Algorithmic Multimodality Approach to Diagnosis and Treatment of Spinal CSF Leak and Venous Fistula in Patients With Spontaneous Intracranial Hypotension

AJR Am J Roentgenol. 2022 Aug;219(2):292-301. doi: 10.2214/AJR.22.27485. Epub 2022 Mar 9.


Spontaneous intracranial hypotension (SIH) is a disorder of CSF dynamics that causes a complex clinical syndrome and severe disability. SIH is challenging to diagnose because of the variability of its presenting clinical symptoms, the potential for subtle imaging findings to be easily overlooked, and the need for specialized diagnostic testing. Once SIH is suggested by clinical history and/or supported by initial neuroim-aging, many patients may undergo initial nontargeted epidural blood patching with variable and indefinite benefit. However, data suggest that precise localization of the CSF leak or CSF-venous fistula (CVF) can lead to more effective and durable treatment strategies. Leak localization can be achieved using a variety of advanced diagnostic imaging techniques, although these may not be widely performed at nontertiary medical centers, leaving many patients with the potential for inadequate workup or treatment. This review describes imaging techniques including dynamic fluoroscopic and CT myelography as well as delayed MR myelography and treatment options including percutaneous, endovascular, and surgical approaches for SIH. These are summarized by an algorithmic framework for radiologists to approach the workup and treatment of patients with suspected SIH. The importance of a multidisciplinary approach is emphasized.

Keywords: CSF leak; CSF-venous fistula; dynamic myelography; spontaneous intracranial hypotension.

Publication types

  • Review

MeSH terms

  • Cerebrospinal Fluid Leak / complications
  • Cerebrospinal Fluid Leak / diagnostic imaging
  • Cerebrospinal Fluid Leak / therapy
  • Fistula*
  • Humans
  • Intracranial Hypotension* / diagnostic imaging
  • Intracranial Hypotension* / etiology
  • Intracranial Hypotension* / therapy
  • Magnetic Resonance Imaging / methods
  • Myelography / adverse effects
  • Tomography, X-Ray Computed / methods