Related Experiment Video
Updated: Mar 9, 2026
Microvascular Decompression: Salient Surgical Principles and Technical Nuances
Published on: July 5, 2011
Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
Hyun-Seung Shin1, Jeong A Kim2, Dong-Seok Kim3
1Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea.
Insights
Chiari malformation type I (CM-I) can cause rare symptoms like syncope. Surgical decompression effectively treated a patient with CM-I presenting with orthostatic syncope.
Area of Science:
- Neurology
- Neurosurgery
- Congenital Disorders
Background:
- Chiari malformation type I (CM-I) involves cerebellar tonsil herniation below the foramen magnum.
- CM-I can cause various neurological symptoms due to cervicomedullary compression.
- Syncope is a rare but reported presentation of CM-I.
Purpose of the Study:
- To present a case of orthostatic syncope in a patient with CM-I.
- To discuss the potential mechanisms and treatment of syncope in CM-I.
- To highlight the effectiveness of surgical intervention for this rare presentation.
Main Methods:
- Case report of a patient with CM-I and orthostatic syncope.
- Review of clinical presentation and diagnostic findings.
- Description of surgical decompression and post-operative outcomes.
Main Results:
- The patient presented with recurrent orthostatic syncope.
- Surgical decompression was performed for CM-I.
- Post-surgical intervention led to resolution of syncope and improved clinical status.
Conclusions:
- Orthostatic syncope can be a manifestation of Chiari malformation type I.
- Surgical decompression is a viable treatment option for CM-I associated syncope.
- Further research is needed to elucidate the pathophysiology of syncope in CM-I.
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.
