A Rare Case of Chiari Malformation Type 4

Kelvin Nemayire; Kantenga Dieu merci Kabulo; Nathaniel Zimani; Nyararai Togarepi; Musara Aaron; Kazadi Kaluile Ntenga Kalangu

doi:doi:10.11648/j.sjcm.20170606.12

A Rare Case of Chiari Malformation Type 4

Kelvin Nemayire, Kantenga Dieu merci Kabulo, Nathaniel Zimani, Nyararai Togarepi, Musara Aaron, Kazadi Kaluile Ntenga Kalangu

Published in Science Journal of Clinical Medicine (Volume 6, Issue 6)

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

Views: Downloads:

Download PDF

Share This Article

Twitter
Linked In
Facebook

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), 2024. Published by Science Publishing Group

Keywords

Chiari Type IV, Hydrocephalus, Meningocele, Hindbrain

References

[1]	Tubbs RS, Elton S, Grabb P, et al. Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery. 2001; 48:1050-1055.
[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).
[11]	Ye S. Bioinformatics Boca Raton [u. a]: Taylor & Francis; 2008.
[12]	Bejjani, Cockerham. Adult Chiari Malformation. 1st ed. Pittsburgh, PA; 2015.
[13]	Avellaneda Fernández A, Isla Guerrero A, Izquierdo Martínez M et al. Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment). BMC Musculoskeletal Disorders. 2009; 10 (Suppl 1).
[14]	Barone CM, Jimenez DF, Helling ER, LaskeyALS: What is the incidence of hypertelorism in Filipino frontonasal encephalocele patients?J Craniofac Surg 2007; 18: 268–273.
[15]	Bozinov O, Tirakotai W, Sure U, BertalanffyH: Surgical closure and reconstruction of alarge occipital encephalocele without parenchymalexcision. Childs Nerv Syst 2005; 21:144–147.
[16]	Raja RA, Qureshi AA, Memon AR, Ali H, Dev V: Pattern of encephaloceles: a case series. J Ayub Med Coll Abbottabad 2008; 20: 125–128.
[17]	Antunes JL, Sharer LR, Pellock JM: Occipitalencephaloce – A case of conjoined twinning? Neurosurgery 1983; 13: 703–707.
[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.
[21]	Kotil K, Kilinc B, Bilge T: Diagnosis and management of large occipitocervical cephaloceles:a 10 year experience. Pediatr Neurosurg 2008; 44: 193–198.
[22]	Ashfaq Ul Hassan, A Giant Occipital Encephalocele Universal Journal of Applied Science 1(1): 8-10, 2013.

Cite This Article

Plain Text BibTeX RIS

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

Copy | Download

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

Copy | Download

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

Copy | Download

@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}
}

Copy | Download

TY - JOUR
T1 - A Rare Case of Chiari Malformation Type 4
AU - Kelvin Nemayire
AU - Kantenga Dieu merci Kabulo
AU - Nathaniel Zimani
AU - Nyararai Togarepi
AU - Musara Aaron
AU - Kazadi Kaluile Ntenga Kalangu
Y1 - 2017/11/07
PY - 2017
N1 - https://doi.org/10.11648/j.sjcm.20170606.12
DO - 10.11648/j.sjcm.20170606.12
T2 - Science Journal of Clinical Medicine
JF - Science Journal of Clinical Medicine
JO - Science Journal of Clinical Medicine
SP - 105
EP - 108
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
IS - 6
ER -

Copy | Download

Author Information

Kelvin Nemayire

Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe
Kantenga Dieu merci Kabulo

Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe
Nathaniel Zimani

Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe
Nyararai Togarepi

Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe
Musara Aaron

Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe
Kazadi Kaluile Ntenga Kalangu

Department of Neurosurgery, University of Zimbabwe, Harare, Zimbabwe

Download PDF

Sections

Plain Text BibTeX RIS

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

Copy | Download

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

Copy | Download

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

Copy | Download

@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}
}

Copy | Download