Chiari type 1 deformity classification

Chiari type 1 deformity classification

Valentini et al. suggested to define an association of Chiari type 1 deformity plus untreated sagittal synostosis, a new subtype of complex CM1. For the high percentage of complications and multiple procedures needed to solve the CM1, they advise identifying by 3D-CT scan these children before performing craniovertebral decompression (CVD). They suggest also that if left untreated, sagittal synostosis may lead to the delayed occurrence of a challenging subset of CM1 1).

Chiari malformation Type 1.5 (CM 1.5) was defined as the association of Chiari malformation Type I (CM I) and brainstem herniation.

Although CM 1.5 patients presented with brainstem herniation and more severe tonsillar herniation, other clinical and imaging features and surgical outcomes were similar to CM I patients. Liu et al. think CM 1.5 is just a subtype of CM I, rather than a unique type of Chiari malformations 2).

Taylor et al. identify two subtypes, crowded and spacious, that can be distinguished by MRI appearance without volumetric analysis. Earlier age at surgery and the presence of syringomyelia are more common in the crowded subtype. The presence of the spacious subtype suggests that crowdedness alone cannot explain the pathogenesis of Chiari I malformation in many patients, supporting the need for further investigation 3).

see Pediatric Chiari type 1 deformity.

see Chiari type 1 deformity and syringomyelia.



Valentini LG, Saletti V, Erbetta A, Chiapparini L, Furlanetto M. Chiari 1 malformation and untreated sagittal synostosis: a new subset of complex Chiari? Childs Nerv Syst. 2019 Jul 21. doi: 10.1007/s00381-019-04283-0. [Epub ahead of print] PubMed PMID: 31327038.

Liu W, Wu H, Aikebaier Y, Wulabieke M, Paerhati R, Yang X. No significant difference between Chiari malformation type 1.5 and type I. Clin Neurol Neurosurg. 2017 Mar 30;157:34-39. doi: 10.1016/j.clineuro.2017.03.024. [Epub ahead of print] PubMed PMID: 28384597.

Taylor DG, Mastorakos P, Jane JA Jr, Oldfield EH. Two distinct populations of Chiari I malformation based on presence or absence of posterior fossa crowdedness on magnetic resonance imaging. J Neurosurg. 2017 Jun;126(6):1934-1940. doi: 10.3171/2016.6.JNS152998. Epub 2016 Sep 2. PubMed PMID: 27588590.

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