The acquired Chiari malformation and syringomyelia following spinal CSF drainage: a study of incidence and management

Acta Neurochir (Wien). 1998;140(5):417-27; discussion 427-8. doi: 10.1007/s007010050119.


Firstly, 14 patients are described who developed either an acquired Chiari malformation (ACM) alone (7 cases) or ACM and syringomyelia (7 cases) after lumbar subarachnoid space (SAS) shunting or in one case, epidural anaesthesia with SAS penetration. Four groups are considered: 3 cases with craniofacial dysostosis and communicating hydrocephalus (CH), 4 cases with CH alone, 3 cases with pseudotumour cerebri (PTC) and a miscellaneous group (4 cases). Initial treatment was varied: resiting the shunt to ventricle or cisterna magna [6], adding an H-V valve [1], syrinx shunting [4] and posterior fossa decompression [3]. Further treatment was required in 6 cases. Secondly, incidence was examined in 87 patients with PTC initially treated either by lumbar SAS shunting [70] or cisterna magna shunting [17]. In the first sub-group, 11 cases (15.7 per cent) developed an ACM, 3 symptomatic (as above) and eight asymptomatic with 1 case also having syringomyelia whereas 1 case occurred in the second group with a questionanably symptomatic ACM. While accurate for symptomatic lesions, these figures are tentative with respect to asymptomatic lesions due to inadequate pre-treatment radiology and detailed MR follow-up. The main conclusions are, first, that the incidence of symptomatic ACM and/or syringomyelia is not high enough to warrant abandoning SAS shunting; second that asymptomatic lesions need not necessarily be treated and third, that when treatment is required, shunt resiting is the first choice.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Arnold-Chiari Malformation / epidemiology
  • Arnold-Chiari Malformation / etiology*
  • Arnold-Chiari Malformation / pathology
  • Arnold-Chiari Malformation / therapy
  • Cerebrospinal Fluid Shunts / adverse effects*
  • Cerebrospinal Fluid Shunts / methods
  • Cerebrospinal Fluid Shunts / standards
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Hydrocephalus / surgery
  • Incidence
  • Infant
  • Intracranial Hypotension / surgery
  • Lumbosacral Region
  • Male
  • New South Wales / epidemiology
  • Pseudotumor Cerebri / surgery
  • Reoperation
  • Retrospective Studies
  • Rhombencephalon / pathology
  • Subarachnoid Space
  • Syringomyelia / epidemiology
  • Syringomyelia / etiology*
  • Syringomyelia / pathology
  • Syringomyelia / therapy