Skip to main content
SpringerLink
Log in

External validity of the chiari severity index and outcomes among pediatric chiari I patients treated with intra- or extra-Dural decompression

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Introduction

Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI.

Methods

We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children’s hospital. Characteristics of headache, syrinx, and myelopathy were collected to derive CSI grade. The primary outcome measure was pre-operative symptom resolution. The proportion of patients with favorable outcome was tabulated for each of the three CSI grades and compared to previously published results.

Results

From 2004 to 2014, 189 patients underwent ID (48%) or ED (52%) decompression at the Children’s Hospital of Philadelphia (CHOP). Follow-up ranged from 1 to 75 months. Rates of symptom resolution (58–64%) and reoperation (8%) were similar regardless of surgical approach. Although proportions of favorable outcomes differed between the CHOP and Washington University (WU) cohorts, the difference was not related to CSI grade (p = 0.63). Furthermore, there was no difference in the proportion of favorable outcomes between the two cohorts regardless of ID (p = 0.26) or ED approach (p = 0.11).

Conclusions

Equivalent rates of symptom resolution and reoperation following ID and ED decompression support the ED approach as a first-line surgical option for pediatric CM-1 patients. In addition, our findings provide preliminary evidence supporting the generalizability of the CSI and its use in future comparative trials.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Abbreviations

CHOP:

Children’s Hospital of Philadelphia

CM-1:

Chiari I malformation

CSF:

Cerebrospinal fluid

CSI:

Chiari severity index

ED:

Extradural

ID:

Intradural

MRI:

Magnetic resonance imaging

QOL:

Quality of life

WU:

Washington University

References

  1. Aboulezz AO, Sartor K, Geyer CA, Gado MH (1985) Position of cerebellar tonsils in the normal population and in patients with chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr 9:1033–1036

    Article  CAS  PubMed  Google Scholar 

  2. Kahn EN, Muraszko KM, Maher CO (2015) Prevalence of chiari I malformation and syringomyelia. Neurosurg Clin N Am 26:501–507. doi:10.1016/j.nec.2015.06.006

    Article  PubMed  Google Scholar 

  3. Durham SR, Fjeld-Olenec K (2008) Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of chiari malformation type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr 2:42–49. doi:10.3171/PED/2008/2/7/042

    Article  PubMed  Google Scholar 

  4. Hankinson T, Tubbs RS, Wellons JC (2011) Duraplasty or not? An evidence-based review of the pediatric chiari I malformation. Childs Nerv Syst 27:35–40. doi:10.1007/s00381-010-1295-7

    Article  PubMed  Google Scholar 

  5. Greenberg JK, Yarbrough CK, Radmanesh A, Godzik J, Yu M, Jeffe DB, Smyth MD, Park TS, Piccirillo JF, Limbrick DD (2015) The chiari severity index: a preoperative grading system for chiari malformation type 1. Neurosurgery 76:279–285 . doi:10.1227/NEU.0000000000000608discussion 285

    Article  PubMed  PubMed Central  Google Scholar 

  6. Galarza M, Sood S, Ham S (2007) Relevance of surgical strategies for the management of pediatric chiari type I malformation. Childs Nerv Syst 23:691–696. doi:10.1007/s00381-007-0297-6

    Article  PubMed  Google Scholar 

  7. Munshi I, Frim D, Stine-Reyes R, Weir B, Hekmatpanah J, Brown F (2000) Effects of posterior fossa decompression with and without duraplasty on chiari malformation-associated hydromyelia. Neurosurgery 46:1384–1390

    Article  CAS  PubMed  Google Scholar 

  8. Navarro 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. doi:10.1007/s00381-003-0883-1

    Article  PubMed  Google Scholar 

  9. Kennedy BC, Kelly KM, Phan MQ, Bruce SS, McDowell MM, Anderson RCE, Feldstein NA (2015) Outcomes after suboccipital decompression without dural opening in children with chiari malformation type I. J Neurosurg Pediatr 16:150–158. doi:10.3171/2014.12.PEDS14487

    Article  PubMed  PubMed Central  Google Scholar 

  10. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC, Rozzelle CJ, Blount JP, Oakes J (2011) Institutional experience with 500 cases of surgically treated pediatric chiari malformation type I. J Neurosurg Pediatr 7:248–256. doi:10.3171/2010.12.PEDS10379

    Article  PubMed  Google Scholar 

  11. Lee A, Yarbrough CK, Greenberg JK, Barber J, Limbrick DDD, Smyth MD (2014) Comparison of posterior fossa decompression with or without duraplasty in children with type I chiari malformation. Childs Nerv Syst 30:1419–1424. doi:10.1007/s00381-014-2424-5

    Article  PubMed  PubMed Central  Google Scholar 

  12. Anderson RCE, Emerson RG, Dowling KC, Feldstein NA (2003) Improvement in brainstem auditory evoked potentials after suboccipital decompression in patients with chiari I malformations. J Neurosurg 98:459–464. doi:10.3171/jns.2003.98.3.0459

