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Advanced Imaging of Chiari I Malformations

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The Chiari Malformations

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Abstract

Chiari type I malformation (CM I), characterized by descent of part of the cerebellum and brain stem through the foramen magnum, is a relatively common imaging diagnosis with a reported incidence of 1–3.6% in children undergoing magnetic resonance imaging (MRI). Standard MRI sequences can establish the diagnosis of CM I rather easily. However, it remains neither sensitive nor specific for predicting symptoms and disease severity and does not contribute to prediction of postoperative success. The development of advanced MRI sequences and techniques has expanded the role of MRI in CM I disease assessment. Advanced imaging including cerebrospinal fluid (CSF) flow studies, cine cerebellar tonsillar motion, diffusion tensor imaging (DTI), and quantitative volumetrics of the posterior fossa and biometrics of the skull base have expanded our understanding of CM I disease pathophysiology, assessment of disease severity, and alterations in CSF flow dynamics, and in certain cases aid in selecting surgical candidates. The goal of this chapter is to review in detail established MR advanced imaging techniques such as CSF flow studies and cine cerebellar tonsillar motion in the diagnosis of CM I while introducing emerging techniques in the assessment of CM I such as DTI and volumetry of the posterior fossa.

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Authors and Affiliations

  1. Department of Neuroradiology, Mallinckrodt Institute of Radiology, Bames Jewish Hospital, Washington University School of Medicine, St. Louis, MO, USA

    Rami W. Eldaya

  2. Department of Neurosurgery, Washington University School of Medicine, Orthopedic Surgery and Pediatrics, Washington University School of Medicine, St. Louis Children’s Hospital, St. Louis, MO, USA

    Jennifer M. Strahle

  3. Department of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, MO, USA

    Manu S. Goyal

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Correspondence to Rami W. Eldaya .

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Editors and Affiliations

  1. Department of Neurosurgery, Tulane University School of Medicine, New Orleans, LA, USA

    R. Shane Tubbs

  2. Department of Neurosurgery, Aydın Adnan Menderes University School of Medicine, Efeler, Aydın, Turkey

    Mehmet Turgut

  3. Departments of Neurosurgery and Pediatrics, University of Alabama, Birmingham, AL, USA

    W. Jerry Oakes

Electronic Supplementary Material

Represents a normal subject with no Chiari I deformity and normal flow studies at the foramen magnum. The videos represent the same patient presented in Fig. 28.1. (a) Sagittal second phase cine with a VENC of 10 showing normal flow at the foramen magnum anteriorly and posteriorly. (MP4 5803 kb)

Sagittal phase-contrast cine with a VENC of 10 showing normal bidirectional flow at the foramen magnum anteriorly and posteriorly (MP4 5874 kb)

Represents a patient with Chiari I deformity with normal flow studies at the foramen magnum. The videos represent the same patient presented in Fig. 28.2. (a) Sagittal second phase cine with a VENC of 10 showing normal flow at the foramen magnum anteriorly and posteriorly. (MP4 4839 kb)

Sagittal phase-contrast cine with a VENC of 10 showing normal bidirectional flow at the foramen magnum anteriorly and posteriorly (MP4 5451 kb)

Represents a patient with Chiari I deformity with mild decrease of flow posteriorly. The videos represent the same patient presented in Fig. 28.3. (a) Sagittal second phase cine with a VENC of 10 showing normal flow at the foramen magnum anteriorly and mildly decreased flow posteriorly. (MP4 2124 kb)

Sagittal phase-contrast cine with a VENC of 10 showing normal flow at the foramen magnum anteriorly and mildly decreased flow posteriorly (MP4 4244 kb)

Represents a patient with Chiari I deformity with absent flow posteriorly. The videos represent the same patient presented in Fig. 28.4. (a) Sagittal second phase cine with a VENC of 10 showing normal flow at the foramen magnum anteriorly with absent flow posteriorly. (MP4 3440 kb)

Sagittal phase-contrast cine with a VENC of 10 showing normal flow at the foramen magnum anteriorly and absent flow posteriorly (MP4 3648 kb)

