Clinicoradiographic data and management of children with Chiari malformation type 1 and 1.5: an Italian case series.

ABSTRACT

INTRODUCTION: The widespread use of imaging has increased Chiari malformation (CM) diagnosis. CM shows clinical heterogeneity that makes management controversial. We aimed to evaluate the occurrence and clinical and radiographic presentation of children with CM-1 and CM-1.5, reporting possible differences according to age and management. METHODS: We retrospectively reviewed 46 children diagnosed with CM-1 or CM-1.5, between 2006 and 2019 at our institute. We evaluated for each subject reason for hospital admission, clinical presentation, age at diagnosis, extent of cerebellar tonsillar herniation (CTH) and type of treatment when carried out. Affected children were assigned to three age groups. In some patients, a clinical follow-up was carried out. RESULTS: Mean age at diagnosis was 7.61 years. Mean CTH was 8.72 mm. Syringomyelia was found in 10.9%. Twenty-six individuals (56.5%) were symptomatic. The most frequent symptom was headache (34.8%). There were no statistically significant differences between the age groups with regard to the amount of CTH (p = 0.81). Thirteen children (28.3%) underwent surgical treatment. CTH was significantly higher in the surgical group (p < 0.01). Twenty-three patients (50%) performed a 3-year mean follow-up, 17 of whom had no surgery treatment. CTH was stable in 58.8%, reduced in three and increased in three, without any change in symptoms. Only one child showed a worsening in herniation and symptoms, then requiring surgery. CONCLUSION: Frequency and type of symptoms were consistent with those reported in the literature. Conservative approach is a viable option for minimally symptomatic patients, most of whom did not show clinical worsening at follow-up.