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Chiari malformation type I surgery in children: French multicenter 10-year cohort
Edouard Mazerand1, Sandro Benichi2, Maxime Taverne3
11Department of Neurosurgery, Angers University Hospital, Angers.
Insights
This study identified three distinct subgroups of pediatric patients with Chiari malformation type I (CM-I) based on anatomical similarities. This classification aids in understanding CM-I mechanisms and personalizing surgical treatment strategies.
Area of Science:
- Neurosurgery
- Pediatric Neurosurgery
- Medical Research
Background:
- Chiari malformation type I (CM-I) is a common pediatric condition posing surgical challenges.
- Current treatment strategies for CM-I lack a validated decision algorithm, leading to institutional variability.
- Patient specificities are crucial for defining effective therapeutic guidelines.
Purpose of the Study:
- To define therapeutic guidelines for pediatric Chiari malformation type I (CM-I) by considering patient specificities.
- To identify distinct patient subgroups based on anatomical characteristics.
- To correlate patient subgroups with surgical outcomes.
Main Methods:
- Retrospective data collection from 255 pediatric patients (<18 years) surgically treated for CM-I between 2008-2018 across 8 French centers.
- Analysis of clinical features, morphological parameters, and surgical techniques.
- Hierarchical clustering to identify anatomical subgroups and comparison of surgical outcomes using the Chicago Chiari Outcome Scale.
Main Results:
- Three distinct subgroups of CM-I patients were identified: bony compression, basilar invagination, and foramen magnum obstruction.
- Syringomyelia was present in 60.2% of patients; 17.3% required re-operation.
- The identified subgroups correlated with specific clinical outcomes, suggesting distinct underlying mechanisms.
Conclusions:
- A morphological classification of pediatric CM-I patients into three subgroups was established.
- This classification aids in understanding the mechanisms of tonsillar herniation.
- Personalized treatment approaches for CM-I may be facilitated by this morphological stratification.
Objective:
Chiari malformation type I (CM-I) is frequent in children and remains a surgical challenge. Several techniques have been described for posterior fossa decompression. No decision algorithm has been validated, and strategies are highly variable between institutions. The goal of this study was to define therapeutic guidelines that take into consideration patient specificities.
Methods:
The authors retrospectively collected data from patients who were < 18 years of age, were diagnosed with CM-I, and were treated surgically between 2008 and 2018 in 8 French pediatric neurosurgical centers. Data on clinical features, morphological parameters, and surgical techniques were collected. Clinical outcomes at 3 and 12 months after surgery were assessed by the Chicago Chiari Outcome Scale. The authors used a hierarchical clustering method to define clusters of patients by considering their anatomical similarities, and then compared outcomes between surgical strategies in each of these clusters.
Results:
Data from 255 patients were collected. The mean age at surgery was 9.6 ± 5.0 years, syringomyelia was reported in 60.2% of patients, the dura mater was opened in 65.0% of patients, and 17.3% of patients underwent a redo surgery for additional treatment. The mean Chicago Chiari Outcome Scale score was 14.4 ± 1.5 at 3 months (n = 211) and 14.6 ± 1.9 at 12 months (n = 157). The hierarchical clustering method identified three subgroups with potentially distinct mechanisms underlying tonsillar herniation: bony compression, basilar invagination, and foramen magnum obstruction. Each cluster matched with specific outcomes.
Conclusions:
This French multicenter retrospective cohort study enabled the identification of three subgroups among pediatric patients who underwent surgery for CM-I, each of which was associated with specific outcomes. This morphological classification of patients might help in understanding the underlying mechanisms and providing personalized treatment.
