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CSF Leakage Syndrome, Craniovertebral Junction
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        TERMINOLOGY

        • Synonyms

          • Dural dehiscence, pseudocyst, cerebrospinal fluid (CSF) leak
        • Definitions

          • Symptomatic leakage of CSF
          • CSF leakage through dural defect may collect under skin and tissue planes and result in pseudomeningocele
          • Primary or spontaneous intracranial hypotension (SIH) = dural defect and CSF leak cannot be attributed to postoperative state or trauma
          • Secondary intracranial hypotension = postoperative, iatrogenic, or post-traumatic CSF leak ± pseudomeningocele

        IMAGING

        • General Features

          • CT Findings

            • MR Findings

              • Other Modality Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Alaraj A et al: Angiographic features of "brain sag". J Neurosurg. 115(3):586-91, 2011
                                      2. Cho KI et al: Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology. 76(13):1139-44, 2011
                                      3. Kranz PG et al: CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 32(5):832-8, 2011
                                      4. Rahman M et al: Spontaneous intracranial hypotension: dilemmas in diagnosis. Neurosurgery. 69(1):4-14, 2011
                                      5. Thomas DL et al: Radionuclide cisternography in detecting cerebrospinal fluid leak in spontaneous intracranial hypotension: a series of four case reports. Clin Nucl Med. 34(7):410-6, 2009
                                      6. Wang YF et al: Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology. 73(22):1892-8, 2009
                                      7. Albayram S et al: Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. AJNR Am J Neuroradiol. 29(1):116-21, 2008
                                      8. Tsai PH et al: Comparisons between heavily T2-weighted MR and CT myelography studies in two patients with spontaneous intracranial hypotension. Cephalalgia. 28(6):653-7, 2008
                                      9. Yoo HM et al: Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with radioisotope cisternography. AJNR Am J Neuroradiol. 29(4):649-54, 2008
                                      10. Tsai PH et al: Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study. Cephalalgia. 27(8):929-34, 2007
                                      11. Cohen-Gadol AA et al: Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks. Neurosurgery. 58(4 Suppl 2):ONS-238-45; discussion ONS-245, 2006
                                      12. Kelley GR et al: Sinking brain syndrome: craniotomy can precipitate brainstem herniation in CSF hypovolemia. Neurology. 62(1):157, 2004
                                      13. Eross EJ et al: Prolonged neurologic complication and MRI abnormalities consequent to intracranial hypotension. Headache. 43(4):415, 2003
                                      14. Luetmer PH et al: Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol. 24(8):1711-4, 2003
                                      15. Rothrock JF: Low pressure headache and pseudosubdural hematomas. Headache. 43(9):1009, 2003
                                      16. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leak associated with "nude nerve root" syndrome: case report. Neurosurgery. 53(5):1216-8; discussion 1218-9, 2003
                                      17. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. J Neurosurg. 99(5):840-2, 2003
                                      18. Whiteley W et al: Spontaneous intracranial hypotension causing confusion and coma: a headache for the neurologist and the neurosurgeon. Br J Neurosurg. 17(5):456-8, 2003
                                      19. Black P: Cerebrospinal fluid leaks following spinal surgery: use of fat grafts for prevention and repair. Technical note. J Neurosurg. 96(2 Suppl):250-2, 2002
                                      20. Chen CJ et al: Spinal MR findings in spontaneous intracranial hypotension. Neuroradiology. 44(12):996-1003, 2002
                                      21. Fujimaki H et al: Cerebrospinal fluid leak demonstrated by three-dimensional computed tomographic myelography in patients with spontaneous intracranial hypotension. Surg Neurol. 58(3-4):280-4; discussion 284-5, 2002
                                      22. Schrijver I et al: Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. J Neurosurg. 96(3):483-9, 2002
                                      23. Dillon WP: Spinal manifestations of intracranial hypotension. AJNR Am J Neuroradiol. 22(7):1233-4, 2001
                                      24. Yousry I et al: Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol. 22(7):1239-50, 2001
                                      25. Patel MR et al: CT-guided percutaneous fibrin glue therapy of cerebrospinal fluid leaks in the spine after surgery. AJR Am J Roentgenol. 175(2):443-6, 2000
                                      26. O'Carroll CP et al: The syndrome of spontaneous intracranial hypotension. Cephalalgia. 19(2):80-7, 1999
                                      27. Atkinson JL et al: Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg. 88(2):237-42, 1998
                                      28. Moayeri NN et al: Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. Report of three cases and review of the literature. J Neurosurg. 88(5):912-8, 1998
                                      29. Rabin BM et al: Spontaneous intracranial hypotension: spinal MR findings. AJNR Am J Neuroradiol. 19(6):1034-9, 1998
                                      30. Vakharia SB et al: Magnetic resonance imaging of cerebrospinal fluid leak and tamponade effect of blood patch in postdural puncture headache. Anesth Analg. 84(3):585-90, 1997
                                      31. McCormack BM et al: Pseudomeningocele/CSF fistula in a patient with lumbar spinal implants treated with epidural blood patch and a brief course of closed subarachnoid drainage. A case report. Spine. 21(19):2273-6, 1996
                                      32. Renowden SA et al: Spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 59(5):511-5, 1995
                                      33. Maycock NF et al: Post-laminectomy cerebrospinal fluid fistula treated with epidural blood patch. Spine. 19(19):2223-5, 1994
                                      34. Lee KS et al: Postlaminectomy lumbar pseudomeningocele: report of four cases. Neurosurgery. 30(1):111-4, 1992
                                      35. Waisman M et al: Postoperative cerebrospinal fluid leakage after lumbar spine operations. Conservative treatment. Spine. 16(1):52-3, 1991
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Pathology

