Skull Base 2005; 15(1): 89-97
DOI: 10.1055/s-2005-868168
CASE REPORT

Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Resolution of Tonsillar Herniation and Cervical Syringomyelia Following Resection of a Large Petrous Meningioma: Case Report and Review of Literature

Benjamin Fox1 , Dattatraya Muzumdar2 , Franco DeMonte3
  • 1Baylor College of Medicine, Houston, Texas
  • 2Department of Neurosurgery, King Edward VII Memorial Hospital, Parel, Mumbai, India
  • 3Department of Neurosurgery, The University of Texas, M.D. Anderson Cancer Center, Houston, Texas
Further Information

Publication History

Publication Date:
14 April 2005 (online)

ABSTRACT

We describe a case of a large, petrous meningioma associated with tonsillar herniation and cervical syringomyelia. The patient, a 53-year-old woman, had a 6-month history of a dull, aching pain in the occipital region associated with numbness in the right C2 dermatome and left gaze evoked nystagmus. Magnetic resonance imaging (MRI) revealed a large tumor in the right posterior fossa associated with moderate supratentorial hydrocephalus. Secondary tonsillar herniation and cervical syringomyelia extending from C2 to C6 were also identified. The tumor, later confirmed to be a meningioma originating from the petrous region, was resected completely via a retrosigmoid approach. Postoperative MRI demonstrated total resolution of the tonsillar herniation and cervical syringomyelia. The radiological features, potential pathophysiological mechanisms, and treatment strategies are discussed in relation to the recent literature.

