Neurosurgery Blog

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.

Neurosurg Rev (2010) 33:271–285. DOI 10.1007/s10143-010-0266-5

The exact pathogenesis of syringomyelia associated with Chiari type 1 malformation is unknown, although a number of authors have reported their theories of syrinx formation. The purpose of this review is to understand evidences based on the known theories and to create a new hypothesis of the pathogenesis. We critically review the literatures on clinicopathological, radiological, and clinical features of this disorder.

The previously proposed theories mainly focused on the driven mechanisms of the cerebrospinal fluid (CSF) into the spinal cord. They did not fully explain radiological features or effects of surgical treatment such as shunting procedures.

Common findings of the syrinx in clinicopathological studies were the communication with the central canal and extracanalicular extension to the posterior gray matter. Most of the magnetic resonance imaging studies demonstrated blockade and alternated CSF dynamics at the foramen magnum, but failed to show direct communication of the syrinx with the CSF spaces.

Pressure studies revealed almost identical intrasyrinx pressure to the subarachnoid space and decreased compliance of the spinal CSF space.

Recent imaging studies suggest that the extracellular fluid accumulation may play an important role. The review of evidences promotes a new hypothesis of syrinx formation. Decreased absorption mechanisms of the extracellular fluid may underlie the pathogenesis of syringomyelia. Reduced compliance of the posterior spinal veins associated with the decreased compliance of the spinal subarachnoid space will result in disturbed absorption of the extracellular fluid through the intramedullary venous channels and formation of syringomyelia.