link
Bookmarks
Chiari 2 Malformation
Roya Sohaey, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
10
2
6

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Chiari 2 malformation
              • Arnold Chiari 2
            • Definitions

              • Small posterior fossa with cerebellar compression and hindbrain herniation through foramen magnum
              • Almost always associated with open neural tube defect (ONTD), typically lumbar meningomyelocele

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Chapman T et al: Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, posterior fossa disorders. Clin Imaging. 39(2):167-175, 2015
                                        2. Kappou D et al: Towards detecting open spina bifida in the first trimester: the examination of the posterior brain. Fetal Diagn Ther. 37(4):294-300, 2015
                                        3. Mignone Philpott C et al: Diffusion-weighted imaging of the cerebellum in the fetus with Chiari II malformation. AJNR Am J Neuroradiol. 34(8):1656-60, 2013
                                        4. Fuchs I et al: Normal intracranial translucency and posterior fossa at 11-13 weeks' gestation in a fetus with closed spina bifida. Ultrasound Obstet Gynecol. 40(2):238-9, 2012
                                        5. Chaoui R et al: Prospective detection of open spina bifida at 11-13 weeks by assessing intracranial translucency and posterior brain. Ultrasound Obstet Gynecol. 38(6):722-6, 2011
                                        6. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. 15(1):9-14, 2010
                                        7. Chaoui R et al: Assessment of intracranial translucency (IT) in the detection of spina bifida at the 11-13-week scan. Ultrasound Obstet Gynecol. 34(3):249-52, 2009
                                        8. Wong SK et al: Supratentorial abnormalities in the Chiari II malformationa, III: The interhemispheric cyst. J Ultrasound Med. 28(8):999-1006, 2009
                                        9. D'Addario V et al: Comparison of six sonographic signs in the prenatal diagnosis of spina bifida. J Perinat Med. 36(4):330-4, 2008
                                        10. Ando K et al: MRI tight posterior fossa sign for prenatal diagnosis of Chiari type II malformation. Neuroradiology. 49(12):1033-9, 2007
                                        11. Fujisawa H et al: New ultrasonographic criteria for the prenatal diagnosis of Chiari type 2 malformation. Acta Obstet Gynecol Scand. 85(12):1426-9, 2006
                                        12. Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
                                        13. Tubbs RS et al: Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst. 20(6):375-81, 2004
                                        14. Adzick NS et al: Myelomeningocele: prenatal diagnosis, pathophysiology and management. Semin Pediatr Surg. 12(3):168-74, 2003
                                        15. Beuls E et al: The Arnold-Chiari type II malformation at midgestation. Pediatr Neurosurg. 39(3):149-58, 2003
                                        16. Buisson O et al: Sonographic diagnosis of spina bifida at 12 weeks: heading towards indirect signs. Ultrasound Obstet Gynecol. 19(3):290-2, 2002
                                        17. Coleman BG et al: Fetal therapy: state of the art. J Ultrasound Med. 21(11):1257-88, 2002
                                        18. Jobe AH: Fetal surgery for myelomeningocele. N Engl J Med. 347(4):230-1, 2002
                                        Related Anatomy
                                        Loading...
                                        Related Differential Diagnoses
                                        Loading...
                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Chiari 2 malformation
                                                    • Arnold Chiari 2
                                                  • Definitions

                                                    • Small posterior fossa with cerebellar compression and hindbrain herniation through foramen magnum
                                                    • Almost always associated with open neural tube defect (ONTD), typically lumbar meningomyelocele

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Staging, Grading, & Classification

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Chapman T et al: Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, posterior fossa disorders. Clin Imaging. 39(2):167-175, 2015
                                                                              2. Kappou D et al: Towards detecting open spina bifida in the first trimester: the examination of the posterior brain. Fetal Diagn Ther. 37(4):294-300, 2015
                                                                              3. Mignone Philpott C et al: Diffusion-weighted imaging of the cerebellum in the fetus with Chiari II malformation. AJNR Am J Neuroradiol. 34(8):1656-60, 2013
                                                                              4. Fuchs I et al: Normal intracranial translucency and posterior fossa at 11-13 weeks' gestation in a fetus with closed spina bifida. Ultrasound Obstet Gynecol. 40(2):238-9, 2012
                                                                              5. Chaoui R et al: Prospective detection of open spina bifida at 11-13 weeks by assessing intracranial translucency and posterior brain. Ultrasound Obstet Gynecol. 38(6):722-6, 2011
                                                                              6. Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. 15(1):9-14, 2010
                                                                              7. Chaoui R et al: Assessment of intracranial translucency (IT) in the detection of spina bifida at the 11-13-week scan. Ultrasound Obstet Gynecol. 34(3):249-52, 2009
                                                                              8. Wong SK et al: Supratentorial abnormalities in the Chiari II malformationa, III: The interhemispheric cyst. J Ultrasound Med. 28(8):999-1006, 2009
                                                                              9. D'Addario V et al: Comparison of six sonographic signs in the prenatal diagnosis of spina bifida. J Perinat Med. 36(4):330-4, 2008
                                                                              10. Ando K et al: MRI tight posterior fossa sign for prenatal diagnosis of Chiari type II malformation. Neuroradiology. 49(12):1033-9, 2007
                                                                              11. Fujisawa H et al: New ultrasonographic criteria for the prenatal diagnosis of Chiari type 2 malformation. Acta Obstet Gynecol Scand. 85(12):1426-9, 2006
                                                                              12. Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus. 16(2):E5, 2004
                                                                              13. Tubbs RS et al: Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst. 20(6):375-81, 2004
                                                                              14. Adzick NS et al: Myelomeningocele: prenatal diagnosis, pathophysiology and management. Semin Pediatr Surg. 12(3):168-74, 2003
                                                                              15. Beuls E et al: The Arnold-Chiari type II malformation at midgestation. Pediatr Neurosurg. 39(3):149-58, 2003
                                                                              16. Buisson O et al: Sonographic diagnosis of spina bifida at 12 weeks: heading towards indirect signs. Ultrasound Obstet Gynecol. 19(3):290-2, 2002
                                                                              17. Coleman BG et al: Fetal therapy: state of the art. J Ultrasound Med. 21(11):1257-88, 2002
                                                                              18. Jobe AH: Fetal surgery for myelomeningocele. N Engl J Med. 347(4):230-1, 2002