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Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery

Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms...

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Autores principales: Shin, Hyun-Seung, Kim, Jeong A, Kim, Dong-Seok, Lee, Joon Soo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Pediatric Society 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5177700/
https://www.ncbi.nlm.nih.gov/pubmed/28018469
http://dx.doi.org/10.3345/kjp.2016.59.11.S149
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author Shin, Hyun-Seung
Kim, Jeong A
Kim, Dong-Seok
Lee, Joon Soo
author_facet Shin, Hyun-Seung
Kim, Jeong A
Kim, Dong-Seok
Lee, Joon Soo
author_sort Shin, Hyun-Seung
collection PubMed
description Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention.
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spelling pubmed-51777002016-12-23 Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery Shin, Hyun-Seung Kim, Jeong A Kim, Dong-Seok Lee, Joon Soo Korean J Pediatr Case Report Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention. The Korean Pediatric Society 2016-11 2016-11-30 /pmc/articles/PMC5177700/ /pubmed/28018469 http://dx.doi.org/10.3345/kjp.2016.59.11.S149 Text en Copyright © 2016 by The Korean Pediatric Society http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Shin, Hyun-Seung
Kim, Jeong A
Kim, Dong-Seok
Lee, Joon Soo
Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
title Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
title_full Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
title_fullStr Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
title_full_unstemmed Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
title_short Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
title_sort type i chiari malformation presenting orthostatic syncope who treated with decompressive surgery
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5177700/
https://www.ncbi.nlm.nih.gov/pubmed/28018469
http://dx.doi.org/10.3345/kjp.2016.59.11.S149
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