Secondary Chiari malformation due to enlarged spinal arachnoid villi-like structure: illustrative case

Secondary Chiari malformation can be caused by various disorders associated with cerebrospinal fluid (CSF) leakage at the spinal level. In this report, the authors describe a rare case of secondary Chiari malformation caused by excessive CSF absorption through the enlarged spinal arachnoid villi-like structure.

A 20-year-old woman presented with progressive severe headache and posterior neck pain. Magnetic resonance imaging showed tonsillar herniation and decreased subarachnoid space around the spinal cord. A hypointense signal area was observed in the ventral spinal canal on a T2-weighted image. An axial image revealed multiple small, arachnoid cyst-like structures at the right T1 nerve root sleeve. Direct surgery revealed that the cyst-like structures were continuous with the arachnoid membrane and protruded into the abnormally large epidural venous sinus. The cyst-like structures were resected, and the dural sleeve was repaired using fascia. The patient showed good improvement of symptoms after surgery.

Excessive CSF absorption through the enlarged spinal arachnoid villi-like structure can cause secondary Chiari malformation. Neurosurgeons should be aware of this unusual mechanism of CSF leakage. Simple posterior fossa decompression will be ineffective or even harmful.