REFERENCES

  • 1 Williams B. Pathogenesis of syringomyelia. In: Batzdorf U Syringomyelia: Current Concepts in Diagnosis and Treatment Baltimore, MD; Williams & Wilkens 1991: 59-90
  • 2 De Reuck J, Alva J, Roels H, Van der Ecken H. Relation between syringomyelia and von Hippel-Lindau's disease.  Eur Neurol. 1974;  12 116-127
  • 3 Williams B, Timperley W R. Three cases of communication syringomyelia secondary to midbrain gliomas.  J Neurol Neurosurg Psychiatry. 1977;  40 80-88
  • 4 Nishio S, Matsuno H, Fukui M, Tateishi J, Kitamura K. Cerebellar medulloblastoma associated with lumbosacral syringomyelia.  J Neurol. 1982;  227 67-73
  • 5 Sieben G, Sieben-Praet M, DeReuck J et al.. Dumb-bell sarcoma of the foramen jugulare with syringomyelia. A radio induced tumor?.  J Neurol. 1980;  222 219-225
  • 6 Hinokuma K, Ohama E, Oyanagi K, Kakita A, Kawai K, Ikuta F. Syringomyelia: a neuropathologic study of 18 autopsy cases.  Acta Pathol Jpn. 1992;  42 25-34
  • 7 Milhorat T H, Capocelli Jr A L, Anzil A P, Kotzen R M, Milhorat R H. Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases.  J Neurosurg. 1995;  82 802-812
  • 8 Hirata Y, Matsukado Y, Kaku M. Syringomyelia associated with a foramen magnum meningioma.  Surg Neurol. 1985;  23 291-294
  • 9 Li K C, Chui M C. Conventional and CT metrizamide myelography in Arnold-Chiari I malformation and syringomyelia.  AJNR Am J Neuroradiol. 1987;  8 11-17
  • 10 Kan S, Fox A J, Vinuela F, Barnett H JM, Peerless S J. Delayed CT metrizamide enhancement of syringomyelia secondary to tumor.  AJNR Am J Neuroradiol. 1983;  4 73-78
  • 11 D'Osvaldo D H, Otero J M, Mosconi J B, Oviedo J D. Regression of symptomatic syringomyelia after resection of posterior fossa tumor.  Acta Neurochir (Wien). 2002;  144 385-388
  • 12 Sgaramella E, Perria C. Regression of syringomyelia after removal of a posterior fossa epidermoid tumor.  Br J Neurosurg. 1996;  10 409-411
  • 13 Tachibana S, Harada K, Abe T, Yamada H, Yokata A. Syringomyelia secondary to tonsillar herniation caused by posterior fossa tumors.  Surg Neurol. 1995;  43 470-477
  • 14 Klekamp J, Samii M, Tatagiba M, Sepehrnia A. Syringomyelia in association with tumors of the posterior fossa. Pathophysiological considerations, based on observation on three related cases.  Acta Neurochir (Wien). 1995;  137 38-43
  • 15 Arunkumar M J, Korah I, Chandy M J. Dynamic CSF flow study in the pathophysiology of syringomyelias associated with arachnoid cysts of the posterior fossa.  Br J Neurosurg. 1998;  12 33-36
  • 16 Banna M. Syringomyelia in association with posterior fossa cysts.  AJNR Am J Neuroradiol. 1988;  9 867-873
  • 17 Cano G, Anderson M, Kutschback P, Borden J, Saris S. Hydromyelia associated with a posterior fossa cyst.  Surg Neurol. 1993;  40 512-515
  • 18 Conti C, Lungardi P, Bozzao A, Liccardo G, Fraioli B. Syringomyelia associated with hydrocephalus and Blake's pouch cyst: case report.  Spine. 2003;  28 E279-E283
  • 19 Jain R, Sawlani V, Phadke R, Kumar R. Retrocerebellar arachnoid cyst with syringomyelia: a case report.  Neurol India. 2000;  48 81-83
  • 20 Tokime T, Okamoto S, Yamagata S, Konishi T. Syringomyelia associated with a posterior fossa cyst. Illustration of two cases.  J Neurosurg. 1997;  86 907
  • 21 Zager E L, Ojemann R G, Poletti C E. Acute presentations of syringomyelia. Report of three cases.  J Neurosurg. 1990;  72 133-138
  • 22 Marcus C D, Galeon M, Peruzzi P et al.. Lhermitte-Duclos disease associated with syringomyelia.  Neuroradiology. 1996;  38 529-531
  • 23 Yasha T C, Mohanty A, Radhesh S, Santosh V, Das S, Shankar S K. Infratentorial dysembryoplastic neuroepithelial tumor (DNT) associated with Arnold-Chiari malformation.  Clin Neuropathol. 1998;  17 305-310
  • 24 Fukui K, Kito A, Iguchi I. Asymptomatic syringomyelia associated with cerebellopontine angle meningioma.  Neurol Med Chir (Tokyo). 1993;  33 833-835
  • 25 Anegawa S, Hayashi T, Torigoe R, Iwaisako K, Higashioka H. Cerebellopontine angle meningioma causing asymptomatic syringomyelia-case report.  Neurol Med Chir (Tokyo). 1997;  37 624-626
  • 26 Jaiswal A K, Chandra P S. Cerebellopontine angle meningioma with acquired Chiari and syringomyelia: neuroimage.  Neurol India. 2001;  49 323
  • 27 Karttunen A, Heikkinen E, Tuominen J, Jartti P. Secondary syringomyelia disappearing after removal of tentorial meningioma.  Acta Neurochir (Wien). 2002;  144 741-742
  • 28 Bhatoe H S. Tonsillar herniation and syringomyelia secondary to a posterior fossa tumor.  Br J Neurosurg. 2004;  18 70-71
  • 29 Pillay P K, Awad I A, Little J R, Hahn J F. Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance.  Neurosurgery. 1991;  28 639-645
  • 30 Gardner W J, Angel J. The mechanism of syringomyelia and its surgical correction.  Clin Neurosurg. 1959;  6 131-140
  • 31 Goel A, Bhatjiwale M, Desai K. Basilar invagination: a study based on 190 surgically treated patients.  J Neurosurg. 1998;  88 962-968
  • 32 Pillay P K, Awad I A, Little J R, Hahn J F. Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance.  Neurosurgery. 1991;  28 639-645
  • 33 Oldfield E H, Muraszko K, Shawker T H, Patronas N J. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils: implications for diagnosis and treatment.  J Neurosurg. 1994;  80 3-15
  • 34 Bindal A K, Dunsker S B, Tew Jr J M. Chiari I malformation: classification and management.  Neurosurgery. 1995;  37 1069-1074
  • 35 Logue V, Edwards M R. Syringomyelia and its surgical treatment-an analysis of 75 patients.  J Neurol Neurosurg Psychiatry. 1981;  44 273-284
  • 36 Williams B. Progress in syringomyelia.  Neurol Res. 1986;  8 130-145
  • 37 Stoodley M A, Gutschmidt B, Jones N R. Cerebrospinal fluid flow in an animal model of noncommunicating syringomyelia.  Neurosurgery. 1999;  44 1065-1075
  • 38 Lhermitte, Boveri. Sur un cas de cavité medullaire consécutive a une compression bulbaire chez l'homme et étude experimental de cavités spinales produites par la compression.  Rev Neurol (Paris). 1912;  23 385-393
  • 39 Tauber, Langworthy. A study of syringomyelia and the formation of cavities in the spinal cord.  J Ment Nerv Dis. 1935;  81 245-264
  • 40 Ball M J, Dayan A D. Pathogenesis of syringomyelia.  Lancet. 1972;  2 799-801
  • 41 Aboulker J. La syringomyelie et les liquids intra-rachidiens [in French].  Neurochirurgie. 1979;  25 9-22
  • 42 Goel A, Desai K. Surgery for syringomyelia: an analysis based on 163 surgical cases.  Acta Neurochir (Wien). 2000;  142 293-301
  • 43 Ellertsson A B, Greitz T. The distending force in the production of communicating syringomyelia.  Lancet. 1970;  1 1234
  • 44 Davis C HG, Symon L. Mechanisms and treatment in post-traumatic syringomyelia.  Br J Neurosurg. 1989;  3 669-674
  • 45 Milhorat T H, Capocelli Jr A I, Kotzen R M, Bolognese P, Heger I M, Cottrell J E. Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension.  Neurosurgery. 1997;  41 1102-1110
  • 46 Levine D N. 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 3-21
  • 47 Armounda R A, Citrin C M, Roley K T, Ellenboen R G. Quantitative cine-mode magnetic resonance imaging of Chiari I malformations: an analysis of cerebrospinal fluid dynamics.  Neurosurgery. 1994;  35 214-224
  • 48 Enzmann D R, Pelc N J. Normal flow patterns with phase-contrast cine MRI imaging.  Radiology. 1991;  178 467-474
  • 49 Quencer R M, Post M JD, Hinks R S. Cine MRI in the evaluation of normal and abnormal CSF flow: intracranial and intraspinal studies.  Neuroradiology. 1990;  32 371-391
  • 50 Sherman J L, Citrin C M, Bowen B J, Gangarosa R E. MR demonstration of altered cerebrospinal fluid flow by obstructive lesions.  AJNR Am J Neuroradiol. 1986;  7 571-579
  • 51 Sakaki T, Tsunoda S, Morimoto T, Utsumi S. Is Chiari I malformation in the aged initiated by mechanical factors? Report of three cases.  Neurol Med Chir (Tokyo). 1990;  30 324-328

