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Chiari I malformation: management evolution and technical innovation

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Abstract

Background and definition

In recent years thanks to the growing use of radiological assessment, Chiari I malformation became one of the major diseases for a neurosurgeon to deal with. CIM can be classified according to the extent of cerebellar tonsil tip into the foramen magnum being a protrusion over five mm considered pathological. Such a disease is a heterogeneous condition with a multifactorial pathogenetic mechanism that can subdivided into a primary and secondary form. Regardless of the form, it seems that CIM is the result of an imbalance between the volume of the braincase and its content. Acquired CIMs are secondary to conditions causing intracranial hypertension or hypotension while the pathogenesis of primary forms is still controversial.

Pathogenesis and treatment

There are several theories in the literature but the most accepted one implies an overcrowding due to a small posterior cranial fossa. While asymptomatic CIM do not need treatment, symptomatic ones prompt for surgical management. Several techniques are proposed being the dilemma centered in the need for dural opening procedures and bony decompression ones.

Conclusion

Alongside the paper, the authors will address the novelty presented in the literature on management, diagnosis and pathogenesis in order to offer a better understanding of such a heterogeneous pathology.

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Data availability

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Abbreviations

CIM:

Chiari I malformation

Cine-PCMR:

Cardiac gated cine phase-contrast MR

CVJ:

Cranio-vertebral junction

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Authors and Affiliations

  1. Pediatric Neurosurgery, Fondazione Policlinico Gemelli IRCCS, Rome, Italy

    Federico Bianchi, Paolo Frassanito, Luca Massimi & Gianpiero Tamburrini

  2. Università Cattolica del Sacro Cuore, Rome, Italy

    Benedetta Montedoro, Luca Massimi & Gianpiero Tamburrini

Authors
  1. Federico Bianchi
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  2. Benedetta Montedoro
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  3. Paolo Frassanito
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  4. Luca Massimi
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  5. Gianpiero Tamburrini
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Contributions

Federico Bianchi wrote the majority of the paper and edited the main manuscript test. Benedetta Montedoro was in charge of classification and pathogenesis. Paolo Frassanito edited the photo. Luca Massimi and Gianpiero Tamburrini supervised the paper and were the main surgeon for the reported patients.

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Correspondence to Federico Bianchi.

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Bianchi, F., Montedoro, B., Frassanito, P. et al. Chiari I malformation: management evolution and technical innovation. Childs Nerv Syst 39, 2757–2769 (2023). https://doi.org/10.1007/s00381-023-06051-7

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