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Dural Dysplasia
Kevin R. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Dural ectasia
              • Definitions

                • Patulous dural sac with posterior vertebral scalloping

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Angiographic Findings

                          • Nonvascular Interventions

                            • 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. Jain VV et al: Dural ectasia in a child with larsen syndrome. J Pediatr Orthop. 34(7):e44-9, 2014
                                                  2. Kono AK et al: Prevalence of dural ectasia in Loeys-Dietz syndrome: comparison with Marfan syndrome and normal controls. PLoS One. 8(9):e75264, 2013
                                                  3. Sznajder M et al: Spinal imaging contributes to the diagnosis of Marfan syndrome. Joint Bone Spine. Epub ahead of print, 2010
                                                  4. García-Estevez DA et al: [Dural ectasia in the cervical spine and neurofibromatosis type 1.] Rev Neurol. 48(1):51, 2009
                                                  5. Sanz-Ayan MP et al: [Dural ectasia and lumbar pain associated with Marfan syndrome.] Rev Neurol. 47(10):559-60, 2008
                                                  6. Kotil K et al: Lumbar radiculopathy in ankylosing spondylitis with dural ectasia. J Clin Neurosci. 14(10):981-3, 2007
                                                  7. Knirsch W et al: Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study. Am J Med Genet A. 140(7):775-81, 2006
                                                  8. Reitman CA et al: Neurologic and morphologic features of dural ectasia in ankylosing spondylitis and rheumatoid arthritis: a case report. Am J Orthop (Belle Mead NJ). 35(11):530-1, 2006
                                                  9. Habermann CR et al: MR evaluation of dural ectasia in Marfan syndrome: reassessment of the established criteria in children, adolescents, and young adults. Radiology. 234(2):535-41, 2005
                                                  10. Knirsch W et al: Normal values of the sagittal diameter of the lumbar spine (vertebral body and dural sac) in children measured by MRI. Pediatr Radiol. 35(4):419-24, 2005
                                                  11. Tsirikos AI et al: Assessment of vertebral scalloping in neurofibromatosis type 1 with plain radiography and MRI. Clin Radiol. 59(11):1009-17, 2004
                                                  12. Nallamshetty L et al: Dural ectasia and back pain: review of the literature and case report. J Spinal Disord Tech. 15(4):326-9, 2002
                                                  13. Tubbs RS et al: Dural ectasia in neurofibromatosis. Pediatr Neurosurg. 37(6):331-2, 2002
                                                  14. Ahn NU et al: Dural ectasia and conventional radiography in the Marfan lumbosacral spine. Skeletal Radiol. 30(6):338-45, 2001
                                                  15. Oosterhof T et al: Quantitative assessment of dural ectasia as a marker for Marfan syndrome. Radiology. 220(2):514-8, 2001
                                                  16. Ahn NU et al: Dural ectasia in the Marfan syndrome: MR and CT findings and criteria. Genet Med. 2(3):173-9, 2000
                                                  17. Ahn NU et al: Dural ectasia is associated with back pain in Marfan syndrome. Spine. 25(12):1562-8, 2000
                                                  18. Rose PS et al: A comparison of the Berlin and Ghent nosologies and the influence of dural ectasia in the diagnosis of Marfan syndrome. Genet Med. 2(5):278-82, 2000
                                                  19. Schonauer C et al: Lumbosacral dural ectasia in type 1 neurofibromatosis. Report of two cases. J Neurosurg Sci. 44(3):165-8; discussion 169, 2000
                                                  20. Sponseller PD et al: Osseous anatomy of the lumbosacral spine in Marfan syndrome. Spine. 25(21):2797-802, 2000
                                                  21. De Paepe A: Dural ectasia and the diagnosis of Marfan's syndrome. Lancet. 354(9182):878-9, 1999
                                                  22. Fattori R et al: Importance of dural ectasia in phenotypic assessment of Marfan's syndrome. Lancet. 354(9182):910-3, 1999
                                                  23. Villeirs GM et al: Widening of the spinal canal and dural ectasia in Marfan's syndrome: assessment by CT. Neuroradiology. 41(11):850-4, 1999
                                                  24. Raff ML et al: Joint hypermobility syndromes. Curr Opin Rheumatol. 8(5):459-66, 1996
                                                  25. Helfen M et al: Intrathoracic dural ectasia mimicking neurofibroma and scoliosis. A case report. Int Orthop. 19(3):181-4, 1995
                                                  26. Bensaid AH et al: Neurofibromatosis with dural ectasia and bilateral symmetrical pedicular clefts: report of two cases. Neuroradiology. 34(2):107-9, 1992
                                                  27. Pyeritz RE et al: Dural ectasia is a common feature of the Marfan syndrome. Am J Hum Genet. 43(5):726-32, 1988
                                                  28. Stern WE: Dural ectasia and the Marfan syndrome. J Neurosurg. 69(2):221-7, 1988
                                                  29. Katz SG et al: Thoracic and lumbar dural ectasia in a two-year-old boy. Pediatr Radiol. 6(4):238-40, 1978
                                                  Related Anatomy
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                                                  Related Differential Diagnoses
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                                                  References
                                                  Tables

                                                  Tables

                                                  KEY FACTS

                                                  • Terminology

                                                    • Imaging

                                                      • Top Differential Diagnoses

                                                        • Pathology

                                                          • Clinical Issues

                                                            • Diagnostic Checklist

                                                              TERMINOLOGY

                                                              • Synonyms

                                                                • Dural ectasia
                                                              • Definitions

