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Intraosseous CSF fistula in a patient with Gorham disease resulting in intracranial hypotension

F Adler1, N Gupta, C P Hess

  • 1Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA, USA.

AJNR. American Journal of Neuroradiology
|June 11, 2011
PubMed

Insights

A spinal cerebrospinal fluid (CSF)-lymphatic fistula caused a young girl's Chiari I malformation, leading to headaches. Closing the fistula provided only temporary relief for her intracranial hypotension.

Area of Science:

  • Neurology
  • Neurosurgery
  • Pediatrics

Background:

  • Chiari I malformation is a condition where brain tissue extends into the spinal canal.
  • Intracranial hypotension can cause symptoms like headaches and cerebellar tonsillar ectopia.
  • Gorham disease is a rare condition characterized by the progressive loss of bone.

Observation:

  • A 7-year-old girl presented with headaches and a history of Gorham disease.
  • She had undergone surgery for Chiari I malformation in infancy.
  • Her cerebellar tonsillar ectopia was found to be caused by a spinal cerebrospinal fluid (CSF)-lymphatic fistula leading to intracranial hypotension.

Findings:

  • The patient's cerebellar tonsillar ectopia was secondary to a long-standing spinal CSF-lymphatic fistula.
  • This fistula resulted in chronic intracranial hypotension.
  • Multiple attempts at percutaneous fistula closure were performed.

Implications:

  • Spinal CSF-lymphatic fistulas can cause or exacerbate Chiari I malformation and intracranial hypotension.
  • The findings highlight the importance of investigating underlying causes for Chiari I malformation, especially in complex cases.
  • Further research is needed to explore long-term effective treatments for spinal CSF-lymphatic fistulas and associated conditions.

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