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 malformations are explained, radiographs are presented, and sample inpatient progress notes are shown. A story from a parent of a child with a Chiari is included to give a perspective on how this disorder can affect families.
Susan McGee and Diane Baudendistel wrote the original chapter and the 2nd edition but did not participate in this revision.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
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):993–1004
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):554–561
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):375–379
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 253–271
Dias MS (1999) Myelomeningocele. In: Choux M, DiRocco C, Hockley AD, Walker ML (eds) Pediatric neurosurgery. Churchill Livingstone, London, pp 33–59
Fric R, Eide PK (2015) Comparison of pulsatile and static pressures within the intracranial and lumbar compartments in patients with Chiari malformation type 1: a prospective observational study. Acta Neurochir 157:1411–1423
Goh S, Bottrell CL, Aiken AH, Dillon WP, Wu YW (2008) Presyrinx in children with chiari malformations. Neurology 71(5):351–356
Greenberg MS (2010) Handbook of neurosurgery, 7th edn. Thieme Medical Publishers, New York
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):637–639
Khoury C (2015) Chiari malformation. In: Patterson MC (ed) UpToDate. Wolters Kluwer Health, Philadelphia
Lazareff JA, Galarza M, Gravori T, Spinks TJ (2002) Tonsillectomy without craniectomy for the management of infantile Chiari I malformation. J Neurosurg 97:1018–1022
Menezes AH (1995) Primary craniovertebral anomalies and the hindbrain syndrome (Chiari I): data base analysis. Pediatr Neurosurg 23:260–269
Menezes AH (1999) Craniovertebral anomalies and syringomyelia. In: Choux M, DiRocco C, Hockley AD, Walker ML (eds) Pediatric neurosurgery. Churchill Livingstone, London, pp 151–184
Menezes AH (2008) Craniovertebral junction database analysis: incidence, classification, presentation, and treatment algorithms. Childs Nerv Syst 24:1101. doi:10.1007/s00381-008-0605-9
Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA (2007) Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine 7(6):601–609
Mueller DM, Oro JJ (2004) Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. J Am Acad Nurse Pract 16:134–138
Narvaro R, Olavarria G, Seshadri R, Gonzales-Portillo G, McLone DG, Tomita T (2004) Surgical results of posterior fossa decompression for patients with Chiari I malformation. Childs Nerv Syst 20:349–356
Nohria V, Oakes WJ (1991) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:222–227
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):179–187
Oakes WJ, Pugh JA, Tubbe RS (2011) Chiari malformations. In: Winn HR (ed) Youmans neurological surgery, 6th edn. Saunders, Philadelphia, pp 1918–1927
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 8:177–183
Payner TD, Prenger E, Berger TB, Crone KR (1994) Acquired Chiari malformations: incidence, diagnosis, and management. Neurosurgery 34(3):429–434
Rekate HL (2008) Natural history of the chiari type I anomaly. J Neurosurg Pediatr 2(3):177–178
Rekate HL, Wallace D (2003) Lumboperitoneal shunts in children. Pediatr Neurosurg 38:41–46
Rider T, Anderson RCE, Hankinson, TC (2015) Ventral decompression in Chiari malformation, basilar invagination, and related disorders. Neurosurg Clin N Am 26:571–578. http://dx.doi.org/10.1016/j.nec.2015.06.011 1042–3680/15/$
Schijman E (2004) History, anatomic forms, pathogenesis of Chiari I malformation. Childs Nerv Syst 20:323–328
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, 8th edn. American Association of Neurological Surgeons, Park Ridge, pp 179–183
Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HR, Maher CO (2011) Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr 8(2):205–213
Strayer A (2001) Chiari I malformation: clinical presentation & management. J Neurosurg Nurs 33(2):90–104
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):1826–1831
Tulipan N, Hernanz-Schulman M, Bruner JP (1998) Reduced hindbrain herniation after intrauterine myelomeningocele repair: a report of four cases. Pediatr Neurosurg 29(5):274–278
Tulipan N, Hernanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31(3):137–142
Weprin BE, Oakes WJ (2001) The Chiari malformations and associated syringohydromyelia. In: McClone DG (ed) Pediatric neurosurgery. WB Saunders Co, Philadelphia, pp 214–235
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 Neurosurg 105(1 Suppl Pediatrics):26–32
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 25:221–226
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing AG
About this chapter
Cite this chapter
Pownall, A.L. (2017). Chiari Malformation and Syringomyelia. In: Cartwright, C., Wallace, D. (eds) Nursing Care of the Pediatric Neurosurgery Patient. Springer, Cham. https://doi.org/10.1007/978-3-319-49319-0_6
Download citation
DOI: https://doi.org/10.1007/978-3-319-49319-0_6
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-49318-3
Online ISBN: 978-3-319-49319-0
eBook Packages: MedicineMedicine (R0)