Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review Rodríguez Blanque, Raquel Piqueras Sola, Beatriz Sánchez García, Juan Carlos Cortés Martín, Jonathan Arnold Chiari Syndrome Arnold Chiari malformation Posterior cranial fossa Syringomyelia Hydrocephalus Scoliosis We are grateful to the Official College of Nursing of Granada (CODEGRA) for their help in the research support program and to the Chair of Research in Nursing Care of the University of Granada and the Official College of Nursing of Granada. Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa. 2023-11-28T13:37:33Z 2023-11-28T13:37:33Z 2023-10-23 info:eu-repo/semantics/article Rodríguez-Blanque, R.; Almazán-Soto, C.; Piqueras-Sola, B.; Sánchez-García, J.C.; Reinoso-Cobo, A.; Menor-Rodríguez, M.J.; Cortés-Martín, J. Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review. J. Clin. Med. 2023, 12, 6694. [https://doi.org/10.3390/jcm12206694] https://hdl.handle.net/10481/85904 10.3390/jcm12206694 eng http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess Atribución 4.0 Internacional MDPI