Skip to main content
SpringerLink
Log in

Chiari Malformation

  • Reference work entry
  • First Online:
Book cover Atlas of Genetic Diagnosis and Counseling

  • 144 Accesses

Abstract

In 1891, Chiari first described a dysplasia of the nervous system consisting of herniation of the cerebellar tonsils into the foramen magnum. There are three types of Chiari malformations. The commonest ones are type I and type II, and the latter has been named the Arnold-Chiari malformation (De Reuck and Theinpont 1976).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Ando, K., Ishikura, R., Ogawa, M., et al. (2007). MRI tight posterior fossa sign for prenatal diagnosis of Chiari type II malformation. Neuroradiology, 49, 1033–1039.

    Article  PubMed  Google Scholar 

  • Aribal, M. E., Gurcan, F., & Aslan, B. (1996). Chiari III malformation: MRI. Neuroradiology, 38, 184–186.

    Article  Google Scholar 

  • Bindal, A. K., Dunsker, S. B., & Tew, J. M. (1995). Chiari I malformation: Classification and management. Neurosurgery, 37, 1069–1074.

    Article  CAS  PubMed  Google Scholar 

  • Caldarelli, M., Rea, G., Cincu, R., et al. (2002). Chiari type III malformation. Child’s Nervous System, 18, 207–210.

    Article  PubMed  Google Scholar 

  • Castillo, M., Quencer, R. M., & Dominquez, R. (1992). Chiari III malformation: Imaging features. AJNR. American Journal of Neuroradiology, 13, 107–113.

    CAS  PubMed  Google Scholar 

  • Cesmebasi, A., Loukas, M., Hogan, E., et al. (2015). The Chiari malformations: A review with emphasis on anatomical traits. Clinical Anatomy, 28, 184–194.

    Article  PubMed  Google Scholar 

  • Chapman, T., Mahalingam, S., Ishak, G. E., et al. (2015). Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: Part 2, posterior fossa disorders. Clinical Imaging, 39, 167–175.

    Article  PubMed  Google Scholar 

  • Dauser, R. C., Dipietro, M. A., & Venes, J. L. (1988). Symptomatic Chiari I malformation in childhood: A report of 7 cases. Pediatric Neuroscience, 14, 184–190.

    Article  CAS  PubMed  Google Scholar 

  • De Reuck, J., & Theinpont, L. (1976). Fetal Chiari’s type III malformation. Child’s Brain, 2, 85–91.

    PubMed  Google Scholar 

  • Di Rocco, C., Frassanito, P., Massimi, L., et al. (2011). Hydrocephalus and Chiari type I malformation. Childs Nervous System, 27, 1653–1664.

    Article  Google Scholar 

  • Dure, L. S., Percy, A. K., Cheek, W. R., et al. (1989). Chiari type I malformation in children. Journal of Pediatrics, 115, 573–576.

    Article  CAS  PubMed  Google Scholar 

  • Dyste, G. N., Menezes, A. H., & Van Gildrer, J. C. (1989). Symptomatic Chiari malformations. Journal of Neurosurgery, 71, 159–168.

    Article  CAS  PubMed  Google Scholar 

  • El Gammal, T., Mark, E. K., & Brooks, B. S. (1988). MR imaging of Chiari II malformation. AJNR. American Journal of Neuroradiology, 35, 1037–1044.

    Google Scholar 

  • Elster, A. D., & Chen, M. Y. M. (1992). Chiari I malformations: Clinical and radiologic reappraisal. Radiology, 183, 347–353.

    Article  CAS  PubMed  Google Scholar 

  • Genitori, L., Peretta, P., Nurisso, C., et al. (2000). Chiari type I anomalies in children and adolescents: Minimally invasive management in a series of 53 cases. Child’s Nervous System, 16, 707–718.

    Article  CAS  PubMed  Google Scholar 

  • Häberle, J., Hülskamp, G., Harms, E., et al. (2001). Cervical encephalocele in a newborn – Chiari III malformation. Case report and review of the literature. Child’s Nervous System, 17, 373–375.

    Article  PubMed  Google Scholar 

  • Iruretagoyena, J. I., Trampe, B., & Shah, D. (2010). Prenatal diagnosis of Chiari malformation with syringomyelia in the second trimester. The Journal of Maternal-Fetal & Neonatal Medicine, 23, 184–186.

    Article  Google Scholar 

  • Iskandar, B., & Oakes, W. (1999). Chiari malformation and syringomyelia. In L. Albright, I. Pollack, & P. Adelson (Eds.), Principles and Practice of Pediatric Neurosurgery (pp. 165–187). New York: Thieme.

