Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature

Obande Joseph Orinya; Offiong Uduak Mayen; Jimoh Abdullahi Onimisi

doi:doi:10.11648/j.js.20160402.12

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Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature

Obande Joseph Orinya, Offiong Uduak Mayen, Jimoh Abdullahi Onimisi

Received: 3 February 2016 Accepted: 21 February 2016 Published: 19 March 2016

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Abstract

Vein of Galen malformations and Fahr’s syndrome are rare neurological disorders. Their neurological manifestations may parallel, as is some radiological presentations in certain scenario. They may be congenital but that may be as far as their similarities go as they are pathologically distinct entities with clearly defined natural courses. The unusual parallel of their characteristic neuroradiological findings in the setting of vein of Galen malformations could becloud diagnosis, due to unfamiliarity with the manifestations as a result of their rarity. Our patient presented post-partial thrombosis of vein of Galen malformation. Neuroimaging showed typical intracranial calcifications of the basal ganglia, subcortical white matter, thalami and cerebellum. The radiological report identified the vein of Galen malformation but also noted that Fahr’s syndrome should be ruled out. The occurrence of intracranial calcifications in the setting of vein of Galen malformation is not a usual event, and normally, in the literatures, vein of Galen malformation is not listed as a cause of multiple intracranial calcifications. Therefore, in such a setting, diagnostic dilemma may occur. We described this patient and reviewed the current literature to clarify mechanism of intracranial calcification formation in vein of Galen malformation.

DOI	10.11648/j.js.20160402.12
Published in	Journal of Surgery (Volume 4, Issue 2, April 2016)
Page(s)	13-19
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

Calcifications, Vein of Galen, Fahr’s Syndrome, Aneurysm, Neurocognition

References

[1]	Pablo F. Recinos, Gazanfar Rahmathulla, Monica Pearl, Violette Renard Recinos, George I. Jallo, Phillipe Gailloud, Edward S. Ahn. Vein of Galen Malformations: Epidemiology, Clinical Presentation, Management. Neurosurgery Clinics of North America, vol. 23, No 1, January 2012. doi: 10.1016/j.nec.2011.09.006.
[2]	Ahad MA, Bala C, Karim S: Fahr’s syndrome. Bangladesh Medical Journal Khulna 2013, 45(1-2): 33-35.
[3]	Bilateral Striopallidodentate Calcinosis. http: //www.orpha.net/consor/cqi-bin/OC Exp.php?
[4]	Manyam BV, Walters AS, Narla KR: Bilateral Striopallidodentate Calcinosis: clinical characteristics of patients seen in a registry. Movement disorders: official journal of the Movement Disorder Society 2001, 16(2): 258-264.
[5]	Ellie E, Julien J, Ferrer X: Familial idiopathic striopallidodentate calcifications. Neurology 1989, 39(3): 381-385.
[6]	Shafaq Saleem, Hafiz Muhammad Aslam, Maheen Anwar, Shahzad Anwar, Maria Saleem, Anum Saleem and Muhammad Asim Khan Rehmani: Fahr’s Syndrome: literature review of current evidence. Orphanet Journal of Rare Diseases 3013, 8: 8: 156 doi: 10.1186/1750-1172-8-156.
[7]	Yilmaz Kiroglu, Cem Calli, Nevzat Karabulut, Cagatay Oncel. Intracranial Calcifications on CT. Diagn Interv Radiol 2010; 16: 263-269.
[8]	Lasjaunias PL, Chng SM, Satchet M, Alvarez H, Rodesch G, Garcia-Monaco R. the Management of Vein of Galen Aneurysmal Malformations. Neurosurgery 59: S3-184-S3-194, 2006 doi: 10.1227/neu.0000237445.39514.16.
[9]	Alvarez H, Monaco G, Rodesch G, Satchet M, Krings T, Lasjaunias P. Vein of Galen aneurismal Malformations. Neuroimaging Clin N Am 2007; 17. 189-206.
[10]	Blaise VJ, William SB, Thomas AT, Justin M. and Kerry RC. Vein of Galen Aneurysmal Malformation: Diagnosis and Treatment of 13 Children with Extended Clinical Follow-up. American Journal of Neuroradiology 2002 23: 1717-1724.
[11]	Multiple intracranial calcifications; In, Handbook of neurosurgery, Greenberg MS (Ed); 7^th edition (2010). New York. Thieme.
[12]	Ai-Hsien L, Derek A, and Karel G. Endovascular Treatment of Vein Galen Aneuyrsmal Malformation: management strategy and 21-year experience in Toronto. J Neurosurg Pediatrics 7: 3-10, 2011.

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APA Style

Obande Joseph Orinya, Offiong Uduak Mayen, Jimoh Abdullahi Onimisi. (2016). Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature. Journal of Surgery, 4(2), 13-19. https://doi.org/10.11648/j.js.20160402.12

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ACS Style

Obande Joseph Orinya; Offiong Uduak Mayen; Jimoh Abdullahi Onimisi. Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature. J. Surg. 2016, 4(2), 13-19. doi: 10.11648/j.js.20160402.12

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AMA Style

Obande Joseph Orinya, Offiong Uduak Mayen, Jimoh Abdullahi Onimisi. Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature. J Surg. 2016;4(2):13-19. doi: 10.11648/j.js.20160402.12

