12.881715 ◽

2011 ◽

Cited By ~ 4

Author(s):

MJ Negahdar ◽

M. Shakeri ◽

E. McDowell ◽

J. Wells ◽

T. Vitaz ◽

...

Keyword(s):

Spinal Cord ◽

Spinal Cord Injury ◽

Cervical Spine ◽

Pilot Study ◽

Phase Contrast ◽

Csf Flow ◽

Phase Contrast Mri ◽

Cord Injury ◽

Cine Phase Contrast

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CSF Flow Improvement after Chiari Malformation Type I Surgery as a Prognostic Indicator for Clinical Improvement

10.1055/s-0040-1702528 ◽

2020 ◽

Author(s):

Luke Mugge ◽

Camila Dassi ◽

Thiago Albonette Felicio ◽

Rafael Martinez-Perez ◽

Giuliano Schmidt Bertazzo Silveira ◽

...

Keyword(s):

Clinical Improvement ◽

Chiari Malformation ◽

Prognostic Indicator ◽

Type I ◽

Csf Flow ◽

Chiari Malformation Type I ◽

Flow Improvement

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Cine phase-contrast MRI evaluation of normal aqueductal csf flow according to sex and age

Diagnostic and Interventional Radiology ◽

10.4261/1305-3825.dir.2321-08.1 ◽

2009 ◽

Cited By ~ 1

Author(s):

Ozkan Unal ◽

Alp Kartum ◽

Serhat Avcu ◽

Omer Etlik ◽

Halil Arslan ◽

...

Keyword(s):

Phase Contrast ◽

Csf Flow ◽

Phase Contrast Mri ◽

Cine Phase Contrast ◽

Mri Evaluation

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Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.5.peds1121 ◽

2011 ◽

Vol 8 (2) ◽

pp. 205-213 ◽

Cited By ~ 111

Author(s):

Jennifer Strahle ◽

Karin M. Muraszko ◽

Joseph Kapurch ◽

J. Rajiv Bapuraj ◽

Hugh J. L. Garton ◽

...

Keyword(s):

Cervical Spine ◽

Mr Imaging ◽

Chiari Malformation ◽

Foramen Magnum ◽

Type I ◽

Csf Flow ◽

Tonsillar Herniation ◽

Chiari Malformation Type I ◽

Age Related ◽

Imaging Characteristics

Object Chiari malformation Type I (CM-I) with an associated spinal syrinx is a common pediatric diagnosis. A better understanding of the relative age-related prevalence and MR imaging characteristics of these associated conditions may lead to improved treatment decisions. Methods The authors performed a retrospective review of 14,116 consecutive individuals 18 years of age or younger who had undergone brain or cervical spine MR imaging at the University of Michigan between November 1997 and August 2008. In the patients with CM-I, demographic, clinical, and radiographic information was recorded. Results Five hundred nine children (3.6%) with CM-I were identified. Among these patients, 23% also had a spinal cord syrinx, and 86% of the syringes were found in the cervical spine. The MR imaging prevalence of CM-I with a syrinx was 1.2% in girls and 0.5% in boys (p < 0.0001). The severity of impaired CSF flow at the foramen magnum was associated with the amount of tonsillar herniation (p < 0.0001) and conformation of the tonsils (p < 0.0001). Patients with CM-I were treated surgically in 35% of cases; these patients exhibited more severe tonsillar herniation (p < 0.0001) and impaired CSF flow (p < 0.0001) as compared with those who did not undergo surgery. On imaging, 32% of all the patients with CM-I were considered symptomatic by the treating physician. Patients were more likely to be considered symptomatic if they were female, had a syrinx, displayed abnormal tonsillar pulsations, or had a greater amount of tonsillar herniation. Conclusions In this study the authors describe the age-related prevalence and MR imaging characteristics of CM-I and its association with a syrinx and other abnormalities in a large group of children who underwent MR imaging for any indication. Syringes are more common in older children, in girls, and in patients with a greater degree of tonsillar descent and CSF flow impairment.

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Resolution of syndromic craniosynostosis-associated Chiari malformation Type I without suboccipital decompression after posterior cranial vault release

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.11.peds11268 ◽

2012 ◽

Vol 9 (2) ◽

pp. 111-115 ◽

Cited By ~ 17

Author(s):

Michael R. Levitt ◽

Toba N. Niazi ◽

Richard A. Hopper ◽

Richard G. Ellenbogen ◽

Jeffrey G. Ojemann

Keyword(s):

Chiari Malformation ◽

Watchful Waiting ◽

Cranial Vault ◽

Type I ◽

Crouzon Syndrome ◽

Csf Flow ◽

Chiari Malformation Type I ◽

Syndromic Craniosynostosis ◽

Suboccipital Decompression

Chiari malformation Type I (CM-I) is associated with syndromic and nonsyndromic craniosynostosis in pediatric patients, and the surgical management of CM-I in such cases is controversial. Previous guidelines have recommended simultaneous cranial vault expansion and suboccipital decompression. However, spontaneous resolution of CM-I has been observed, and the combined procedure carries additional surgical risks. The authors report the case of a 6-month-old boy with Crouzon syndrome, CM-I, and a cervical syrinx who underwent posterior cranial vault release without suboccipital decompression. Imaging at the 3-month follow-up visit demonstrated complete resolution of the CM-I, improvement in CSF flow, and reduction in the size of the syrinx. This case suggests that up-front suboccipital decompression may not be necessary in patients with craniosynostosis and CM-I. A strategy of initial cranial vault release, followed by watchful waiting and radiographic surveillance, is proposed.

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Pediatric Chiari malformation Type 0: a 12-year institutional experience

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.4.peds10528 ◽

2011 ◽

Vol 8 (1) ◽

pp. 1-5 ◽

Cited By ~ 52

Author(s):

Joshua J. Chern ◽

Amber J. Gordon ◽

Martin M. Mortazavi ◽

R. Shane Tubbs ◽

W. Jerry Oakes

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Foramen Magnum ◽

Posterior Fossa Decompression ◽

Type I ◽

Csf Flow ◽

Chiari Malformation Type I ◽

Institutional Experience ◽

Physical Barriers ◽

Small Cohort

Object In 1998 the authors identified 5 patients with syringomyelia and no evidence of Chiari malformation Type I (CM-I). Magnetic resonance imaging of the entire neuraxis ruled out other causes of a syrinx. Ultimately, abnormal CSF flow at the foramen magnum was the suspected cause. The label “Chiari 0” was used to categorize these unique cases with no tonsillar ectopia. All of the patients underwent posterior fossa decompression and duraplasty identical to the technique used to treat patients with CM-I. Significant syrinx and symptom resolution occurred in these patients. Herein, the authors report on a follow-up study of patients with CM-0 who were derived from over 400 operative cases of pediatric CM-I decompression. Methods The authors present their 12-year experience with this group of patients. Results Fifteen patients (3.7%) were identified. At surgery, many were found to have physical barriers to CSF flow near the foramen magnum. In most of them, the syringomyelia was greatly diminished postoperatively. Conclusions The authors stress that this subgroup represents a very small cohort among patients with Chiari malformations. They emphasize that careful patient selection is critical when diagnosing CM-0. Without an obvious CM-I, other etiologies of a spinal syrinx must be conclusively ruled out. Only then can one reasonably expect to ameliorate the clinical course of these patients via posterior fossa decompression.

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