                                                                • Patulous dural sac with posterior vertebral scalloping

                                                              IMAGING

                                                              • General Features

                                                                • Radiographic Findings

                                                                  • CT Findings

                                                                    • MR Findings

                                                                      • Ultrasonographic Findings

                                                                        • Angiographic Findings

                                                                          • Nonvascular Interventions

                                                                            • 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. Jain VV et al: Dural ectasia in a child with larsen syndrome. J Pediatr Orthop. 34(7):e44-9, 2014
                                                                                                  2. Kono AK et al: Prevalence of dural ectasia in Loeys-Dietz syndrome: comparison with Marfan syndrome and normal controls. PLoS One. 8(9):e75264, 2013
                                                                                                  3. Sznajder M et al: Spinal imaging contributes to the diagnosis of Marfan syndrome. Joint Bone Spine. Epub ahead of print, 2010
                                                                                                  4. García-Estevez DA et al: [Dural ectasia in the cervical spine and neurofibromatosis type 1.] Rev Neurol. 48(1):51, 2009
                                                                                                  5. Sanz-Ayan MP et al: [Dural ectasia and lumbar pain associated with Marfan syndrome.] Rev Neurol. 47(10):559-60, 2008
                                                                                                  6. Kotil K et al: Lumbar radiculopathy in ankylosing spondylitis with dural ectasia. J Clin Neurosci. 14(10):981-3, 2007
                                                                                                  7. Knirsch W et al: Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study. Am J Med Genet A. 140(7):775-81, 2006
                                                                                                  8. Reitman CA et al: Neurologic and morphologic features of dural ectasia in ankylosing spondylitis and rheumatoid arthritis: a case report. Am J Orthop (Belle Mead NJ). 35(11):530-1, 2006
                                                                                                  9. Habermann CR et al: MR evaluation of dural ectasia in Marfan syndrome: reassessment of the established criteria in children, adolescents, and young adults. Radiology. 234(2):535-41, 2005
                                                                                                  10. Knirsch W et al: Normal values of the sagittal diameter of the lumbar spine (vertebral body and dural sac) in children measured by MRI. Pediatr Radiol. 35(4):419-24, 2005
                                                                                                  11. Tsirikos AI et al: Assessment of vertebral scalloping in neurofibromatosis type 1 with plain radiography and MRI. Clin Radiol. 59(11):1009-17, 2004
                                                                                                  12. Nallamshetty L et al: Dural ectasia and back pain: review of the literature and case report. J Spinal Disord Tech. 15(4):326-9, 2002
                                                                                                  13. Tubbs RS et al: Dural ectasia in neurofibromatosis. Pediatr Neurosurg. 37(6):331-2, 2002
                                                                                                  14. Ahn NU et al: Dural ectasia and conventional radiography in the Marfan lumbosacral spine. Skeletal Radiol. 30(6):338-45, 2001
                                                                                                  15. Oosterhof T et al: Quantitative assessment of dural ectasia as a marker for Marfan syndrome. Radiology. 220(2):514-8, 2001
                                                                                                  16. Ahn NU et al: Dural ectasia in the Marfan syndrome: MR and CT findings and criteria. Genet Med. 2(3):173-9, 2000
                                                                                                  17. Ahn NU et al: Dural ectasia is associated with back pain in Marfan syndrome. Spine. 25(12):1562-8, 2000
                                                                                                  18. Rose PS et al: A comparison of the Berlin and Ghent nosologies and the influence of dural ectasia in the diagnosis of Marfan syndrome. Genet Med. 2(5):278-82, 2000
                                                                                                  19. Schonauer C et al: Lumbosacral dural ectasia in type 1 neurofibromatosis. Report of two cases. J Neurosurg Sci. 44(3):165-8; discussion 169, 2000
                                                                                                  20. Sponseller PD et al: Osseous anatomy of the lumbosacral spine in Marfan syndrome. Spine. 25(21):2797-802, 2000
                                                                                                  21. De Paepe A: Dural ectasia and the diagnosis of Marfan's syndrome. Lancet. 354(9182):878-9, 1999
                                                                                                  22. Fattori R et al: Importance of dural ectasia in phenotypic assessment of Marfan's syndrome. Lancet. 354(9182):910-3, 1999
                                                                                                  23. Villeirs GM et al: Widening of the spinal canal and dural ectasia in Marfan's syndrome: assessment by CT. Neuroradiology. 41(11):850-4, 1999
                                                                                                  24. Raff ML et al: Joint hypermobility syndromes. Curr Opin Rheumatol. 8(5):459-66, 1996
                                                                                                  25. Helfen M et al: Intrathoracic dural ectasia mimicking neurofibroma and scoliosis. A case report. Int Orthop. 19(3):181-4, 1995
                                                                                                  26. Bensaid AH et al: Neurofibromatosis with dural ectasia and bilateral symmetrical pedicular clefts: report of two cases. Neuroradiology. 34(2):107-9, 1992
                                                                                                  27. Pyeritz RE et al: Dural ectasia is a common feature of the Marfan syndrome. Am J Hum Genet. 43(5):726-32, 1988
                                                                                                  28. Stern WE: Dural ectasia and the Marfan syndrome. J Neurosurg. 69(2):221-7, 1988
                                                                                                  29. Katz SG et al: Thoracic and lumbar dural ectasia in a two-year-old boy. Pediatr Radiol. 6(4):238-40, 1978