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MRI findings differentiating tonsillar herniation caused by idiopathic intracranial hypertension from Chiari I malformation

  • Diagnostic Neuroradiology
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Abstract

Purpose

Some patients with idiopathic intracranial hypertension (IIH) have cerebellar tonsillar herniation ≥ 5 mm mimicking Chiari malformation I (CMI), which can result in misdiagnosis and unjustified treatment. Our purpose was to identify IIH patients with tonsillar herniation ≥ 5 mm (IIHTH) and compare with CMI patients to assess imaging findings that could distinguish the two conditions.

Methods

Ninety-eight patients with IIH, 81 patients with CMI, and 99 controls were retrospectively assessed. Two neuroradiologists blindly reviewed MR images. IIHTH patients were compared with CMI patients and controls regarding the extent of tonsillar herniation (ETH), bilateral transverse sinus stenosis (BTSS), hypophysis-sella ratio (HSR), and bilateral tortuosity of optic nerve (BTON).

Results

13/98 (13.2%) IIH patients had tonsillar herniation ≥ 5 mm (IIHTH) and were significantly younger and had higher BMI compared with CMI patients and controls. ETH was significantly less in the IIHTH than CMI (6.5 ± 2.4 mm vs. 10.9 ± 4.4 mm; p < 0.001). BTSS and HSR < 0.5 were more common in IIHTH than CMI (p < 0.001 and p = 0.003, respectively). No differences were seen between CMI and controls. BTON was significantly more common in IIHTH compared to control (p = 0.01) but not to the CMI (p = 0.36). Sensitivity and specificity to differentiate IIHTH from CMI were 69.2% and 96.1% for BTSS and 69.2% and 75.3% for HSR < 0.5.

Conclusion

The presence of BTSS and/or HSR < 0.5 in patients with ETH ≥ 5 mm should suggest further evaluation to exclude IIH before considering CMI surgery.

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Funding

No funding was received for this study.

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Authors and Affiliations

  1. Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, WCB90, 330 Brookline Ave, Boston, MB, 02215, USA

    Seyed Amir Ebrahimzadeh, Elizabeth Du, Yu-Ming Chang & Rafeeque A. Bhadelia

  2. Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

    Marc Bouffard

  3. Department of Mechanical and Industrial Engineering, Northeastern University, Boston, MA, USA

    Francis Loth

Authors
  1. Seyed Amir Ebrahimzadeh
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  2. Elizabeth Du
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  3. Yu-Ming Chang
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Contributions

Seyed Amir Ebrahimzadeh, MD, MPH

Data collection, statistical analysis, manuscript preparation, and editing

Elizabeth Du, MD

Data collection

Yu-Ming Chang, MD, PhD

Data collection, manuscript preparation, and editing

Marc Bouffard, MD

Manuscript preparation and editing

Francis Loth, PhD

Manuscript preparation and editing

Rafeeque A Bhadelia, MD

Conceptualization, study design, data collection, manuscript preparation, and editing

Corresponding author

Correspondence to Seyed Amir Ebrahimzadeh.

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Conflict of interest

The authors have no relevant financial or non-financial interests to disclose.

Ethical approval

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The Human Investigation Committee (IRB) of Beth Israel Deaconess Medical Center approved this study.

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For this type of study, formal consent was not required and was waived by the institutional review board.

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Ebrahimzadeh, S.A., Du, E., Chang, YM. et al. MRI findings differentiating tonsillar herniation caused by idiopathic intracranial hypertension from Chiari I malformation. Neuroradiology 64, 2307–2314 (2022). https://doi.org/10.1007/s00234-022-02993-y

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  • DOI: https://doi.org/10.1007/s00234-022-02993-y

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