Chen Jason A, Lazareff Jorge A
Department of Neurosurgery, David Geffen School of Medicine at UCLA, 18-210 Semel, Box 957039, Los Angeles, CA, 90095-7039, USA,
Childs Nerv Syst. 2014 Mar;30(3):531-4. doi: 10.1007/s00381-013-2250-1. Epub 2013 Aug 7.
Chiari malformations are characterized by hindbrain herniation. Historically, some types have been linked to neural tube defects, but the causal relationship between the two conditions is still unclear.
We report on a full-term male neonate with a prenatally diagnosed posterior thoracic meningocele, whose MRI demonstrated Chiari malformation. The meningocele was closed in the second week of life. The Chiari-related symptoms rapidly improved following surgery. Postoperative MRI at 7 months of age showed ascent of the cerebellar tonsils to a normal position.
This case suggests that the traction on the cerebellum by a fixed spinal cord may play a role in the pathogenesis of the multifaceted Chiari malformations.
Chiari 畸形的特征是后脑疝。从历史上看,某些类型与神经管缺陷有关,但这两种情况之间的因果关系仍不清楚。
我们报告了一名足月男性新生儿,产前诊断为胸段后位脊膜膨出,其 MRI 显示 Chiari 畸形。脊膜膨出在出生后第二周进行了闭合。与 Chiari 相关的症状在手术后迅速改善。7 个月大时的术后 MRI 显示小脑扁桃体上升至正常位置。
该病例表明,固定脊髓对小脑的牵拉可能在多方面的 Chiari 畸形发病机制中起作用。