Syringomyelia: a brief review of ontogenetic, experimental and clinical aspects

The generally accepted definition of syringomyelia is that it is a chronically progressive illness characterized by the presence of cavities or syrinxes in the spinal cord. As manifold as the terminology of syringomyelia are the hypotheses of the etiology. Nowadays with MRI without and with gadolinium it is possible to recognize intramedullar cavities safely, the MR especially the cine-MR provides information on pathophysiological details of the flow and intracavitary pressure dependent pulsations of the CSF. Animal models and the findings of own experimental studies have enabled us to study a form of syringomyelia which very closely resembles that brought about by dysrhaphic malformations in the human being and to examine the effectiveness of certain types of surgical therapy. In this paper the term syringomyelia is only used for dysrhaphic cavities in the medulla. After our experience with 61 patients with syringomyelia now we perform the operative decompression of the craniocervical transition as the first step in the operative treatment of the progressive syringomyelia combined with severe craniocervical malformations. In cases with insufficient treatment response we suggest the syringoarachnoid shunting of persisting large intramedullar cavities.