Poretti Andrea, Ashmawy Ramy, Garzon-Muvdi Tomas, Jallo George I, Huisman Thierry A G M, Raybaud Charles
Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.
Department of Neuroradiology, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Neuropediatrics. 2016 Oct;47(5):293-307. doi: 10.1055/s-0036-1584563. Epub 2016 Jun 23.
Our understanding of cerebellar tonsillar herniation evolved over time and nowadays various pathomechanisms have been proposed. Causes of tonsillar herniation share a discrepancy between content (fore- and hindbrain) and container (supratentorial cranial vault, posterior fossa), may be associated with abnormalities of the craniocervical junction, and may have a developmental or acquired nature. In tonsillar herniation, the hindbrain is not malformed but deformed. Accordingly, "Chiari type 1 deformity," not "Chiari type 1 malformation" is the correct term to characterize primary tonsillar herniation. Chiari type 1 deformity is commonly seen in pediatric neurology, neuroradiology, and neurosurgery and may have various clinical presentations depending on patient age. In addition, Chiari type 1 deformity is increasingly found by neuroimaging studies as an incidental finding in asymptomatic children. An accurate and reliable selection of patients based on clinical and neuroimaging findings is paramount for the success of neurosurgical treatment. Future studies are needed to provide selection criteria with a higher sensitivity and specificity.
我们对小脑扁桃体疝的认识是随着时间逐渐发展的，如今已提出了多种发病机制。扁桃体疝的病因在于内容物（前脑和后脑）与容器（幕上颅腔、后颅窝）之间的差异，可能与颅颈交界区的异常有关，且可能具有发育性或后天性。在扁桃体疝中，后脑并非畸形而是变形。因此，“Chiari 1型畸形”，而非“Chiari 1型发育异常”，才是描述原发性扁桃体疝的正确术语。Chiari 1型畸形在儿科神经学、神经放射学和神经外科中较为常见，根据患者年龄可能有多种临床表现。此外，通过神经影像学研究越来越多地发现，Chiari 1型畸形在无症状儿童中是偶然发现。基于临床和神经影像学结果准确可靠地选择患者对于神经外科治疗的成功至关重要。未来需要开展研究以提供具有更高敏感性和特异性的选择标准。
