Management of Chiari I malformations: a paradigm in evolution

Abstract

Purpose

Despite decades of experience and research, the etiology and management of Chiari I malformations (CM-I) continue to raise more questions than answers. Controversy abounds in every aspect of management, including the indications, timing, and type of surgery, as well as clinical and radiographic outcomes. This review aims to outline past experiences, consolidate current evidence, and recommend directions for the future management of the Chiari I malformation.

Methods

A review of recent literature on the management of CM-I in pediatric patients is presented, along with our experience in managing 1073 patients who were diagnosed with CM-I over the past two decades (1998–2018) at Children’s National Medical Center (CNMC) in Washington DC.

Results

The general trend reveals an increase in the diagnosis of CM-I at younger ages with a significant proportion of these being incidental findings (0.5–3.6%) in asymptomatic patients as well as a rise in the number of patients undergoing Chiari posterior fossa decompression surgery (PFD). The type of surgical intervention varies widely. At our institution, 104 (37%) Chiari surgeries were bone-only PFD with/without outer leaf durectomy, whereas 177 (63%) were PFD with duraplasty. We did not find a significant difference in outcomes between the PFD and PFDD groups (p = 0.59). An analysis of failures revealed a significant difference between patients who underwent tonsillar coagulation versus those whose tonsils were not manipulated (p = 0.02).

Conclusion

While the optimal surgical intervention continues to remain elusive, there is a shift away from intradural techniques in favor of a simple, extradural approach (including dural delamination) in pediatric patients due to high rates of clinical and radiographic success, along with a lower complication rate. The efficacy, safety, and necessity of tonsillar manipulation continue to be heavily contested, as evidence increasingly supports the efficacy and safety of less tonsillar manipulation, including our own experience.

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Acknowledgements

The authors would like to acknowledge the support of the American Syringomyelia and Chiari Alliance Project for grant support in the above clinical research at CNMC.

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Correspondence to Robert F. Keating.

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Alexander, H., Tsering, D., Myseros, J.S. et al. Management of Chiari I malformations: a paradigm in evolution. Childs Nerv Syst 35, 1809–1826 (2019). https://doi.org/10.1007/s00381-019-04265-2

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Keywords

  • Chiari malformation
  • Syringomyelia
  • Long-term outcomes
  • Decompression
  • Duraplasty
  • Tonsillar coagulation
  • Shunt
  • Pediatric