                                            TERMINOLOGY

                                            • Synonyms

                                              • Dural dehiscence, pseudocyst, cerebrospinal fluid (CSF) leak
                                            • Definitions

                                              • Symptomatic leakage of CSF
                                              • CSF leakage through dural defect may collect under skin and tissue planes and result in pseudomeningocele
                                              • Primary or spontaneous intracranial hypotension (SIH) = dural defect and CSF leak cannot be attributed to postoperative state or trauma
                                              • Secondary intracranial hypotension = postoperative, iatrogenic, or post-traumatic CSF leak ± pseudomeningocele

                                            IMAGING

                                            • General Features

                                              • CT Findings

                                                • MR Findings

                                                  • Other Modality Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Gross Pathologic & Surgical Features

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Consider

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Alaraj A et al: Angiographic features of "brain sag". J Neurosurg. 115(3):586-91, 2011
                                                                          2. Cho KI et al: Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology. 76(13):1139-44, 2011
                                                                          3. Kranz PG et al: CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 32(5):832-8, 2011
                                                                          4. Rahman M et al: Spontaneous intracranial hypotension: dilemmas in diagnosis. Neurosurgery. 69(1):4-14, 2011
                                                                          5. Thomas DL et al: Radionuclide cisternography in detecting cerebrospinal fluid leak in spontaneous intracranial hypotension: a series of four case reports. Clin Nucl Med. 34(7):410-6, 2009
                                                                          6. Wang YF et al: Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology. 73(22):1892-8, 2009
                                                                          7. Albayram S et al: Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. AJNR Am J Neuroradiol. 29(1):116-21, 2008
                                                                          8. Tsai PH et al: Comparisons between heavily T2-weighted MR and CT myelography studies in two patients with spontaneous intracranial hypotension. Cephalalgia. 28(6):653-7, 2008
                                                                          9. Yoo HM et al: Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with radioisotope cisternography. AJNR Am J Neuroradiol. 29(4):649-54, 2008
                                                                          10. Tsai PH et al: Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study. Cephalalgia. 27(8):929-34, 2007
                                                                          11. Cohen-Gadol AA et al: Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks. Neurosurgery. 58(4 Suppl 2):ONS-238-45; discussion ONS-245, 2006
                                                                          12. Kelley GR et al: Sinking brain syndrome: craniotomy can precipitate brainstem herniation in CSF hypovolemia. Neurology. 62(1):157, 2004
                                                                          13. Eross EJ et al: Prolonged neurologic complication and MRI abnormalities consequent to intracranial hypotension. Headache. 43(4):415, 2003
                                                                          14. Luetmer PH et al: Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol. 24(8):1711-4, 2003
                                                                          15. Rothrock JF: Low pressure headache and pseudosubdural hematomas. Headache. 43(9):1009, 2003
