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

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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.

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Acknowledgements

The authors wish to thank American Manuscript Editors (USA) for editing the manuscript.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Policlinico G. Rodolico, Via Santa Sofia 78, 95123, Catania, Italy

    Alessandro Giallongo, Piero Pavone & Federica Filosco

  2. Neonatal Intensive Care Unit, Umberto I Hospital, 96100, Siracusa, Italy

    Stefania Piera Tomarchio

  3. Pediatric Emergency Department, University Hospital “Policlinico-Vittorio Emanuele”, Catania, Italy

    Raffaele Falsaperla

  4. Department of General Surgery and Medical Surgical Specialties, Section of Orthopaedics and Traumatologic Surgery, University of Catania, 95123, Catania, Italy

    Gianluca Testa & Vito Pavone

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  1. Alessandro Giallongo
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  3. Stefania Piera Tomarchio
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Correspondence to Piero Pavone.

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The study conforms to The Code of Ethics of the World Medical Association (Declaration of Helsinki). This is a retrospective study and no free informed consent form was needed, and subject’s identity was not divulged.

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Giallongo, A., Pavone, P., Tomarchio, S.P. et al. Clinicoradiographic data and management of children with Chiari malformation type 1 and 1.5: an Italian case series. Acta Neurol Belg 121, 1547–1554 (2021). https://doi.org/10.1007/s13760-020-01398-z

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