Represents a patient with Chiari I deformity with diminished flow anteriorly and absent flow posteriorly. The videos represent the same patient presented in Fig. 28.5. (a) Sagittal second phase cine with a VENC of 10 showing diminished flow at the foramen magnum anteriorly with absent flow posteriorly. (MP4 2671 kb)

Sagittal phase-contrast cine with a VENC of 10 showing diminished flow at the foramen magnum anteriorly and absent flow posteriorly (MP4 3589 kb)

Represents a patient with Chiari I deformity with complex flow anteriorly and absent flow posteriorly. The videos represent the same patient presented in Fig. 28.6. (a) Sagittal second phase cine with a VENC of 5 showing relatively normal flow at the foramen magnum anteriorly with absent flow posteriorly. (MP4 6293 kb)

Sagittal phase-contrast cine with a VENC of 5 showing aliasing flow at the foramen magnum anteriorly and absent flow posteriorly (see Fig. 28.6 and text for detailed discussion of pathophysiology of aliasing). (MP4 9613 kb)

Sagittal phase-contrast cine with a VENC of 10 showing marked improvement of the aliasing flow at the foramen magnum anteriorly with persistent absent flow posteriorly (see Fig. 28.6 and text for detailed discussion of pathophysiology of aliasing). (MP4 8371 kb)

Axial phase-contrast cine at the foramen magnum with a VENC of 10 showing residual aliasing flow at the foramen magnum anteriorly with persistent absent flow posteriorly (see Fig. 28.6 and text for detailed discussion of pathophysiology of aliasing) (MP4 4339 kb)

Represents a patient with Chiari I deformity with absent flow anteriorly and posteriorly. The videos represent the same patient presented in Fig. 28.7. (a) Sagittal second phase cine with a VENC of 10 showing absent flow at the foramen magnum anteriorly and posteriorly. (MP4 4298 kb)

Sagittal phase-contrast cine with a VENC of 10 showing absent flow at the foramen magnum anteriorly and posteriorly (MP4 4112 kb)

Represents a patient with Chiari I deformity with transient obstruction of flow anteriorly and posteriorly secondary to marked tonsillar pulsatility. The videos represent the same patient presented in Fig. 28.8. (a) Sagittal second phase cine with a VENC of 10 showing transient absent flow at the foramen magnum anteriorly and posteriorly secondary to marked tonsillar pulsatility (MP4 2508 kb)

Sagittal phase-contrast cine with a VENC of 10 showing transient absent flow at the foramen magnum anteriorly and posteriorly secondary to marked tonsillar pulsatility (MP4 3173 kb)

Sagittal true-FISP video shows hyperdynamic pulsatile cerebellar tonsils demonstrating marked anterior and inferior motion through the cardiac cycle (video slowed to 4 frames per second (normal display is at 30 frames per second) to better demonstrate the tonsillar motion). (MP4 1672 kb)

Sagittal second phase cine with a VENC of 10 postsurgical decompression showing significant improvement of the flow at the foramen magnum with resolution of the preoperative transient absent flow at the foramen magnum. (MP4 1662 kb)

Sagittal phase-contrast cine with a VENC of 10 postsurgical decompression showing significant improvement of the flow at the foramen magnum with resolution of the preoperative transient absent flow at the foramen magnum. (MP4 2839 kb)

Sagittal true-FISP video shows persistent but improved pulsatile cerebellar tonsillar motion through the cardiac cycle (video slowed to 4 frames per second (normal display is at 30 frames per second) to better demonstrate the tonsillar motion) (MP4 1760 kb)

Represents a patient with Chiari I deformity with increased tonsillar pulsatility. Note the increased pulsatile craniocaudal and anteroposterior displacement (MP4 1911 kb)

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Eldaya, R.W., Strahle, J.M., Goyal, M.S. (2020). Advanced Imaging of Chiari I Malformations. In: Tubbs, R., Turgut, M., Oakes, W. (eds) The Chiari Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_28

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  • DOI: https://doi.org/10.1007/978-3-030-44862-2_28

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  • Online ISBN: 978-3-030-44862-2

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