Chiari malformation: CSF flow dynamics in the craniocervical junction and syrinx

Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder. It is associated with morphological changes similar to those of other connective tissue disorders, with the unique distinction of multiple, often bilateral and large, lateral meningoceles herniating through the spinal foramina. In some cases, these lateral meningoceles can cause pain and discomfort due to their presence within retroperitoneal tissues or cause direct compression of the spinal nerve root exiting the foramen; in some cases compression may also involve motor weakness. The presence of lateral meningoceles imposes unique challenges related to CSF flow dynamics, especially with concurrent Chiari malformation, which also occurs with increased frequency in individuals with LMS. The authors present the case of a 6-month-old female with LMS with multiple lateral meningoceles throughout the thoracic and lumbar spine. The infant experienced a focal neurological abnormality due to enlargement of her lateral meningoceles following decompression of a symptomatic Chiari malformation and endoscopic third ventriculostomy. The finding was reversed through implantation of a ventriculoperitoneal shunt, which reduced the burden of CSF upon the lateral meningoceles. Such a case compels consideration that CSF flow dynamics in addition to altered connective tissue play a role in the presence of lateral meningoceles in patients within this and similar patient populations.

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Prenatal and postnatal evaluation for syringomyelia in patients with spinal dysraphism

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.5.peds13482 ◽

2014 ◽

Vol 14 (3) ◽

pp. 316-321 ◽

Cited By ~ 10

Author(s):

Ben J. Bixenmann ◽

Beth M. Kline-Fath ◽

Karin S. Bierbrauer ◽

Danesh Bansal

Keyword(s):

Chiari Malformation ◽

Spinal Dysraphism ◽

Tethered Cord ◽

Fetal Mri ◽

Clinical History ◽

Craniocervical Junction ◽

Csf Flow ◽

Third Trimester ◽

Flow Disturbances

Object Syringomyelia can be diagnosed in isolation but is more commonly found in the presence of craniocervical junction anomalies or spinal dysraphism. The origin of syringomyelia has been hypothesized to be either congenital or acquired. The purpose of this study was to determine the incidence of syringomyelia within the fetal and postnatal population with neural tube defects (NTDs). Methods A review was performed of the authors' fetal MRI database of pregnancies with imaging between March 2004 and November 2011 for evaluation of an intrauterine anomaly detected via prenatal ultrasonography. Those cases with an NTD were then selected and a chart review was performed of all prenatal and postnatal imaging as well as available clinical history. Results A total of 2362 fetal MRI examinations were performed, and 109 of these were patients with an NTD. Of the 2362 studies reviewed, 2 cases of fetal syringomyelia were identified. Both fetal syrinxes were identified in fetuses with CSF flow disturbances (1 case each of encephalocele and myelomeningocele). Both fetal MRI examinations were performed late in gestation, at 31 and 38 weeks, respectively. The patient with an encephalocele was excluded from the spinal NTD population; therefore a syrinx was identified in 0.08% (2/2362) of the entire population of fetuses who underwent MRI, or 0.9% (1/109) of fetuses with a spinal NTD. Sixty-three of the 109 patients with an NTD had postnatal clinical data available for review. Twenty-nine (46%) of 63 had a syrinx identified during the follow-up period. Of this group, 50 patients had an open NTD and 27 (54%) of 50 developed a syrinx. Among the patients with an open NTD who developed a syrinx, only 7% did not have or develop hydrocephalus, compared with 35% of the patients who did not develop a syrinx (p < 0.05). There were nonsignificantly more frequent shunt revisions among those patients who developed a syrinx, and a syrinx developed in all patients who required surgical Chiari malformation decompression or tethered cord release. The initial identification of a spinal cord syrinx varied greatly between patients, ranging from 38 weeks gestation to greater than 4 years of age. Conclusions These data suggest that syringomyelia is not a congenital embryonic condition. A syrinx was not identified in fetuses who underwent imaging for other intrauterine anomalies. In the population of patients with NTDs who are known to be at high risk for developing syringomyelia, the pathology was only identified in 2 third-trimester fetuses or postnatally, typically in the presence of hydrocephalus, shunt placement, Chiari malformation decompression, or tethered cord release. The study supports the authors' hypothesis that a syrinx is an acquired lesion, most likely due to the effects of abnormal CSF flow.

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Chiari malformation and spinal interdural cyst: A proposed association and review of the literature

Surgical Neurology International ◽

10.25259/sni_1034_2021 ◽

2021 ◽

Vol 12 ◽

pp. 626

Author(s):

Raj Thakrar ◽

Bruce Tranmer ◽

Paul Penar

Keyword(s):

Chiari Malformation ◽

Treatment Options ◽

Flow Dynamics ◽

Review Of The Literature ◽

Csf Flow ◽

Csf Diversion ◽

Unclear Etiology ◽

Lumbar Cerebrospinal Fluid ◽

Meningeal Cysts ◽

Mass Lesions

Background: Interdural cysts are rare meningeal cysts with an unclear etiology. They are often mistaken for other mass lesions, including arachnoid cysts and tumors. Correctly identifying and classifying these cysts, as well as how they have formed in individual patients, are crucial to providing effective treatment options for patients. Case Description: We report a case of a patient with shunted idiopathic intracranial hypertension who developed a symptomatic Chiari malformation and was subsequently discovered to have a spinal interdural cyst. The Chiari malformation was likely due to intracranial hypotension secondary to lumbar cerebrospinal fluid (CSF) diversion. Once the shunt was removed, a spinal interdural cyst became clinically and radiographically evident, and the Chiari resolved, suggesting that both entities were effects of shared CSF flow dynamics. Conclusion: This cyst likely originated due to the trauma from remote repeated lumbar punctures and lumboperitoneal shunt placement, allowing CSF to enter the interdural space after the catheter was removed.

