Chiari malformation type I surgery in children: French multicenter 10-year cohort

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  • 1 Department of Neurosurgery, Angers University Hospital, Angers;
  • | 2 Department of Pediatric Neurosurgery, Necker–Enfants Malades Hospital, Assistance Publique—Hôpitaux de Paris;
  • | 3 Craniofacial Growth and Form, Necker–Enfants Malades Hospital, Assistance Publique—Hôpitaux de Paris;
  • | 4 Department of Pediatric Neurosurgery, Montpellier University Hospital, Montpellier;
  • | 5 Department of Neurosurgery, Toulouse University Hospital, Toulouse;
  • | 6 Department of Neurosurgery, Strasbourg University Hospital, Strasbourg;
  • | 7 Department of Pediatric Neurosurgery, Bordeaux University Hospital, Bordeaux;
  • | 8 Department of Neurosurgery, Rouen University Hospital, Rouen;
  • | 9 Department of Neurosurgery, Rennes University Hospital, Rennes;
  • | 10 Department of Neurosurgery, French Reference Center for Chiari and Rare Vertebral and Medullary Malformations (C-MAVEM), Montpellier University Hospital, Montpellier;
  • | 11 Department of Pediatric Maxillofacial and Plastic Surgery, Necker–Enfants Malades Hospital, Assistance Publique—Hôpitaux de Paris; and
  • | 12 Department of Pediatric Neurosurgery, CHU Timone Enfant, Marseille, France
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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.

ABBREVIATIONS

CCOS = Chicago Chiari Outcome Scale; CM-I = Chiari malformation type I; FM = foramen magnum; HCPC = hierarchical clustering on PCs; pB-C2 = perpendicular distance to the line from the basion to the posterior and inferior point of the C2 body; PC = principal component; PCA = PC analysis; TH = tonsillar herniation.

Images from Oushy et al. (pp 195–202).

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