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@article{10.11648/j.js.20160402.12,
  author = {Obande Joseph Orinya and Offiong Uduak Mayen and Jimoh Abdullahi Onimisi},
  title = {Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature},
  journal = {Journal of Surgery},
  volume = {4},
  number = {2},
  pages = {13-19},
  doi = {10.11648/j.js.20160402.12},
  url = {https://doi.org/10.11648/j.js.20160402.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.js.20160402.12},
  abstract = {Vein of Galen malformations and Fahr’s syndrome are rare neurological disorders. Their neurological manifestations may parallel, as is some radiological presentations in certain scenario. They may be congenital but that may be as far as their similarities go as they are pathologically distinct entities with clearly defined natural courses. The unusual parallel of their characteristic neuroradiological findings in the setting of vein of Galen malformations could becloud diagnosis, due to unfamiliarity with the manifestations as a result of their rarity. Our patient presented post-partial thrombosis of vein of Galen malformation. Neuroimaging showed typical intracranial calcifications of the basal ganglia, subcortical white matter, thalami and cerebellum. The radiological report identified the vein of Galen malformation but also noted that Fahr’s syndrome should be ruled out. The occurrence of intracranial calcifications in the setting of vein of Galen malformation is not a usual event, and normally, in the literatures, vein of Galen malformation is not listed as a cause of multiple intracranial calcifications. Therefore, in such a setting, diagnostic dilemma may occur. We described this patient and reviewed the current literature to clarify mechanism of intracranial calcification formation in vein of Galen malformation.},
 year = {2016}
}

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TY - JOUR
T1 - Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature
AU - Obande Joseph Orinya
AU - Offiong Uduak Mayen
AU - Jimoh Abdullahi Onimisi
Y1 - 2016/03/19
PY - 2016
N1 - https://doi.org/10.11648/j.js.20160402.12
DO - 10.11648/j.js.20160402.12
T2 - Journal of Surgery
JF - Journal of Surgery
JO - Journal of Surgery
SP - 13
EP - 19
PB - Science Publishing Group
SN - 2330-0930
UR - https://doi.org/10.11648/j.js.20160402.12
AB - Vein of Galen malformations and Fahr’s syndrome are rare neurological disorders. Their neurological manifestations may parallel, as is some radiological presentations in certain scenario. They may be congenital but that may be as far as their similarities go as they are pathologically distinct entities with clearly defined natural courses. The unusual parallel of their characteristic neuroradiological findings in the setting of vein of Galen malformations could becloud diagnosis, due to unfamiliarity with the manifestations as a result of their rarity. Our patient presented post-partial thrombosis of vein of Galen malformation. Neuroimaging showed typical intracranial calcifications of the basal ganglia, subcortical white matter, thalami and cerebellum. The radiological report identified the vein of Galen malformation but also noted that Fahr’s syndrome should be ruled out. The occurrence of intracranial calcifications in the setting of vein of Galen malformation is not a usual event, and normally, in the literatures, vein of Galen malformation is not listed as a cause of multiple intracranial calcifications. Therefore, in such a setting, diagnostic dilemma may occur. We described this patient and reviewed the current literature to clarify mechanism of intracranial calcification formation in vein of Galen malformation.
VL - 4
IS - 2
ER -

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Author Information

Obande Joseph Orinya

Division of Neurosurgery, Department of Surgery, University of Abuja Teaching Hospital, Gwagwalada-Abuja, Nigeria
Offiong Uduak Mayen

Neurology Unit, Department of Paediatrics, University of Abuja Teaching Hospital, Gwagwalada-Abuja, Nigeria
Jimoh Abdullahi Onimisi

Division of Neurosurgery, Department of Surgery, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria

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APA Style

Obande Joseph Orinya, Offiong Uduak Mayen, Jimoh Abdullahi Onimisi. (2016). Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature. Journal of Surgery, 4(2), 13-19. https://doi.org/10.11648/j.js.20160402.12

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ACS Style

Obande Joseph Orinya; Offiong Uduak Mayen; Jimoh Abdullahi Onimisi. Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature. J. Surg. 2016, 4(2), 13-19. doi: 10.11648/j.js.20160402.12

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AMA Style

Obande Joseph Orinya, Offiong Uduak Mayen, Jimoh Abdullahi Onimisi. Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature. J Surg. 2016;4(2):13-19. doi: 10.11648/j.js.20160402.12

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@article{10.11648/j.js.20160402.12,
  author = {Obande Joseph Orinya and Offiong Uduak Mayen and Jimoh Abdullahi Onimisi},
  title = {Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature},
  journal = {Journal of Surgery},
  volume = {4},
  number = {2},
  pages = {13-19},
  doi = {10.11648/j.js.20160402.12},
  url = {https://doi.org/10.11648/j.js.20160402.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.js.20160402.12},
  abstract = {Vein of Galen malformations and Fahr’s syndrome are rare neurological disorders. Their neurological manifestations may parallel, as is some radiological presentations in certain scenario. They may be congenital but that may be as far as their similarities go as they are pathologically distinct entities with clearly defined natural courses. The unusual parallel of their characteristic neuroradiological findings in the setting of vein of Galen malformations could becloud diagnosis, due to unfamiliarity with the manifestations as a result of their rarity. Our patient presented post-partial thrombosis of vein of Galen malformation. Neuroimaging showed typical intracranial calcifications of the basal ganglia, subcortical white matter, thalami and cerebellum. The radiological report identified the vein of Galen malformation but also noted that Fahr’s syndrome should be ruled out. The occurrence of intracranial calcifications in the setting of vein of Galen malformation is not a usual event, and normally, in the literatures, vein of Galen malformation is not listed as a cause of multiple intracranial calcifications. Therefore, in such a setting, diagnostic dilemma may occur. We described this patient and reviewed the current literature to clarify mechanism of intracranial calcification formation in vein of Galen malformation.},
 year = {2016}
}

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