Milczarek Olga, Kuzaj Julia, Bębenek Adam, Hartwich Patryk, Zgoda-Aleksandrowicz Magdalena, Krzyżewski Roger, Kwinta Borys, Kwiatkowski Stanisław
Department of Children Neurosurgery, Jagiellonian University Medical College, Kraków, Poland.
Department of Intensive Care, Ujastek Medical Center, Kraków, Poland.
World Neurosurg. 2025 May;197:123853. doi: 10.1016/j.wneu.2025.123853. Epub 2025 Mar 5.
Chiari malformation type 2 (CMII) is a herniation of anatomical structures of the posterior fossa due to myelomeningocele (MMC), the most common neural tube defect. Most patients with MMC exhibit radiologic signs of CMII. The study aimed to evaluate radiological parameters of the posterior fossa in patients undergoing surgery for CMII, with the goal of establishing structural indications for surgical treatment to prevent clinical manifestations and life-threatening symptoms.
The study included all constitutive patients with CMII diagnosed between 2016 and 2018. The following measurements were taken and analyzed for associations: size of the fourth ventricle, the McRae line, the depth of tonsillar herniation, the amount of cerebrospinal fluid (CSF) at the C0 level, the presence of syringomyelia, hydrocephalus, ventriculoperitoneal shunt (VPS) implantation, and supracerebellar cistern.
The length of the McRae line and the depth of tonsillar herniation were strongly positively correlated. There was a negative relationship between the length of McRae line and the amount of CSF at the C0 level. There was a strong positive correlation between depth of tonsillar herniation and hydrocephalus. MMC and VPS implantation were associated with a larger depth of tonsillar herniation.
The depth of the tonsillar herniation may not be the most viable finding influencing operative indication due to its dependency on the length of the McRae line. A small amount of CSF at the level of foramen magnum also should not necessarily be considered an early sign of syringomyelia. Ventriculoperitoneal shunt implantation seems not to influence the depth of tonsillar herniation.
2型Chiari畸形(CMII)是由于脊髓脊膜膨出(MMC,最常见的神经管缺陷)导致的后颅窝解剖结构疝出。大多数MMC患者表现出CMII的放射学征象。本研究旨在评估接受CMII手术患者的后颅窝放射学参数,目的是建立手术治疗的结构指征以预防临床表现和危及生命的症状。
本研究纳入了2016年至2018年间确诊的所有CMII患者。进行了以下测量并分析其相关性:第四脑室大小、McRae线、扁桃体疝深度、C0水平脑脊液(CSF)量、脊髓空洞症的存在、脑积水、脑室腹腔分流术(VPS)植入情况以及小脑上池。
McRae线长度与扁桃体疝深度呈强正相关。McRae线长度与C0水平脑脊液量呈负相关。扁桃体疝深度与脑积水呈强正相关。MMC和VPS植入与更大的扁桃体疝深度相关。
由于扁桃体疝深度依赖于McRae线长度,它可能不是影响手术指征的最可行指标。枕骨大孔水平少量脑脊液也不一定应被视为脊髓空洞症的早期征象。脑室腹腔分流术植入似乎不影响扁桃体疝深度。