Pseudo Chiari with holocord syringomyelia secondary to cerebrospinal fluid hypotension. Case report

Abstract

Introduction: Pseudo-Chiari malformation results from caudal displacement of brain structures secondary to cerebrospinal fluid (CSF) hypotension. Some case reports in the syringomyelia literature are associated with a pseudo-Chiari malformation. We present an example of a case of this type, treated surgically at our institution and review the literature on this rare condition.Case descriptionA 27-year-old female patient with a history of headache, brachial paresthesias, progressive motor deficit and gait instability presented, 12 mm caudal descent of the cerebellar tonsils into the foramen magnum on MRI, holocord syringomyelia as well as signs of pseudo-Chiari malformation, compatible CSF hypotension secondary to CSF fistula.InterventionSurgical treatment included posterior decompression with repair using a dural patch and an epidural blood patch. Patient had a favorable clinical outcome in line with imaging findings observed during follow-up. Conclusion: Posterior decompression surgery combined with epidural blood patch allowed successful clinical management and good results on imaging.