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REVIEW ARTICLE
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 15-19

Familial chiari malformation


Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Correspondence Address:
Krishnakumar Kesavapisharady
Professor, Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joss.joss_32_21

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Chiari malformation type 1 (CM 1) is defined as herniation of cerebellar tonsils 4-5 mm below the foramen magnum. Multiple developmental factors like defective development of pontine flexure and shallow posterior fossa are the proposed factors which predispose the development of this syndrome. These reports on familial occurrence of this syndrome correlates with the embryonal- developmental etiology of Chiari 1 malformation. Recent advances in the understanding of the influence of genes and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.


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