Department of Neurosurgery, University Hospital of North Staffordshire NHS Trust, Stoke-on-Trent, UK
Department of Neurosurgery, University Hospital of North Staffordshire NHS Trust, Stoke-on-Trent, UK.
BMJ Case Rep. 2021 Jun 21;14(6):e241789. doi: 10.1136/bcr-2021-241789.
The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With the increasing availability of magnetic resonance imaging, CM-I is placing a growing burden on neurosurgical services. However, its natural history remains poorly understood, and the timing and nature of surgical intervention is controversial. We present a case of a significant, symptomatic CM-I with associated syrinx which underwent complete spontaneous resolution over a 4-year period. Spontaneous regression of Chiari malformation and syringomyelia is exceedingly rare; a literature review reveals 15 other cases and only one case which underwent complete resolution. The present case and literature review suggest a more benign natural history of CM-I and support a more conservative approach to its management. Further studies are required to determine whether any factors can predict resolution for certain patient cohorts.
Chiari I 畸形(CM-I)的特征是后颅窝拥挤和小脑扁桃体下降,并与脊髓空洞症有关。随着磁共振成像的日益普及,CM-I 给神经外科服务带来了越来越大的负担。然而,其自然病史仍知之甚少,手术干预的时机和性质存在争议。我们报告了一例伴有脊髓空洞症的明显、症状性 CM-I,在 4 年内完全自发消退。Chiari 畸形和脊髓空洞症的自发消退极为罕见;文献回顾显示,还有 15 例其他病例,只有 1 例完全消退。本病例和文献回顾提示 CM-I 的自然病史更为良性,并支持对其进行更保守的治疗。需要进一步的研究来确定是否有任何因素可以预测某些患者群体的消退。