Decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry

Restricted access

OBJECTIVE

The purpose of this study was to determine the rate of decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in rates of Chiari II decompression across NSBPR institutions, examined the relationship between Chiari II decompression and functional lesion level of the myelomeningocele, age, and need for tracheostomy, and they evaluated for temporal trends in rates of Chiari II decompression.

METHODS

The authors queried the NSBPR to identify all individuals with myelomeningocele between 2009 and 2015. Among these patients, they identified individuals who had undergone at least 1 Chiari II decompression as well as those who had undergone tracheostomy. For each participating NSBPR institution, the authors calculated the proportion of patients enrolled at that site who underwent Chiari II decompression. Logistic regression was performed to analyze the relationship between Chiari II decompression, functional lesion level, age at decompression, and history of tracheostomy.

RESULTS

Of 4448 individuals with myelomeningocele identified from 26 institutions, 407 (9.15%) had undergone at least 1 Chiari II decompression. Fifty-one patients had undergone tracheostomy. Logistic regression demonstrated a statistically significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, with a more rostral lesion level associated with a higher likelihood of posterior fossa decompression. Similarly, children born before 2005 and those with history of tracheostomy had a significantly higher likelihood of Chiari II decompression. There was no association between functional lesion level and need for tracheostomy. However, among those children who underwent Chiari II decompression, the likelihood of also undergoing tracheostomy increased significantly with younger age at decompression.

CONCLUSIONS

The rate of Chiari II decompression in patients with myelomeningocele in the NSBPR is consistent with that in previously published literature. There is a significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, which has not previously been reported. Younger children who undergo Chiari II decompression are more likely to have undergone tracheostomy. There appears to be a shift away from Chiari II decompression, as children born before 2005 were more likely to undergo Chiari II decompression than those born in 2005 or later.

ABBREVIATIONS CDC = Centers for Disease Control and Prevention; NSBPR = National Spina Bifida Patient Registry.
Article Information

Contributor Notes

Correspondence Brandon G. Rocque: University of Alabama at Birmingham, AL. brandon.rocque@childrensal.org.INCLUDE WHEN CITING Published online August 24, 2018; DOI: 10.3171/2018.5.PEDS18160.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

Headings
References
  • 1

    Akbari SHLimbrick DD JrKim DHNarayan PLeonard JRSmyth MD: Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst 29:114311542013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Bowman RMMcLone DGGrant JATomita TIto JA: Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 34:1141202001

  • 3

    Charney EBRorke LBSutton LNSchut L: Management of Chiari II complications in infants with myelomeningocele. J Pediatr 111:3643711987

  • 4

    Kim IHopson BAban IRizk EBDias MSBowman R: Treated hydrocephalus in individuals with myelomeningocele in the National Spina Bifida Patient Registry. J Neurosurg Pediatr [epub ahead of print August 24 2018. DOI: 10.3171/2018.5.PEDS18161]

    • Search Google Scholar
    • Export Citation
  • 5

    McLone DG: Results of treatment of children born with a myelomeningocele. Clin Neurosurg 30:4074121983

  • 6

    Messing-Jünger MRöhrig A: Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 29:155315622013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Park TSHoffman HJHendrick EBHumphreys RP: Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele. Neurosurgery 13:1471521983

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Pollack IFPang DAlbright ALKrieger D: Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77:8818881992

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Sawin KJLiu TWard EThibadeau JSchechter MSSoe MM: The National Spina Bifida Patient Registry: profile of a large cohort of participants from the first 10 clinics. J Pediatr 166:444450 450.e1 2015

    • Search Google Scholar
    • Export Citation
  • 10

    Stevenson KL: Chiari Type II malformation: past, present, and future. Neurosurg Focus 16(2):E52004

  • 11

    Talamonti GZella S: Surgical treatment of CM2 and syringomyelia in a series of 231 myelomeningocele patients. Neurol Sci 32 (Suppl 3):S331S3332011

    • Search Google Scholar
    • Export Citation
  • 12

    Thibadeau JKWard EASoe MMLiu TSwanson MSawin KJ: Testing the feasibility of a National Spina Bifida Patient Registry. Birth Defects Res A Clin Mol Teratol 97:36412013

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 13

    Tubbs RSOakes WJ: Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20:3753812004

  • 14

    Vandertop WPAsai AHoffman HJDrake JMHumphreys RPRutka JT: Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg 77:5415441992

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
TrendMD
Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 389 389 49
Full Text Views 129 129 5
PDF Downloads 145 145 5
EPUB Downloads 0 0 0
PubMed
Google Scholar