Background: Syringomyelia due to intracranial hypotension is rarely described. As a consequence, intracranial hypotension is less recognized as a potential cause of syringomyelia or mistaken with Chiari type 1 malformation. The pathogeny is poorly understood, and we lack diagnostic and therapeutic strategies for this particular setting.
Case description: We describe a 45-year-old patient who developed syringomyelia after about 10 years of undiagnosed intracranial hypotension caused by traumatic C6 cerebrospinal fluid (CSF) leak. Surgical closing of the leak was required to treat intracranial hypotension after failure of conservative measures and blind epidural patches. It led to a marked improvement of cerebral and spinal signs. We discuss the pathogeny of syringomyelia caused by intracranial hypotension and highlight a mechanical theory of hyperpressure against the cervical spine due to blockage of CSF flow by descent of cerebellar tonsils at the foramen magnum level. We describe discriminating clinical and radiologic signs to differentiate intracranial hypotension from Chiari type 1 malformation and discuss mechanisms and causality relating trauma and intracranial hypotension.
Conclusions: Syringomyelia can be a consequence of long-term progression of intracranial hypotension, which must be differentiated from Chiari type 1 malformation. In our case, resolution was achieved by detecting and closing the CSF leak causing the intracranial hypotension. Reports of similar cases are necessary to understand the origin of CSF leak in traumatic intracranial hypotension and assess the best therapeutic strategy.
Keywords: Cerebrospinal fluid leak; Chiari malformation type 1; intracranial hypotension; spinal surgery; syringomyelia.
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