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Chiari I—a ‘not so’ congenital malformation?

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Abstract

The term Chiari I malformation (CIM) is imbedded in the paediatric neurosurgical lexicon; however, the diagnostic criteria for this entity are imprecise, its pathophysiology variable, and the treatment options diverse. Until recently, CIM has been considered to be a discrete congenital malformation requiring a uniform approach to treatment. Increasingly, it is recognised that this is an oversimplification and that a more critical, etiologically based approach to the evaluation of children with this diagnosis is essential, not only to select those children who might be suitable for surgical treatment (and, of course those who might be better served by conservative management) but also to determine the most appropriate surgical strategy. Whilst good outcomes can be anticipated in the majority of children with CIM following foramen magnum decompression, treatment failures and complication rates are not insignificant. Arguably, poor or suboptimal outcomes following treatment for CIM reflect, not only a failure of surgical technique, but incorrect patient selection and failure to acknowledge the diverse pathophysiology underlying the phenomenon of CIM. The investigation of the child with ‘hindbrain herniation’ should be aimed at better understanding the mechanisms underlying the herniation so that these may be addressed by an appropriate choice of treatment.

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Acknowledgements

This article is an abridged version dealing with aetiology of Chiari I malformation. For a more extensive review of current Chiari I management including a review of investigation and treatment, see Thompson D Chiari I Malformation and Associated syringomyelia In Textbook of Paediatric Neurosurgery Eds. DiRocco C, Pang D, Rutka J Springer 2019 ISBN 978-3-319-72167-5.

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  1. Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK

    Dominic N. P. Thompson

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Thompson, D.N.P. Chiari I—a ‘not so’ congenital malformation?. Childs Nerv Syst 35, 1653–1664 (2019). https://doi.org/10.1007/s00381-019-04296-9

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