Science Journal of Clinical Medicine

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A Rare Case of Chiari Malformation Type 4

Received: 27 August 2017    Accepted: 27 September 2017    Published: 07 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.

DOI 10.11648/j.sjcm.20170606.12
Published in Science Journal of Clinical Medicine (Volume 6, Issue 6, November 2017)
Page(s) 105-108
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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), 2024. 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.
[6] Banerji NK, Millar JHD: Chiari malformation presenting in adult life. Brain 97:157-168, 1974.
[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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[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.
[20] Andarabi Y, Nejat F, El-Khashab M: Progressive skin necrosis of a huge occipital encephalocele. Indian J Plast Surg 2008; 41: 82–84.
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[22] Ashfaq Ul Hassan, A Giant Occipital Encephalocele Universal Journal of Applied Science 1(1): 8-10, 2013.
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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    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://download.sciencepg.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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    T1  - A Rare Case of Chiari Malformation Type 4
    AU  - Kelvin Nemayire
    AU  - Kantenga Dieu merci Kabulo
    AU  - Nathaniel Zimani
    AU  - Nyararai Togarepi
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    JF  - Science Journal of Clinical Medicine
    JO  - Science Journal of Clinical Medicine
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    PB  - Science Publishing Group
    SN  - 2327-2732
    UR  - https://doi.org/10.11648/j.sjcm.20170606.12
    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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