                                                                          16. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leak associated with "nude nerve root" syndrome: case report. Neurosurgery. 53(5):1216-8; discussion 1218-9, 2003
                                                                          17. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. J Neurosurg. 99(5):840-2, 2003
                                                                          18. Whiteley W et al: Spontaneous intracranial hypotension causing confusion and coma: a headache for the neurologist and the neurosurgeon. Br J Neurosurg. 17(5):456-8, 2003
                                                                          19. Black P: Cerebrospinal fluid leaks following spinal surgery: use of fat grafts for prevention and repair. Technical note. J Neurosurg. 96(2 Suppl):250-2, 2002
                                                                          20. Chen CJ et al: Spinal MR findings in spontaneous intracranial hypotension. Neuroradiology. 44(12):996-1003, 2002
                                                                          21. Fujimaki H et al: Cerebrospinal fluid leak demonstrated by three-dimensional computed tomographic myelography in patients with spontaneous intracranial hypotension. Surg Neurol. 58(3-4):280-4; discussion 284-5, 2002
                                                                          22. Schrijver I et al: Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. J Neurosurg. 96(3):483-9, 2002
                                                                          23. Dillon WP: Spinal manifestations of intracranial hypotension. AJNR Am J Neuroradiol. 22(7):1233-4, 2001
                                                                          24. Yousry I et al: Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol. 22(7):1239-50, 2001
                                                                          25. Patel MR et al: CT-guided percutaneous fibrin glue therapy of cerebrospinal fluid leaks in the spine after surgery. AJR Am J Roentgenol. 175(2):443-6, 2000
                                                                          26. O'Carroll CP et al: The syndrome of spontaneous intracranial hypotension. Cephalalgia. 19(2):80-7, 1999
                                                                          27. Atkinson JL et al: Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg. 88(2):237-42, 1998
                                                                          28. Moayeri NN et al: Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. Report of three cases and review of the literature. J Neurosurg. 88(5):912-8, 1998
                                                                          29. Rabin BM et al: Spontaneous intracranial hypotension: spinal MR findings. AJNR Am J Neuroradiol. 19(6):1034-9, 1998
                                                                          30. Vakharia SB et al: Magnetic resonance imaging of cerebrospinal fluid leak and tamponade effect of blood patch in postdural puncture headache. Anesth Analg. 84(3):585-90, 1997
                                                                          31. McCormack BM et al: Pseudomeningocele/CSF fistula in a patient with lumbar spinal implants treated with epidural blood patch and a brief course of closed subarachnoid drainage. A case report. Spine. 21(19):2273-6, 1996
                                                                          32. Renowden SA et al: Spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 59(5):511-5, 1995
                                                                          33. Maycock NF et al: Post-laminectomy cerebrospinal fluid fistula treated with epidural blood patch. Spine. 19(19):2223-5, 1994
                                                                          34. Lee KS et al: Postlaminectomy lumbar pseudomeningocele: report of four cases. Neurosurgery. 30(1):111-4, 1992
                                                                          35. Waisman M et al: Postoperative cerebrospinal fluid leakage after lumbar spine operations. Conservative treatment. Spine. 16(1):52-3, 1991