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Chiari Malformation and Syringomyelia

  • Susan McGee
  • Diane Baudendistel
Chapter

Abstract

Chiari malformation is a developmental anomaly that is often accompanied with syringomyelia. This chapter discusses the pathophysiology, etiology, presenting symptoms, radiodiagnostic imaging, surgical management, and nursing care of children with Chiari malformation and/or syringomyelia. The different types of Chiari are explained with accompanying radiographs. Stories from parents of children with Chiari are included to give a perspective on how this disorder can affect families.

Keywords

Posterior Fossa Fourth Ventricle Chiari Malformation Cerebellar Tonsil Craniocervical Junction 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364(11):993–1004PubMedCrossRefGoogle Scholar
  2. Albert GW, Menezes AH, Hansen DR, Greenlee JD, Weinstein SL (2010) Chiari malformation type I in children younger than age 6 years: presentation and surgical outcome. J Neurosurg Pediatr 5(6):554–561PubMedCrossRefGoogle Scholar
  3. Benglis D, Covington D, Bhatia R, Bhatia S, Elhammady MS, Ragheb J et al (2011) Outcomes in pediatric patients with chiari malformation type I followed up without surgery. J Neurosurg Pediatr 7(4):375–379PubMedCrossRefGoogle Scholar
  4. Detwiler PW, Porter RW, Rekate HL (1999) Hydrocephalus-clinical features and management. In: Choux M, DiRocco C, Hockley AD, Walker ML (eds) Pediatric neurosurgery. Churchill Livingstone, London, pp 253–271Google Scholar
  5. Dias MS (1999) Myelomeningocele. In: Choux M, DiRocco C, Hockley AD, Walker ML (eds) Pediatric neurosurgery. Churchill Livingstone, London, pp 33–59Google Scholar
  6. Goh S, Bottrell CL, Aiken AH, Dillon WP, Wu YW (2008) Presyrinx in children with chiari malformations. Neurology 71(5):351–356PubMedCrossRefGoogle Scholar
  7. Greenberg MS (2010) Handbook of neurosurgery, 7th edn. Thieme Medical Publishers, New YorkGoogle Scholar
  8. Hankinson TC, Klimo P Jr, Feldstein NA, Anderson RC, Brockmeyer D (2007) Chiari malformations, syringohydromyelia and scoliosis. Neurosurg Clin N Am 18(3):549–568PubMedCrossRefGoogle Scholar
  9. Hida K, Iwasaki Y, Koyanagi I, Sawamura Y, Abe H (1995) Surgical indication & results of foremen magnum decompression versus syringosubarachnoid shunting for syringomyelia associated with Chiari I malformation. Neurosurgery 37(4):637–639Google Scholar
  10. Khoury C (2011) Chiari malformation. In: Patterson MC (ed) UpToDate. Wolters Kluwer Health, PhiladelphiaGoogle Scholar
  11. Lazareff JA, Galarza M, Gravori T, Spinks TJ (2002) Tonsillectomy without craniectomy for the management of infantile Chiari I malformation. J Neurosurg 97:1018–1022PubMedCrossRefGoogle Scholar
  12. Menezes AH (1995) Primary craniovertebral anomalies and the hindbrain syndrome (Chiari I): data base analysis. Pediatr Neurosurg 23:260–269PubMedCrossRefGoogle Scholar
  13. Menezes AH (1999) Craniovertebral anomalies and syringomyelia. In: Choux M, DiRocco C, Hockley AD, Walker ML (eds) Pediatric neurosurgery. Churchillw Livingstone, London, pp 151–184Google Scholar
  14. Narvaro R, Olavarria G, Seshadri R, Gonzales-Portillo G, McLone DG, Tomita T (2004) Surgical results of powsterior fossa decompression for patients with Chiari I malformation. Childs Nerv Syst 20:349–356CrossRefGoogle Scholar
  15. Nohria V, Oakes WJ (1991) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:222–227CrossRefGoogle Scholar
  16. Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B et al (2008) The natural history of the chiari type I anomaly. J Neurosurg Pediatr 2(3):179–187PubMedCrossRefGoogle Scholar
  17. Oakes WJ, Pugh JA, Tubbe RS (2011) Chiari malformations. In: Winn HR (ed) Youmans neurological surgery, 6th edn. Saunders, Philadelphia, pp 1918–1927Google Scholar
  18. Parker SR, Harris P, Cummings TJ, George T, Fuchs H, Grand G (2011) Complications following decompression of Chiari malformation Type I in children: dural graft or sealant? J Neurosurg Pediatr 8:177–183PubMedCrossRefGoogle Scholar
  19. Payner TD, Prenger E, Berger TB, Crone KR (1994) Acquired Chiari malformations: incidence, diagnosis, and management. Neurosurgery 34(3):429–434PubMedCrossRefGoogle Scholar
  20. Rekate HL (2008) Natural history of the chiari type I anomaly. J Neurosurg Pediatr 2(3):177–178PubMedCrossRefGoogle Scholar
  21. Rekate HL, Wallace D (2003) Lumboperitoneal shunts in children. Pediatr Neurosurg 38:41–46PubMedCrossRefGoogle Scholar
  22. Schijman E (2004) History, anatomic forms, pathogenesis of Chiari I malformation. Childs Nerv Syst 20:323–328PubMedCrossRefGoogle Scholar
  23. Sherman J, Larson JJ, Crone KR (1999) Posterior fossa decompression without dural opening for the treatment of Chiari I malformation. In: Rensachary SS, Wilkins RH (eds) Neurosurgical operative atlas, vol.8 edn. American Association of Neurological Surgeons, Park Ridge, pp 179–183Google Scholar
  24. Strayer A (2001) Chiari I malformation: clinical presentation & management. J Neurosci Nurs 33(2):90–104PubMedCrossRefGoogle Scholar
  25. Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW (1999) Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282(19):1826–1831PubMedCrossRefGoogle Scholar
  26. Tulipan N, Hernanz-Schulman M, Bruner JP (1998) Reduced hindbrain herniation after intrauterine myelomeningocele repair: a report of four cases. Pediatr Neurosurg 29(5):274–278PubMedCrossRefGoogle Scholar
  27. Tulipan N, Hernanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31(3):137–142PubMedCrossRefGoogle Scholar
  28. Weprin BE, Oakes WJ (2001) The Chiari malformations and associated syringohydromyelia. In: McClone DG (ed) Pediatric neurosurgery. WB Saunders Co, Philadelphia, pp 214–235Google Scholar
  29. Yeh DD, Koch B, Crone KR (2006) Intraoperative ultrasonography used to determine the extent of surgery necessary during posterior fossa decompression in children with chiari malformation Type I. J. Neurosurgery ( 1 Suppl Pediatrics) 105:26–32Google Scholar
  30. Yundt KD, Park TS, Tantuwaya VS, Kaufman BA (1996) Posterior fossa decompression without duraplasty in infants and young children for treatment of Chiari malformation and achondroplasia. Pediatr Neurosurg 25:221–226PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  1. 1.Developmental and Behavioral Pediatrics, Pediatric NeurosurgeryEwingUSA

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