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Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.1.peds18755 ◽

2020 ◽

Vol 26 (1) ◽

pp. 53-59 ◽

Cited By ~ 2

Author(s):

Jennifer M. Strahle ◽

Rukayat Taiwo ◽

Christine Averill ◽

James Torner ◽

Jordan I. Gewirtz ◽

...

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Craniocervical Junction ◽

Curve Progression ◽

Posterior Fossa Decompression ◽

Type I ◽

Younger Age ◽

Curve Magnitude ◽

The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

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Assessment of Cerebrospinal Fluid Hydrodynamics Using Magnetic Resonance Imaging in Postcraniospinal Surgery Patients

Indian Journal of Radiology and Imaging ◽

10.1055/s-0041-1734362 ◽

2021 ◽

Author(s):

Pankaj Arora ◽

Kanica Rawat ◽

Rajiv Azad ◽

Kehkashan Chouhan

Keyword(s):

Magnetic Resonance Imaging ◽

Cerebrospinal Fluid ◽

Magnetic Resonance ◽

Clinical Outcome ◽

Phase Contrast ◽

Flow Dynamics ◽

Resonance Imaging ◽

Csf Flow ◽

Group I ◽

Group Ii

Abstract Objective Aim of this study is to evaluate the effect of craniospinal interventions on cerebrospinal fluid (CSF) flow hydrodynamics and study the correlation of postoperative changes in flow alteration with clinical outcome. Materials and Methods Fifty patients who underwent various craniospinal procedures were studied using conventional and phase-contrast magnetic resonance imaging (PCMRI) protocol. CSF flow quantification was performed at cerebral aqueduct, foramen magnum, C2–3, and D12–L1 vertebral levels with site showing maximal alteration of CSF flow dynamics considered as the region of interest. Velocity encoding was kept at 20 cm/s. Patients with pathology atcraniovertebral junction were considered separately (group I) from others (group II) due to different flow dynamics. Follow-up scans were performed after an interval of 1 month for temporal evaluation of changes in CSF flow dynamics. Results Patients in both groups showed a significant change in peak CSF velocity postoperatively (mean change of 1.34 cm/s in group I and 0.28 cm/s in group II) with bidirectional improvement in flow on cine-phase-contrast qualitative images. Regional pain (82%) and headache (46%) were seen in most of the patients preoperatively. Postoperatively clinical symptoms improved in 59.5%, static in 26.2%, and worsened in 14.3%. In both the groups, an improvement in clinical symptomatology had significant correlation with mean changes in peak CSF velocity postoperatively (p = 0.04 in both groups). Conclusion PCMRI can effectively evaluate changes in CSF flow noninvasively both pre- and postoperatively. This may have potential role in determining clinical outcome and prognosis of patients undergoing procedures in craniospinal axis.

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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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Analysis of Intracranial CSF Flow Dynamics in Phase-Contrast Cine MRI

Japanese Journal of Radiological Technology ◽

10.6009/jjrt.kj00001354933 ◽

1996 ◽

Vol 52 (9) ◽

pp. 1191

Author(s):

Harumasa Kasai ◽

Toshiaki Miyati ◽

Tatsuo Banno ◽

Kazuya Ohashi ◽

Takahiro Sakurai ◽

...

Keyword(s):

Phase Contrast ◽

Flow Dynamics ◽

Cine Mri ◽

Csf Flow

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Percutaneous endoscopic treatment of suprasellar arachnoid cysts: ventriculocystostomy or ventriculocystocisternostomy?

Journal of Neurosurgery ◽

10.3171/jns.1996.84.4.0696 ◽

1996 ◽

Vol 84 (4) ◽

pp. 696-701 ◽

Cited By ~ 85

Author(s):

Philippe Decq ◽

Pierre Brugières ◽

Caroline Le Guerinel ◽

Michel Djindjian ◽

Yves Kéravel ◽

...

Keyword(s):

Magnetic Resonance ◽

Flow Dynamics ◽

The Other ◽

Arachnoid Cysts ◽

Csf Flow ◽

Content Type ◽

Secondary Closure ◽

Fine Print

✓ The use of an endoscope in the treatment of suprasellar arachnoid cysts provides an opening of the upper and lower cyst walls, thereby allowing the surgeon to perform a ventriculocystostomy (VC) or a ventriculocystocysternostomy (VCC). To discover which procedure is appropriate, magnetic resonance (MR)—imaged cerebrospinal fluid (CSF) flow dynamics in two patients were analyzed, one having undergone a VC and the other a VCC using a rigid endoscope. Magnetic resonance imaging studies were performed before and after treatment, with long-term follow-up periods (18 months and 2 years). The two patients were reoperated on during the follow-up period because of slight headache recurrence in one case and MR—imaged CSF flow dynamics modifications in the other. In each case surgery confirmed the CSF flow dynamics modifications appearing on MR imaging. In both cases, long-term MR imaging follow-up studies showed a secondary closing of the upper wall orifice. After VCC, however, the lower communication between the cyst and the cisterns remained functional. The secondary closure of the upper orifice may be explained as follows: when opened, the upper wall becomes unnecessary and tends to return to a normal shape, leading to a secondary closure. The patent sylvian aqueduct aids this phenomenon, as observed after ventriculostomy when the aqueduct is secondarily functional. The simplicity of the VCC performed using endoscopic control, which is the only procedure to allow the opening in the cyst's lower wall to remain patent, leads the authors to advocate this technique in the treatment of suprasellar arachnoid cysts.

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