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Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

机译：儿童朗格汉斯细胞组织细胞增生症的中枢神经系统成像-参考中心分析

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Background. The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation.Methods. We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed.Results. Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (?o 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary.Conclusions. The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials.
机译：背景。我们研究的目的是（1）根据发送到参考中心并满足最低要求的图像来描述患有Langerhans细胞组织细胞增生症（LCH）的儿童的中枢神经系统（CNS）表现，以及（2）评估评估者之间的协议中枢神经系统核磁共振成像的结果，代表了这项研究的总体可重复性。我们回顾性地回顾了LCH患儿的MRI最低要求得到的脑MRI检查。由两名经验丰富的神经放射科医生对异常情况进行评分，并评估评分者之间的一致性。在总共94项影像学研究中，只有31项MRI符合最低标准，包括至少两个不同剖面上对比之前/之后的T2w，FLAIR，T1w图像，以及穿过蝶鞍的对比后矢状薄层T1w。最常见的变化是骨异常，然后是松果体的坚实增大，增粗的增强茎和齿状核的信号变化。尽管评估大多数中枢神经系统病变的评估者之间的共识相对较高（？o> 0.61），但采用最低标准常常无法评估垂体后叶。来自不同机构的放射线规程的多样性导致LCH儿童中枢神经系统异常的诊断困难。尽管神经放射医师之间的评分者之间的共识很高，但使用最低标准时，并非所有LCH表现都可以完全排除。因此，脑部MRI应该遵循LCH指南方案，并包括T1 d前矢状位图像，并进行集中检查以改善临床试验的比较。

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