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Posterior cranial fossa and cervical spine morphometric abnormalities in symptomatic Chiari type 0 and Chiari type 1 malformation patients with and without syringomyelia

  • Original Article - CSF Circulation
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Abstract

Background

To better understand how anatomical features of Chiari malformation type 0 (CM0) result in the manifestation of Chiari malformation type 1 (CM1) signs and symptoms, we conducted a morphometric study of the posterior cranial fossa (PCF) and cervical canal in patients with CM1 and CM0.

Methods

This retrospective study had a STROBE design and included 120 adult patients with MRI evidence of a small PCF (SPCF), typical clinical symptoms of CM1, and a diagnosis of CM1, CM0, or SPCF-TH0-only (SPCF with cerebellar ectopia less than 2 mm and without syringomyelia). Patients were divided by MRI findings into 4 groups: SPCF-TH0-only, SPCF-TH0-syr (CM0 with SPCF and syringomyelia), SPCF-CM1-only (SPCF with cerebellar ectopia 5 mm or more without syringomyelia), and SPCF-CM1-syr (CM1 with syringomyelia). Neurological examination data and MRI parameters were analyzed.

Results

All patient cohorts had morphometric evidence of a small, flattened, and overcrowded PCF. The PCF phenotype of the SPCF-TH0-only group differed from that of other CM cohorts in that the length of clivus and supraocciput and the height of the PF were longer, the upper CSF spaces of PCF were taller, and the area of the foramen magnum was smaller. The SPCF-TH0 groups had a more significant narrowing of the superior cervical canal and a smaller decrease in PCF height than the SPCF-CM1 groups.

Conclusions

Patients with SPCF-TH0 with and without syringomyelia developed Chiari 1 symptoms and signs. Patients with SPCF-TH0-syr (Chiari 0) had more constriction of their CSF pathways in and around the foramen magnum than patients with SPCF-TH0-only.

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Abbreviations

BI:

Basilar invagination

CM:

CM1 and CM0

CM0:

Chiari type 0 malformation

CM1.5:

Chiari malformation type 1.5

CM1:

Chiari type 1 malformation

CON:

Control group

CVJ:

Cranio-vertebral junction

FM:

Foramen magnum

PCF:

Posterior cranial fossa

S:

Syringomyelia

SPCF:

Small posterior cranial fossa

SPCF-TH0-only:

Patients with SPCF, Chiari 1 symptoms, tonsillar ectopia less than 2 mm, and without S

SPCF-TH0-syr:

CM0 with SPCF and with S

SPCF-CM1-only:

CM1 with SPCF and without S

SPCF-CM1-syr:

CM1 with SPCF and with S

SPCF-TH0:

Joint group SPCF-TH0-only and SPCF-TH0-syr

SPCF-CM1:

Joint group SPCF-CM1-only and SPCF-CM1-syr

SPCF-only:

Joint group SPCF-TH0-only and SPCF-CM1-only

SPCF-syr:

Joint group SPCF-TH0-syr and SPCF-CM1-syr

TH:

Tonsillar herniation

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Authors and Affiliations

  1. Department of Neurology and Rehabilitation, Kazan State Medical University, Butlerov str. 49, Kazan, Russian Federation

    Enver I. Bogdanov & Aisylu T. Faizutdinova

  2. Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, 10-3D20, MSC-1414, Bethesda, MD, 20892-1414, USA

    John D. Heiss

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  1. Enver I. Bogdanov
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Contributions

All authors contributed to the study's conception and design. Enver I. Bogdanov, Aisylu T. Faizutdinova, and John D. Heiss performed material preparation, data collection, and analysis. Enver I. Bogdanov wrote the first draft of the manuscript. All authors commented on previous versions of the manuscript and read and approved the final manuscript.

In this retrospective research, we followed STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) rules. Patient data were selected from an institutional database also containing data from a regional epidemiological study of CM1 and CM0 (approved by the local ethics committee) [5]. This manuscript describes a retrospective study and involves anonymized data. Formal consent is not required for this type of study.

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Correspondence to Aisylu T. Faizutdinova.

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Bogdanov, E.I., Faizutdinova, A.T. & Heiss, J.D. Posterior cranial fossa and cervical spine morphometric abnormalities in symptomatic Chiari type 0 and Chiari type 1 malformation patients with and without syringomyelia. Acta Neurochir 163, 3051–3064 (2021). https://doi.org/10.1007/s00701-021-04941-w

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