Relationship Between Intracranial Calcifications in Vein of Galen Malformations and Fahr’s Syndrome: A Case Report and Review of the Literature
Journal of Surgery
Volume 4, Issue 2, April 2016, Pages: 13-19
Received: Feb. 3, 2016;
Accepted: Feb. 21, 2016;
Published: Mar. 19, 2016
Views 4749 Downloads 108
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
Follow on us
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.
Calcifications, Vein of Galen, Fahr’s Syndrome, Aneurysm, Neurocognition
To cite this article
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, Journal of Surgery.
Vol. 4, No. 2,
2016, pp. 13-19.
Copyright © 2016 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/
) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Ahad MA, Bala C, Karim S: Fahr’s syndrome. Bangladesh Medical Journal Khulna 2013, 45(1-2): 33-35.
Bilateral Striopallidodentate Calcinosis. http: //www.orpha.net/consor/cqi-bin/OC Exp.php?
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.
Ellie E, Julien J, Ferrer X: Familial idiopathic striopallidodentate calcifications. Neurology 1989, 39(3): 381-385.
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.
Yilmaz Kiroglu, Cem Calli, Nevzat Karabulut, Cagatay Oncel. Intracranial Calcifications on CT. Diagn Interv Radiol 2010; 16: 263-269.
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.
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.
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.
Multiple intracranial calcifications; In, Handbook of neurosurgery, Greenberg MS (Ed); 7th edition (2010). New York. Thieme.
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.