Tubbs R Shane, Bui Cuong J, Rice William C, Loukas Marios, Naftel Robert P, Holcombe Michael Paul, Oakes W Jerry
Section of Pediatric Neurosurgery, University of Alabama at Birmingham and Children's Hospital, Alabama 35233, USA.
J Neurosurg. 2007 Mar;106(3 Suppl):196-200. doi: 10.3171/ped.2007.106.3.196.
Occasional comments are found in the literature regarding patients with lipomyelomeningocele and concomitant Chiari malformation Type I (CM-I). The object of this study was to explore the association between these two conditions.
The authors performed a retrospective database analysis of lipomyelomeningocele cases to identify cases of concomitant CM-I. Analysis of posterior fossa volume (based on the Cavalieri principle) was performed in all identified cases in which appropriate neuroimages were available, and the results were compared with those obtained in age-matched controls. Seven (13%) of 54 patients with lipomyelomeningocele were found to also have CM-I. Two of these were symptomatic (cervicothoracic syrinx and occipital headaches) and required posterior fossa decompression. No correlation was found between the amount of hindbrain herniation and the level of the conus medullaris or the type of lipomyelome-ningocele (for example, caudal or transitional). Volumetric studies of the posterior fossa revealed normal age-matched volumes in all but one patient (who had asymptomatic CM-I).
The incidence of CM-I in patients with lipomyelomeningocele appears to be significantly greater than that of the general population and the association rate is too high for the finding to be a chance occurrence. Decreases in the volume of the posterior cranial fossa were not found in the majority of patients in this small cohort; therefore, the cause of the concomitant occurrence of lipomyelomeningocele and CM-I remains undetermined. Clinicians should consider obtaining imaging studies of the entire neuraxis in patients with lipomyelomeningoceles and should investigate other causes for syringes found in association with lipomyelomeningoceles.
文献中偶尔会提及患有脂肪脊髓脊膜膨出并伴有Ⅰ型Chiari畸形（CM-I）的患者。本研究的目的是探讨这两种病症之间的关联。
作者对脂肪脊髓脊膜膨出病例进行了回顾性数据库分析，以确定伴有CM-I的病例。对所有可获得合适神经影像的确诊病例进行了后颅窝容积分析（基于卡瓦列里原理），并将结果与年龄匹配的对照组进行比较。54例脂肪脊髓脊膜膨出患者中有7例（13%）也患有CM-I。其中2例有症状（颈胸段脊髓空洞症和枕部头痛），需要进行后颅窝减压。未发现后脑疝的程度与脊髓圆锥水平或脂肪脊髓脊膜膨出的类型（例如，尾侧型或过渡型）之间存在相关性。后颅窝的容积研究显示，除1例患者（患有无症状CM-I）外，所有患者的后颅窝容积与年龄匹配的正常容积相符。
脂肪脊髓脊膜膨出患者中CM-I的发生率似乎明显高于一般人群，且关联率过高，不太可能是偶然发生。在这个小队列中的大多数患者中未发现后颅窝容积减小；因此，脂肪脊髓脊膜膨出与CM-I同时发生的原因仍未确定。临床医生应考虑对脂肪脊髓脊膜膨出患者进行全神经轴的影像学检查，并应调查与脂肪脊髓脊膜膨出相关的脊髓空洞症的其他原因。
