Le siringomielie primitivamente spinali

Syringomielia could be classified according to the presence or absence of association with hindbrain pathology. Therefore, hindbrain-related syringomielia could either appear as a result of an hindrance to the cranio-spinal CSF flow (3/4 cases) usually as a consequence of a malformation (Dandy-Walker, Chiari I-II, basilar impression) or could less frequently show up as an acquired form caused by tumors or foramen magnum arachnoiditis. In the non hindbrain-related syringomielia, the perimedullary CSF flow is slowed by a diffuse spinal meningeal fibrosis, caused by spinal trauma, bacterial or chemical (contrast medium) meningitis, Pott disease, scoliosis, spinal canal stenosis. The aim of the present review is to analyze the most convincing etiopathogenetic hypotheses regarding the so called non hindbrain syringomielia and, in the light of these etiologic concepts, to review the current therapeutic options. A typical case of primitive spinal syringomielia, associated with Pott disease, is also reported, describing the rationale of the adopted surgical procedure and the therapeutic result, also evaluated with spinal MRI