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A Rare Case of Chiari Malformation Type 4

Received: 27 August 2017     Accepted: 27 September 2017     Published: 7 November 2017
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Abstract

The Chiari malformations are a family of conditions characterized by developmental or, less commonly, acquired displacements of the cerebellum. The original 19th century description by Hans Chiari delineated 4 types, but only types 1 and 2 are more than just curiosities. In his initial description, Chiari classified the hindbrain malformations into type I, II and III and then latter added type IV malformation. Type IV is a very rare type. It is characterized by cerebellar hypoplasia or aplasia and tentorial hypoplasia. There is no hindbrain herniation in this type. We report a case of a 6 year old male patient who presented to us with a 6 year history of an occipitocervical mass and inability to stand and walk for one year and a 5 month history of headache and vomiting. CT scan of the brain showed a midline posterior fossa bone defect with a meningocele with active obstructive hydrocephalus and hypoplastic cerebellum without hindbrain herniation. A diagnosis of a posterior fossa congenital anomaly (Chiari 4) with obstructive hydrocephalus and occipito-cervical meningocele was made. Ventriculo-peritoneal shunt was inserted three days post admission. Patient was then electively taken to theatre five months later for repair of the occipitocervical meningocele.

Published in Science Journal of Clinical Medicine (Volume 6, Issue 6)
DOI 10.11648/j.sjcm.20170606.12
Page(s) 105-108
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2017. Published by Science Publishing Group

Keywords

Chiari Type IV, Hydrocephalus, Meningocele, Hindbrain

References
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[2] Chiari H. Concerning alterations in the cerebellum resulting from cerebral hydrocephalus. PediatNeurosci. 1987; 13:3-8.
[3] L. P Rowland, T. A Pedley. Merritt’s Neurology. Lippincott William and Wikins, 12th edition, Philadelphia. 2010:590-594.
[4] P. Vannemreddy, A. Nourbakhsh, B. Willis, B. Guthikonda. Congenital Chiarimalformation. Neurology India. 2010; 58:6-14.
[5] Ashfaqul Hassan , Sabah Yaseen et al Arnold-Chiari Malformation: Anatomical Variations and Latest Embryological Perspective. Review of Literature| Volume 3 | Issue 5 | May 2016.
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[7] American Syringomyelia Alliance Project Inc. C&S Patient Education Foundation The World Arnold Chiari Malformation Association.
[8] Milhorat TM, Nishikawa M, Kula RW, Dlugacz YD. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as a guide to clinical management. Acta Neurochir. 2010; 152:1117-1127.
[9] Boyles A, Enterline D, Hammock P et al. Phenotypic definition of Chiari type Imalformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15. Am J Med Genet 2006.
[10] Urbizu A, Toma C, Poca M et al. Chiari Malformation Type I: A Case-ControlAssociation Study of 58 Developmental Genes. 2013; 8(2).
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[12] Bejjani, Cockerham. Adult Chiari Malformation. 1st ed. Pittsburgh, PA; 2015.
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[18] Aslan A, Eser O, Doğru O, Aktepe F, Yurumez Y: Occipital mega encephalocele associated with acute inflammation. Pediatr Neurosurg 2007; 43: 65–66.
[19] Moorthy RK, Rajshekhar V: Management of hydrocephalus associated with occipital encephalocele using endoscopic third ventriculostomy: report of two cases. Surg Neurol 2002; 57: 351–355.
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Cite This Article
  • APA Style

    Kelvin Nemayire, Kantenga Dieu merci Kabulo, Nathaniel Zimani, Nyararai Togarepi, Musara Aaron, et al. (2017). A Rare Case of Chiari Malformation Type 4. Science Journal of Clinical Medicine, 6(6), 105-108. https://doi.org/10.11648/j.sjcm.20170606.12

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    ACS Style

    Kelvin Nemayire; Kantenga Dieu merci Kabulo; Nathaniel Zimani; Nyararai Togarepi; Musara Aaron, et al. A Rare Case of Chiari Malformation Type 4. Sci. J. Clin. Med. 2017, 6(6), 105-108. doi: 10.11648/j.sjcm.20170606.12

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    AMA Style

    Kelvin Nemayire, Kantenga Dieu merci Kabulo, Nathaniel Zimani, Nyararai Togarepi, Musara Aaron, et al. A Rare Case of Chiari Malformation Type 4. Sci J Clin Med. 2017;6(6):105-108. doi: 10.11648/j.sjcm.20170606.12

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  • @article{10.11648/j.sjcm.20170606.12,
      author = {Kelvin Nemayire and Kantenga Dieu merci Kabulo and Nathaniel Zimani and Nyararai Togarepi and Musara Aaron and Kazadi Kaluile Ntenga Kalangu},
      title = {A Rare Case of Chiari Malformation Type 4},
      journal = {Science Journal of Clinical Medicine},
      volume = {6},
      number = {6},
      pages = {105-108},
      doi = {10.11648/j.sjcm.20170606.12},
      url = {https://doi.org/10.11648/j.sjcm.20170606.12},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.sjcm.20170606.12},
      abstract = {The Chiari malformations are a family of conditions characterized by developmental or, less commonly, acquired displacements of the cerebellum. The original 19th century description by Hans Chiari delineated 4 types, but only types 1 and 2 are more than just curiosities. In his initial description, Chiari classified the hindbrain malformations into type I, II and III and then latter added type IV malformation. Type IV is a very rare type. It is characterized by cerebellar hypoplasia or aplasia and tentorial hypoplasia. There is no hindbrain herniation in this type. We report a case of a 6 year old male patient who presented to us with a 6 year history of an occipitocervical mass and inability to stand and walk for one year and a 5 month history of headache and vomiting. CT scan of the brain showed a midline posterior fossa bone defect with a meningocele with active obstructive hydrocephalus and hypoplastic cerebellum without hindbrain herniation. A diagnosis of a posterior fossa congenital anomaly (Chiari 4) with obstructive hydrocephalus and occipito-cervical meningocele was made. Ventriculo-peritoneal shunt was inserted three days post admission. Patient was then electively taken to theatre five months later for repair of the occipitocervical meningocele.},
     year = {2017}
    }
    

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    AU  - Kelvin Nemayire
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    AU  - Nathaniel Zimani
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    JO  - Science Journal of Clinical Medicine
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    AB  - The Chiari malformations are a family of conditions characterized by developmental or, less commonly, acquired displacements of the cerebellum. The original 19th century description by Hans Chiari delineated 4 types, but only types 1 and 2 are more than just curiosities. In his initial description, Chiari classified the hindbrain malformations into type I, II and III and then latter added type IV malformation. Type IV is a very rare type. It is characterized by cerebellar hypoplasia or aplasia and tentorial hypoplasia. There is no hindbrain herniation in this type. We report a case of a 6 year old male patient who presented to us with a 6 year history of an occipitocervical mass and inability to stand and walk for one year and a 5 month history of headache and vomiting. CT scan of the brain showed a midline posterior fossa bone defect with a meningocele with active obstructive hydrocephalus and hypoplastic cerebellum without hindbrain herniation. A diagnosis of a posterior fossa congenital anomaly (Chiari 4) with obstructive hydrocephalus and occipito-cervical meningocele was made. Ventriculo-peritoneal shunt was inserted three days post admission. Patient was then electively taken to theatre five months later for repair of the occipitocervical meningocele.
    VL  - 6
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Author Information
  • Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe

  • Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe

  • Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe

  • Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe

  • Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe

  • Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe

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