4.1 Article

Chiari I malformation: management evolution and technical innovation

Journal

CHILDS NERVOUS SYSTEM
Volume -, Issue -, Pages -

Publisher

SPRINGER
DOI: 10.1007/s00381-023-06051-7

Keywords

Chiari; Malformation; Syringomyelia; Management

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Thanks to the growing use of radiological assessment in recent years, Chiari I malformation has become a major disease for neurosurgeons to deal with. It can be classified based on the extent of protrusion of the cerebellar tonsil tip into the foramen magnum. The pathogenesis of primary CIM is still controversial, while acquired CIMs are secondary to intracranial hypertension or hypotension.
Background and definitionIn 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 treatmentThere 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.ConclusionAlongside 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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