Abstract
In McLone’s unified theory of the physiopathology of Chiari type II malformation (CM II) in myelomeningocele (MMC), CM II is the consequence of cerebrospinal fluid (CSF) leakage causing the decrease of hydrostatic pressure gradient, thus leading to cerebellar peg (CP) descent. Because of its supposed physiopathology, the cerebellar peg was thought to be irreversible. However, it has been proven that ascent of the cerebellar tonsil and even complete reversibility of a CM II after MMC repair are possible. The reversibility of this condition can be explained, thanks again to McLone’s theory: The MMC closure would restore the normal hydrodynamic forces inside the posterior fossa and the spine, which should lead to the ascent of the CP. This chapter reviews the exciting data on the reversibility of the CM II to allow the reader to better understand this phenomenon.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Cruveilhier J. Anatomie Pathologie Du Corps Humain. Paris: JB Baillière; 1829.
Goodrich JT. Ch. 1 Historical review of the surgical treatment of spina bifida. In: Özek MM, Cinalli G, Maixner WJ, editors. Spina bifida: management and outcome. Italia: Springer-Verlag; 2008.
McLone DG, Dias MS. The Chiari II malformation: cause and impact. Childs Nerv Syst. 2003;19(7–8):540–50.
McLone DG, Knepper PA. The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 1989;15(1):1–12.
Tulipan N, Hernanz-Schulman M, Lowe LH, Bruner JP. Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg. 1999;31(3):137–42.
Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, et al; MOMS Investigators. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):993–1004.
Morota N, Ihara S. Postnatal ascent of the cerebellar tonsils in Chiari malformation Type II following surgical repair of myelomeningocele. J Neurosurg Pediatr. 2008;2(3):188–93.
Hashiguchi K, Morioka T, Murakami N, Togao O, Hiwatashi A, Ochiai M, et al. Sequential morphological change of Chiari malformation type II following surgical repair of myelomeningocele. Childs Nerv Syst. 2016;32(6):1069–78.
Beuriat PA, Szathmari A, Rousselle C, Sabatier I, di Rocco F, Mottolese C. Complete reversibility of the Chiari type II malformation following post natal repair of myelomeningocele. World Neurosurg. 2017;108:62–8.
Talamonti G, D’Aliberti G, Collice M. Myelomeningocele: long-term neurosurgical treatment and follow-up in 202 patients. J Neurosurg. 2007;107(5 Suppl):368–86.
Lapras C, Guilburd JN, Patet JD. [Spina bifida aperta--myelomeningocele. Chiari’s malformation type II]. Neurochirurgie. 1988;34 Suppl 1:53–58.
Park TS, Hoffman HJ, Hendrick EB, Humphreys RP. Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele. Neurosurgery. 1983;13(2):147–52.
Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 2001;34(3):114–20.
Beuriat P-A, Poirot I, Hameury F, Szathmari A, Rousselle C, Sabatier I, et al. Postnatal management of myelomeningocele: outcome with a multidisciplinary team experience. World Neurosurg. 2018;110:e24–31.
Tulipan N, Hernanz-Schulman M, Bruner JP. Reduced hindbrain herniation after intrauterine myelomeningocele repair: a report of four cases. Pediatr Neurosurg. 1998;29(5):274–8.
Bruner JP, Tulipan N, Paschall RL, Boehm FH, Walsh WF, Silva SR, et al. Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA. 1999;282(19):1819–25.
Kabagambe SK, Jensen GW, Chen YJ, Vanover MA, Farmer DL. Fetal surgery for myelomeningocele: a systematic review and meta-analysis of outcomes in fetoscopic versus open repair. Fetal Diagn Ther. 2018;43(3):161–74.
Araujo E, Tonni G, Martins WP. Outcomes of infants followed-up at least 12 months after fetal open and endoscopic surgery for meningomyelocele: a systematic review and meta-analysis. J Evid Based Med. 2016;9(3):125–35.
Zamłyński J, Olejek A, Koszutski T, Ziomek G, Horzelska E, Gajewska-Kucharek A, et al. Comparison of prenatal and postnatal treatments of spina bifida in Poland–a non-randomized, single-center study. J Matern Fetal Neonatal Med. 2014;27(14):1409–17.
Danzer E, Finkel RS, Rintoul NE, Bebbington MW, Schwartz ES, Zarnow DM, et al. Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function. Neuropediatrics. 2008;39(6):359–62.
Moldenhauer JS, Soni S, Rintoul NE, Spinner SS, Khalek N, Martinez-Poyer J, et al. Fetal myelomeningocele repair: the post-MOMS experience at the Children’s Hospital of Philadelphia. Fetal Diagn Ther. 2015;37(3):235–40.
DAL P, Zanon N, Nishikuni K, Moreira de Sá RA, Acacio GL, Chmait RH, et al. Endoscopic surgery for the antenatal treatment of myelomeningocele: the CECAM trial. Am J Obstet Gynecol. 2016;214(1):111.e1–111.e11.
Belfort MA, Whitehead WE, Shamshirsaz AA, Bateni ZH, Olutoye OO, Olutoye OA, et al. Fetoscopic open neural tube defect repair: development and refinement of a two-port, carbon dioxide insufflation technique. Obstet Gynecol. 2017;129(4):734–43.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Beuriat, PA., Szathmari, A., Di Rocco, F., Mottolese, C. (2020). Chiari Type II Malformation: Reversibility Following Myelomeningocele Closure. In: Tubbs, R., Turgut, M., Oakes, W. (eds) The Chiari Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_40
Download citation
DOI: https://doi.org/10.1007/978-3-030-44862-2_40
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-44861-5
Online ISBN: 978-3-030-44862-2
eBook Packages: MedicineMedicine (R0)