    Article  PubMed  Google Scholar 

  13. Zamel K, Galloway G, Kosnik EJ, Rasian M, Adeli A (2009) Intraoperative neurophysiologic monitoring in 80 patients with chiari I malformation: role of duraplasty. J Clin Neurophysiol 26:70–75. doi:10.1097/WNP.0b013e31819f9058

    Article  PubMed  Google Scholar 

  14. Chotai S, Medhkour A (2014) Surgical outcomes after posterior fossa decompression with and without duraplasty in chiari malformation-I. Clin Neurol Neurosurg 125:182–188. doi:10.1016/j.clineuro.2014.07.027

    Article  PubMed  Google Scholar 

  15. Gurbuz MS, Karaaslan N, Caliskan T, Unai E, Berkman MZ (2015) Comparison of the surgical results for foramen magnum decompression with and without duraplasty in chiari malformation type 1. Turk Neurosurg 25:419–424. doi:10.5137/1019-5149.JTN.11235-14.1

    PubMed  Google Scholar 

  16. Rocque BG, George TM, Kestle J, Iskandar BJ (2011) Treatment practices for chiari malformation type I with syringomyelia: results of a survey of the American Society of Pediatric Neurosurgeons. J Neurosurg Pediatr 8:430–437. doi:10.3171/2011.8.PEDS10427

    Article  PubMed  Google Scholar 

  17. Mutchnick IS, Janjua RM, Moeller K, Moriarty TM (2010) Decompression of chiari malformation with and without duraplasty: morbidity versus recurrence. J Neurosurg Pediatr 5:474–478. doi:10.3171/2010.1.PEDS09218

    Article  PubMed  Google Scholar 

  18. Caldarelli M, Novegno F, Massimi L, Romani R, Tamburrini G, DiRocco C (2007) The role of limited posterior fossa craniectomy in the surgical treatment of chiari malformation type I: experience with a pediatric series. J Neurosurg 106:187–195. doi:10.3171/ped.2007.106.3.187

    PubMed  Google Scholar 

  19. 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 105:26–32. doi:10.3171/ped.2006.105.1.26

    Article  PubMed  Google Scholar 

  20. Bao C, Yang F, Liu L, Wang B, Li D, Gu Y, Zhang S, Chen L (2013) Surgical treatment of chiari I malformation complicated with syringomyelia. Exp Ther Med 5:333–337. doi:10.3892/etm.2012.784

    PubMed  Google Scholar 

  21. Depreitere B, Van Calenbergh F, van Loon J, Goffin J, Plets C (2000) Posterior fossa decompression in syringomyelia associated with a chiari malformation: a retrospective analysis of 22 patients. Clin Neurol Neurosurg 102:91–96

    Article  CAS  PubMed  Google Scholar 

  22. Litvack ZN, Lindsay RA, Selden NR (2013) Dura splitting decompression for chiari I malformation in pediatric patients: clinical outcomes, healthcare costs, and resource utilization. Neurosurgery 72:922–929. doi:10.1227/NEU.0b013e31828ca1ed

    Article  PubMed  Google Scholar 

  23. Limonadi FM, Selden NR (2004) Dura-splitting decompression of the craniocervical junction: reduced operative time, hospital stay, and cost with equivalent early outcome. J Neurosurg 101:184–188. doi:10.3171/ped.2004.101.2.0184

    PubMed  Google Scholar 

  24. Greenberg JK, Milner E, Yarbrough CK, Lipsey K, Piccirilo JF, Smyth MD, Park TS (2015) Outcome methods used in clinical studies of chiari malformation type I: a systematic review. J Neurosurg 122:262–272. doi:10.3171/2014.9.JNS14406

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, University of Pennsylvania, 3400 Spruce Street, 3rd Floor Silverstein Pavilion, Philadelphia, PA, 19104, USA

    Jared M . Pisapia, Maxwell B. Merkow, Danielle Brewington, Leslie N. Sutton, Phillip B. Storm & Gregory G. Heuer

  2. Division of Neurosurgery, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Rosemary E. Henn, Leslie N. Sutton, Phillip B. Storm & Gregory G. Heuer

Authors
  1. Jared M . Pisapia
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Maxwell B. Merkow
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Danielle Brewington
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rosemary E. Henn
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Leslie N. Sutton
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Phillip B. Storm
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Gregory G. Heuer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jared M . Pisapia.

Ethics declarations

Funding source

No external funding was secured for this study.

Financial disclosure

The authors have no financial relationships relevant to this article to disclose.

Conflict of interest

The authors have no conflicts of interest to disclose.

Additional information

Jared M. Pisapia and Maxwell B. Merkow contributed equally

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pisapia, J.M..., Merkow, M.B., Brewington, D. et al. External validity of the chiari severity index and outcomes among pediatric chiari I patients treated with intra- or extra-Dural decompression. Childs Nerv Syst 33, 313–320 (2017). https://doi.org/10.1007/s00381-016-3300-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-016-3300-2

Keywords

Search

Navigation