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NEUROIMAGE
Year : 2021  |  Volume : 69  |  Issue : 4  |  Page : 1137-1138

Giant Acoustic Schwannoma with Marked Cerebellar Tonsillar Herniation and Secondary Syringomyelia


1 Professor and Head of Unit, Department of Neurosurgery, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Mumbai, India
2 Intern, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Mumbai, India

Date of Submission02-Dec-2020
Date of Decision22-Dec-2020
Date of Acceptance15-Feb-2021
Date of Web Publication2-Sep-2021

Correspondence Address:
Alhad Mulkalwar
Department of Neurosurgery, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Acharya Donde Marg, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.325326

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How to cite this article:
Chagla A, Mulkalwar A. Giant Acoustic Schwannoma with Marked Cerebellar Tonsillar Herniation and Secondary Syringomyelia. Neurol India 2021;69:1137-8

How to cite this URL:
Chagla A, Mulkalwar A. Giant Acoustic Schwannoma with Marked Cerebellar Tonsillar Herniation and Secondary Syringomyelia. Neurol India [serial online] 2021 [cited 2021 Sep 25];69:1137-8. Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1137/325326




Acquired herniation of cerebellar tonsils has previously commonly been described in association with hydrocephalus and increased drainage of cerebrospinal fluid. Syringomyelia is also often reported in patients with congenital craniovertebral anomalies such as Chiari I malformation and basilar invagination. The triad of cerebellar tonsillar herniation and syringomyelia related to posterior fossa tumors, however, is uncommon.[1],[2] Earlier evidence on this unique association was based on postmortem reports[3] or its suggested presence as demonstrated in myelographic studies.[4]

A 40-year-old female presented to the Neurosurgery Outpatient Department of King Edward Memorial Hospital, Mumbai with decreased hearing from the right ear, giddiness and imbalance on walking, and tingling sensation in all four limbs, which developed over 2 years. Examination revealed right-sided horizontal gaze nystagmus and right-sided sensorineural hearing loss with right-sided cerebellar ataxia. Magnetic resonance imaging (MRI) brain revealed a huge heterogenous enhancing tumor (5.5*4.7*4.5 cm) situated in the right cerebellopontine angle and centered on the internal auditory meatus, expanding its orifice. It was causing mass effect on the right cerebellum and pons, displacing the same extensively. This produced cerebellar tonsillar herniation upto the C2 vertebral level and distortion of the fourth ventricle [Figure 1]. Cystic expansion of neural canal with dilatation of cervical spinal cord from C3 to C7 vertebral levels could also be appreciated [Figure 2]. The patient underwent right retrosigmoid craniectomy and excision of the lesion with good postoperative clinical outcome and partial resolution of the tonsillar herniation and syringomyelia as seen on the postoperative MRI scan [Figure 3]. Thus, it could be concluded that the posterior fossa mass caused secondary cerebellar tonsillar herniation leading to cervical syringomyelia.
Figure 1: (a) Pre operative Contrast enhanced Magnetic Resonance Imaging of brain and cervical spinal cord (axial plane) revealed a huge heterogenous enhancing tumor (5.5*4.7*4.5 cm) situated in the right cerebellopontine angle, centered on the internal auditory meatus, causing mass effect on ipsilateral cerebellum and pons, producing distortion and compression of the fourth ventricle and marked cerebellar tonsillar herniation upto the C2 vertebral level. (b) Pre operative Contrast enhanced Magnetic Resonance Imaging of brain and cervical spinal cord (Coronal plane)

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Figure 2: Pre operative Magnetic Resonance Imaging of posterior fossa with cervical spine revealed cystic expansion of neural canal with dilatation of cervical cord extending from C3 to C7. Mild abnormal T2 hyperintense cord signal is seen in cervical spinal cord superior and inferior to the cystic dilatation. Cerebellar tonsillar herniation upto C2 vertebral level can be appreciated

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Figure 3: Post operative Magnetic Resonance Imaging of brain and posterior fossa with cervical spine (saggital plane) depicting complete resolution of the tonsillar herniation and syringomyelia

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  References Top

1.
Williams B. Pathogenesis of syringomyelia. In: Batzdorf U, editor. Syringomyelia: Current Concepts in Diagnosis and Treatment. Baltimore, MD: Williams & Wilkens; 1991. p. 59-90.  Back to cited text no. 1
    
2.
Jaiswal AK, Chandra PS. Cerebellopontine angle meningioma with acquired chiari and syringomyelia: Neuroimage. Neurol India 2001;49:323.  Back to cited text no. 2
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3.
De Reuck J, Alva J, Roels H, Ecken H Van der. Relation between syringomyelia and von Hippel-Lindau's disease. Eur Neurol 1974;12:116-27.  Back to cited text no. 3
    
4.
Hirata Y, Matsukado Y, Kaku M. Syringomyelia associated with a foramen magnum meningioma. Surg Neurol 1985;23:291-4.  Back to cited text no. 4
    


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