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Chiari 1 malformation: age-based outcomes in a paediatric surgical cohort
Charles Yates1, Robert Campbell2, Martin Wood2
1Department of Neurosurgery, Queensland Children's Hospital, Brisbane, Queensland, Australia. Charles.Yates@health.qld.gov.au.
Insights
Younger children (0-6 years) with Chiari 1 malformation (CM1) have poorer surgical outcomes after decompression compared to older children (7-18 years). This suggests age-related anatomical differences may impact treatment effectiveness for CM1.
Area of Science:
- Neurosurgery
- Paediatric Neurology
- Congenital Malformations
Background:
- Chiari 1 malformation (CM1) presents differently in paediatric versus adult patients.
- Distinct clinical presentations are noted between very young (0-6 years) and older (7-18 years) paediatric CM1 patients.
- Limited data exists on how these age-related differences influence surgical outcomes in paediatric CM1.
Purpose of the Study:
- To determine if inter-paediatric age groups affect surgical outcomes for Chiari 1 malformation decompression.
- To compare surgical results between very young (0-6 years) and older (7-18 years) paediatric CM1 patients.
Main Methods:
- Retrospective chart review of 65 paediatric patients undergoing posterior fossa decompression (2006-2018).
- Patients stratified into two age groups: very young (0-6 years) and older (7-18 years).
- Outcomes assessed using the validated Chicago Chiari Outcome Scale (CCOS).
Main Results:
- Very young patients (n=21) showed significantly lower overall CCOS scores (12.1±3.2) than older patients (n=44) (14.2±1.6; p=0.011).
- Younger patients had worse outcomes in non-pain symptoms, functionality, and complications, and higher rates of re-operation (47.6% vs 13.6%; p=0.003).
- Very young patients presented with more oropharyngeal (38.1% vs 9.0%; p=0.014) and motor symptoms (47.6% vs 22.7%; p=0.042).
Conclusions:
- Standard posterior fossa decompression yields less favorable outcomes in very young paediatric CM1 patients (0-6 years) compared to older children (7-18 years).
- Potential underlying anatomical variations in younger children may contribute to these differing surgical responses.
- Further research into age-specific anatomical factors is warranted for optimizing CM1 surgical management in paediatric populations.
Purpose:
Substantial evidence exists describing differences between paediatric and adult Chiari 1 malformation (CM1) patients. Differences in clinical presentation between very young (0-6 years old) and older (7-18 years old) paediatric patients is similarly well-established. However, progression on these findings with regard to surgical outcomes is limited. We aimed to establish whether inter-paediatric age group modifies surgical outcome for CM1 decompression.
Methods:
Retrospective chart review was conducted for 65 patients receiving posterior fossa decompression between 2006 and 2018. Presenting features, surgical management, and outcome were evaluated and stratified into very young patients (0-6 years) or older patients (7-18 years). Outcomes were assessed using the Chicago Chiari Outcome Scale (CCOS), a validated 16-point framework for comparison.
Results:
Very young patients (21 patients) scored significantly lower in surgical outcome overall compared with older patients (44 patients) (12.1 ± 3.2/16 vs 14.2 ± 1.6/16, p = 0.011), and across 3/4 CCOS subscores: non-pain symptoms, functionality, and complications. Very young patients also returned to theatre more commonly (47.6% vs 13.6%, p = 0.003), primarily for re-do decompression (7/10 patients, 70%). Finally, the presentation of very young patients differed to older patients with significantly more oropharyngeal (38.1% vs 9.0%, p = 0.014) and motor symptoms (47.6% vs 22.7%, p = 0.042).
Discussion:
Very young patients (0-6 years) do not appear to respond as well to standard posterior fossa decompression, as their older (7-18 years) paediatric counterparts, in the absence of several baseline cohort characteristic differences. We hypothesise underlying anatomical differences may contribute to this finding.