    Google Scholar 

  • Ivashchuk, G., Loukas, M., Blount, J. P., et al. (2015). Chiari III malformation: A comprehensive review of this enigmatic anomaly. Childs Nervous System, Aug 9. [Epub ahead of print]

    Google Scholar 

  • Koehler, J., Schwarz, M., Urban, P. P., et al. (2001). Masseter reflex and blink reflex abnormalities in Chiari II malformation. Muscle & Nerve, 24, 425–427.

    Article  CAS  Google Scholar 

  • Loukas, M., Shayota, B. J., Oelhafen, K., et al. (2011). Associated disorders of Chiari type I malformations: a review. Neurosurgery Focus, 31, E3–E8.

    Google Scholar 

  • McVige, J. W., & Leonardo, J. (2014). Imaging of Chiari type I malformation and syringohydromeyelia. Neurology clinic, 32, 95–126.

    Google Scholar 

  • Milhorat, T. H., Chou, M. W., Trinidad, E. M., et al. (1999). Chiari I malformation redefined: Clinical and radiographic findings for 364 symptomatic patients. Neurosurgery, 44, 1005–1017.

    Article  CAS  PubMed  Google Scholar 

  • Nagib, M. G. (1994). An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatric Neurosurgery, 21, 31–35.

    Article  CAS  PubMed  Google Scholar 

  • Naidich, T. P. (1981). Cranial CT signs of the Chiari II malformation. Journal of Neuroradiology, 8, 207–227.

    CAS  Google Scholar 

  • Naya Galvez, M. J., Fraile Rodrigo, J. J., Liesa, R. F., et al. (2002). Otorhinolaryngologic manifestations in Chiari malformation. American Journal of Otolaryngology, 23, 99–104.

    Article  PubMed  Google Scholar 

  • Paul, K. S., Lye, R. H., Strang, F. A., et al. (1983). Arnold-Chiari malformation. Review of 71 cases. Journal of Neurosurgery, 58, 183–187.

    Article  CAS  PubMed  Google Scholar 

  • Rauzzino, M., & Oakes, W. J. (1995). Chiari II malformation and syringomyelia. Neurosurgery Clinics of North America, 6, 293–309.

    CAS  PubMed  Google Scholar 

  • Righini, A., Parazzini, C., Doneda, C., et al. (2011). Fetal MRI features related to the Chiari malformations. Neurological Science, 32(Suppl. 3), S279–S281.

    Article  Google Scholar 

  • Schanker, B. D., Walcott, B. P., Nahed, B. V., et al. (2011). Familial Chiari malformation: Case series. Neurosurgical Focus, 31, E1–E6.

    Article  PubMed  Google Scholar 

  • Siasios, J., Kapsalaki, E. Z., & Fountas, K. N. (2012). Surgical management of patients with Chiari I malformation. International Journal of Pediatrics, 2012, 1–10.

    Article  Google Scholar 

  • Smith, A. B., Gupta, N., Otto, C., et al. (2007). Diagnosis of Chiari III malformation by second trimester fetal MRI with postnatal MRI and CT correlation. Pediatric Radiology, 37, 1035–1038.

    Article  PubMed  Google Scholar 

  • Thomas, M., Mike, C., Elizabeth, T., et al. (1999). Chiari I malformation redefined: Clinical and radiographic findings for 364 symptomatic patients. Neurosurgery, 44, 1005–1017.

    Article  Google Scholar 

  • Ventureyra, E. C., Aziz, H. A., & Vassilyadi, M. (2003). The role of cine flow MRI in children with Chiari I malformation. Child’s Nervous System, 19, 109–113.

    PubMed  Google Scholar 

  • Weinberg, J. S., Freed, D. L., Sadock, J., et al. (1998). Headache and Chiari I malformation in the pediatric population. Pediatric Neurosurgery, 29, 14–18.

    Article  CAS  PubMed  Google Scholar 

  • Wellons, J. C., III, Tubbs, R. S., & Oakes, W. J. (2005). Chiari malformations and syringohydromyelia. In S. S. Rengachary & R. G. Ellenbogen (Eds.), Principles of neurosurgery (pp. 181–195). Edinburgh: Elsevier Mosby.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Medical Genetics, Shriners Hospitals for Children, Perinatal and Clinical Genetics, Department of Pediatrics, LSU Health Sciences Center, Shreveport, LA, USA

    Harold Chen

Authors
  1. Harold Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer Science+Business Media LLC

About this entry

Cite this entry

Chen, H. (2017). Chiari Malformation. In: Atlas of Genetic Diagnosis and Counseling. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2401-1_40

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-2401-1_40

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-2400-4

  • Online ISBN: 978-1-4939-2401-1

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation