DOI QR코드

DOI QR Code

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

  • Shin, Hyun-Seung (Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine) ;
  • Kim, Jeong A (Division of Pediatric Neurology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine) ;
  • Kim, Dong-Seok (Department of Neurosurgery, Severance Hospital, Yonsei University College of Medicine) ;
  • Lee, Joon Soo (Division of Pediatric Neurology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine)
  • Received : 2014.09.04
  • Accepted : 2014.10.20
  • Published : 2016.11.15

Abstract

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.

Keywords

References

  1. Dones J, De Jesus O, Colen CB, Toledo MM, Delgado M. Clinical outcomes in patients with Chiari I malformation: a review of 27 cases. Surg Neurol 2003;60:142-7. https://doi.org/10.1016/S0090-3019(03)00131-9
  2. Mueller DM, Oro' JJ. Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. J Am Acad Nurse Pract 2004;16:134-8. https://doi.org/10.1111/j.1745-7599.2004.tb00384.x
  3. Tarani L, Del Balzo F, Costantino F, Properzi E, D'Eufemia P, Liberati N, et al. Chiari type I malformation, syncope, headache, hypoglycemia and hepatic steatosis in an 8-year old girl: a causal association? Pediatr Rep 2010;2:e8.
  4. Bindal AK, Dunsker SB, Tew JM Jr. Chiari I malformation: classification and management. Neurosurgery 1995;37:1069-74. https://doi.org/10.1227/00006123-199512000-00005
  5. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 1999;44:1005-17. https://doi.org/10.1097/00006123-199905000-00042
  6. Prilipko O, Dehdashti AR, Zaim S, Seeck M. Orthostatic intolerance and syncope associated with Chiari type I malformation. J Neurol Neurosurg Psychiatry 2005;76:1034-6. https://doi.org/10.1136/jnnp.2004.048330
  7. Garland EM, Robertson D. Chiari I malformation as a cause of orthostatic intolerance symptoms: a media myth? Am J Med 2001;111:546-52. https://doi.org/10.1016/S0002-9343(01)00922-6
  8. Stewart JM. Update on the theory and management of orthostatic intolerance and related syndromes in adolescents and children. Expert Rev Cardiovasc Ther 2012;10:1387-99. https://doi.org/10.1586/erc.12.139
  9. Lahrmann H, Cortelli P, Hilz M, Mathias CJ, Struhal W, Tassinari M. EFNS guidelines on the diagnosis and management of orthostatic hypotension. Eur J Neurol 2006;13:930-6. https://doi.org/10.1111/j.1468-1331.2006.01512.x
  10. Medow MS, Stewart JM, Sanyal S, Mumtaz A, Sica D, Frishman WH. Pathophysiology, diagnosis, and treatment of orthostatic hypotension and vasovagal syncope. Cardiol Rev 2008;16:4-20. https://doi.org/10.1097/CRD.0b013e31815c8032
  11. Straus D, Foster K, Zimmerman F, Frim D. Chiari drop attacks: surgical decompression and the role of tilt table testing. Pediatr Neurosurg 2009;45:384-9. https://doi.org/10.1159/000260909
  12. Mangubat EZ, Wilson T, Mitchell BA, Byrne RW. Chiari I malformation associated with atlanto-occipital assimilation presenting as orthopnea and cough syncope. J Clin Neurosci 2014;21:320-3. https://doi.org/10.1016/j.jocn.2013.03.004