Franco DeMonteM.D. 

Department of Neurosurgery, The University of Texas, M.D. Anderson Cancer Center

1515 Holcombe Blvd., Box 0442, Houston, TX 77030

Email: fdemonte@mdanderson.org

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    Skull Base 2005; 15(1): 98
    DOI: 10.1055/s-2005-868168
    CASE REPORT

    Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

    Commentary

    Chandranath Sen1 ,
    • 1Department of Neurosurgery, St. Lukes Roosevelt Hospital, New York, New York.
    Further Information

    Publication History

    Publication Date:
    14 April 2005 (online)

    In this interesting case report, a patient with a large posterior fossa meningioma presented with a Chiari I malformation and cervical syringomyelia. The Chiari malformation and syrinx resolved after the tumor was removed and a decompressive craniectomy, C1-C2 laminectomy, and duraplasty were performed.

    As the authors noted, this triad is rare, despite the incidence of posterior fossa meningiomas. Consequently, this patient's posterior fossa may already have been small. The authors treated both pathologies within the same surgical setting. Not surprisingly, these abnormalities resolved completely.

      >
      Skull Base 2005; 15(1): 98
      DOI: 10.1055/s-2005-868168
      CASE REPORT

      Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

      Commentary

      Mark E. Linskey1 ,
      • 1Neurological Surgery, University of California, Irvine, Orange, California
      Further Information

      Publication History

      Publication Date:
      14 April 2005 (online)

      Mr. Fox and colleagues present a well-written and well-illustrated case report along with a thorough review of the literature highlighting the potential for tumor-associated hindbrain herniation to lead to secondary spinal cord syrinx formation. While a rare association, it is one that should be recognized by skull base surgeons, particularly to avoid the mistake of initiating direct treatments for the syrinx, which almost always resolves over time once the hindbrain herniation has been reduced and normal flow of cerebrospinal fluid (CSF) and pressure equilibration dynamics have been restored.

      Indeed, we are increasingly recognizing that spinal cord syrinxes are almost always secondary phenomena. They usually improve or resolve after successful treatment of their primary cause. This holds true for lysing spinal canal arachnoid adhesions for post-traumatic or postmeningitic syrinxes; releasing caudal cord tension for tethered cord syrinxes (e.g., thickened filum, lipomeningocele, or scarred myelomeningocele repair); removing the spinal cord tumor for tumor-associated syrinxes; posterior fossa decompression of Chiari malformation-induced syrinx; anterior decompression for ventral compression or instability-induced syrinx (e.g., platybasia, C1-C2 rheumatoid arthritis); removing the shunt for lumboperitoneal shunt-induced syrinxes; and shunting the ventricles for hydrocephalus-induced hindbrain herniation leading to spinal cord syrinx. Removing a mass lesion (e.g., tumor) leading to hindbrain herniation should now be added to that list. Restoring normal flow of CSF and pressure equilibration dynamics throughout the craniospinal axis should be the goal. Primarily shunting or fenestrating a spinal cord syrinx should become a rare procedure, reserved for the small subset of cases where chronicity of the condition does not allow sufficient spontaneous resolution.

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