Tag: syrinx

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Duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression for pediatric Chiari malformation type 1 with syrinx

Acta Neurochirurgica (2024) 166:70

In posterior fossa decompression for pediatric Chiari malformation type 1 (CM-1), duraplasty methods using various dural substitutes have been reported to improve surgical outcomes and minimize postoperative complications. To obtain sufficient posterior fossa decompression without cerebrospinal fluid-related complications, we developed a novel duraplasty technique using a combination of a pedicled dural flap and collagen matrix. The objective of this study was to describe the operative nuances of duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression for pediatric CM-1.

Methods We reviewed the clinical and radiographic records of 11 consecutive pediatric patients who underwent posterior fossa decompression with duraplasty using a combination of a pedicled dural flap and collagen matrix followed by expansile cranioplasty for CM-1. The largest area of the syrinx and the size of the posterior fossa were calculated.

Results The maximum syrinx area was reduced by a mean of 68.5% ± 27.3% from preoperatively to postoperatively. Four patients (36.4%) had near-complete syrinx resolution (> 90%, grade III reduction), five (45.5%) had 50% to 90% reduction (grade II), and two (18.2%) had < 50% reduction (grade I). The posterior fossa area in the midsagittal section increased by 8.9% from preoperatively to postoperatively. There were no postoperative complications, including cerebrospinal fluid leakage, pseudomeningocele formation, or infection.

Conclusion Duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression is a promising safe and effective surgical technique for pediatric CM-1 with syrinx.

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Chiari malformation and syringomyelia

J Neurosurg Spine 31:619–628, 2019

Chiari malformation was first described over a century ago, and consists of posterior fossa anomalies that generally share the feature of cerebellar tonsillar descent through the foramen magnum. Our understanding of this disorder was initially based on autopsy studies, and has been greatly enhanced by the advent of MRI.

The surgical management of Chiari anomalies has also evolved in a parallel fashion. Although the exact surgical technique varies among individual surgeons, the goals of surgery remain constant and consist of relieving brainstem compression and cranial nerve distortion, restoring the normal flow of CSF across the foramen magnum, and reducing the size of any associated syrinx cavity. Syrinx cavities are most commonly associated with Chiari anomalies, yet primary spinal syringomyelia (PSS) can be caused by traumatic, infectious, degenerative, and other etiologies that cause at least a partial CSF flow obstruction in the spinal subarachnoid space. As with syringomyelia associated with Chiari anomalies, the main goal of PSS surgery is to reestablish CSF flow across the area of obstruction. In addition to MRI, myelography with CT can be very helpful in the evaluation and management of these patients by identifying focal regions of CSF obstruction that may be amenable to surgical intervention.

Future directions for the treatment of Chiari anomalies and syringomyelia include the application of advanced imaging techniques, more widespread use of genetic evaluation, large-scale outcome studies, and the further refinement of surgical technique.

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Volumetric analysis of syringomyelia following hindbrain decompression for Chiari malformation Type I: syringomyelia resolution follows exponential kinetics

Neurosurg Focus 31 (3):E4, 2011. DOI: 10.3171/2011.6.FOCUS11106

Resolution of syringomyelia is common following hindbrain decompression for Chiari malformation, yet little is known about the kinetics governing this process. The authors sought to establish the volumetric rate of syringomyelia resolution.

Methods. A retrospective cohort of patients undergoing hindbrain decompression for a Chiari malformation Type I with preoperative cervical or thoracic syringomyelia was identified. Patients were included in the study if they had at least 3 neuroimaging studies that detailed the entirety of their preoperative syringomyelia over a minimum of 6 months postoperatively. The authors reconstructed the MR images in 3 dimensions and calculated the volume of the syringomyelia. They plotted the syringomyelia volume over time and constructed regression models using the method of least squares. The Akaike information criterion and Bayesian information criterion were used to calculate the relative goodness of fit. The coefficients of determination R2 (unadjusted and adjusted) were calculated to describe the proportion of variability in each individual data set accounted for by the statistical model.

Results. Two patients were identified as meeting inclusion criteria. Plots of the least-squares best fit were identified as 4.01459e-0.0180804x and 13.2556e-0.00615859x. Decay of the syringomyelia followed an exponential model in both patients (R2 = 0.989582 and 0.948864).

Conclusions. Three-dimensional analysis of syringomyelia resolution over time enables the kinetics to be estimated. This technique is yet to be validated in a large cohort. Because syringomyelia is the final common pathway for a number of different pathological processes, it is possible that this exponential only applies to syringomyelia related to treatment of Chiari malformation Type I.

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Natural history of Chiari malformation Type I

J Neurosurg Pediatrics 8:214–221, 2011.  DOI: 10.3171/2011.5.PEDS1122

The natural history of the Chiari malformation Type I (CM-I) is incompletely understood. The authors report on the outcome of a large group of patients with CM-I that were initially selected for nonsurgical management.

Methods. The authors identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis.

Results. Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not.

Conclusions. In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen.

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