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CC BY-NC-ND 4.0 · Asian J Neurosurg 2022; 17(03): 515-520
DOI: 10.1055/s-0042-1756634
Case Report

Posterior Cranial Fossa Meningioma Causing Tonsillar Herniation and Giant Cervicothoracic Syringomyelia: Case Report and Review of Literature

Estelle Anne How Hong
1   Department of Neurosurgery, Hull University Teaching Hospitals, Hull, United Kingdom
,
Ahmed Shalid
1   Department of Neurosurgery, Hull University Teaching Hospitals, Hull, United Kingdom
,
Srihari Deepak
1   Department of Neurosurgery, Hull University Teaching Hospitals, Hull, United Kingdom
,
Gueorgui Kounin
1   Department of Neurosurgery, Hull University Teaching Hospitals, Hull, United Kingdom
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Abstract

Syringomyelia is a fluid-filled cyst within the spinal cord and is usually associated with conditions that obstruct the cerebrospinal fluid (CSF) (flow at the foramen magnum or spinal levels such as Chiari malformations, arachnoiditis, and basilar invaginations). Very rarely, posterior cranial fossa tumors can lead to tonsillar herniation and secondary syringomyelia. There are only nine reported cases in the literature. We report a rare case of a 56-year-old female with posterior cranial meningioma and secondary syringomyelia, admitted with headache, nausea, vomiting, and ataxic gait. Magnetic resonance imaging (MRI) demonstrated a large posterior fossa lesion causing early ventriculomegaly and a giant cervicothoracic syrinx within the upper spinal cord extending from the hindbrain inferiorly to the level of T8. She underwent a posterior fossa craniectomy with left C1 hemilaminectomy and complete excision of the tumor. In 6 months following her procedure, an MRI scan showed a significant reduction in the caliber of the syringomyelia throughout its length, and there was a significant improvement in symptoms. Although the pathophysiology of syrinx formation is still poorly understood, the alteration of CSF dynamic flow has been implicated. A common unifying cause appears to be increased transcranial difference in intracranial pressure across the foramen magnum causing tonsillar herniation, irrespective of location in the posterior fossa. With high syrinx pressure, mechanical stress-induced structural change of the spinal cord occurs, allowing the persistence and progression of the syrinx in the spinal cord. Syringomyelia appears to be resolved partially or completely after craniotomy and excision of the posterior fossa lesion.

Keywords

syringomyelia - posterior cranial fossa - meningioma - tonsillar herniation

Informed Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.




Publication History

Article published online:
08 October 2022

© 2022. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Tachibana S, Harada K, Abe T, Yamada H, Yokota A. Syringomyelia secondary to tonsillar herniation caused by posterior fossa tumors. Surg Neurol 1995; 43 (05) 470-475 , discussion 475–477
    CrossrefPubMedGoogle Scholar
  • 2 Goel A, Shah AH, Vutha R, Goel A. External syringomyelia in longstanding benign foramen magnum tumors. Surg Neurol Int 2020; 11: 92
    CrossrefPubMedGoogle Scholar
  • 3 Bhatoe HS. Tonsillar herniation and syringomyelia secondary to a posterior fossa tumour. Br J Neurosurg 2004; 18 (01) 70-71
    CrossrefPubMedGoogle Scholar
  • 4 Wang J, Alotaibi NM, Samuel N, Ibrahim GM, Fallah A, Cusimano MD. Acquired Chiari malformation and syringomyelia secondary to space-occupying lesions: a systematic review. World Neurosurg 2017; 98: 800-808.e2
    CrossrefPubMedGoogle Scholar
  • 5 Galarza M, López-Guerrero AL, Martínez-Lage JF. Posterior fossa arachnoid cysts and cerebellar tonsillar descent: short review. Neurosurg Rev 2010; 33 (03) 305-314 , discussion 314
    CrossrefPubMedGoogle Scholar
  • 6 Levine DN. The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis. J Neurol Sci 2004; 220 (1-2): 3-21
    CrossrefPubMedGoogle Scholar
  • 7 Fox B, Muzumdar D, DeMonte F. Resolution of tonsillar herniation and cervical syringomyelia following resection of a large petrous meningioma: case report and review of literature. Skull Base 2005; 15 (01) 89-97 , discussion 98
    Article in Thieme ConnectPubMedGoogle Scholar
  • 8 Jaiswal AK, Chandra PS. Cerebellopontine angle meningioma with acquired Chiari and syringomyelia: neuroimage. Neurol India 2001; 49 (03) 323
    PubMedGoogle Scholar
  • 9 Del Maestro M, De Paulis D, Ricci A, Di Cola F, Galzio R. Syringobulbia associated with posterior fossa meningioma: a review of the literature. Childs Nerv Syst 2014; 30 (10) 1749-1752
    CrossrefPubMedGoogle Scholar
  • 10 Kankane VK, Jaiswal G, Gupta T. Large tentorium meningioma causing Chiari malformation type-1 with syringomalia with complete resolution of syrinx and Chiari after surgical excision: rare case report with review of literature. Rom Neurosurg 2016; 30: 127-131
    Google Scholar
  • 11 Bouras TI, Kouyialis AT, Boviatsis EJ, Sakas DE. Symptomatic syringomyelia secondary to clinically obscure infratentorial tumour. Br J Neurosurg 2006; 20 (01) 48-50
    CrossrefPubMedGoogle Scholar
  • 12 Karttunen A, Heikkinen E, Tuominen J, Jartti P. Secondary syringomyelia disappearing after removal of tentorial meningioma. Acta Neurochir (Wien) 2002; 144 (07) 741-742
    CrossrefPubMedGoogle Scholar