Subarachnoid pressure-dependent change in syrinx size in a patient with syringomyelia associated with adhesive arachnoiditis - Case report

The pathophysiology of syringomyelia is still not well understood. Current prevailing theories involve the assumption that cerebrospinal fluid (CSF) flows into the syrinx from the subarachnoid space through the perivascular space of Virchow-Robin. Reported here is the case of a patient with syringomyelia in which this course is clearly contradicted. This patient with a holocord syrinx associated with adhesive arachnoiditis was treated 3 years previously with insertion of a subarachnoid-peritoneal shunt and had recently experienced worsening myelopathy. On surgical exploration, the shunt system was functioning normally. The medium-pressure shunt valve was replaced with an adjustable valve with a higher closing pressure setting, thus increasing the CSF pressure in the subarachnoid space. Contrary to prevailing theories, this procedure markedly reduced the size of the syrinx. This case provides direct evidence that the syrinx size is inversely related to subarachnoid CSF pressure and supports the hypothesis that the pressure gradient across the spinal cord parenchyma is the force that generates syringes in syringomyelia.