Palumbo Giovanni, Arrigoni Filippo, Peruzzo Denis, Parazzini Cecilia, D'Errico Ignazio, Agazzi Giorgio Maria, Pinelli Lorenzo, Triulzi Fabio, Righini Andrea
Pediatric Radiology and Neuroradiology Department, Children's Hospital V. Buzzi, Via Castelvetro 32, 20154, Milan, Italy.
Neuroimaging Unit, Scientific Institute, IRCCS "Eugenio Medea", Bosisio Parini, Lecco, Italy.
Neuroradiology. 2023 Sep;65(9):1387-1394. doi: 10.1007/s00234-023-03183-0. Epub 2023 Jun 17.
Morphometric studies on idiopathic Chiari malformation type 1 (CM1) pathogenesis have been mainly based on post-natal neuroimaging. Prenatal clues related to CM1 development are lacking. We present pre- and post-natal imaging time course in idiopathic CM1 and assess fetal skull and brain biometry to establish if clues about CM1 development are present at fetal age.
Multicenter databases were screened to retrieve intrauterine magnetic resonance (iuMR) of children presenting CM1 features at post-natal scan. Syndromes interfering with skull-brain growth were excluded. Twenty-two morphometric parameters were measured at fetal (average 24.4 weeks; range 21 to 32) and post-natal (average 15.4 months; range 1 to 45) age; matched controls were included.
Among 7000 iuMR cases, post-natal scans were available for 925, with postnatal CM1 features reported in seven. None of the fetuses presented CM1 features. Tonsillar descent was clear at a later post-natal scan in all seven cases. Six fetal parameters resulted to be statistically different between CM1 and controls: basal angle (p = 0.006), clivo-supraoccipital angle (p = 0.044), clivus' length (p = 0.043), posterior cranial fossa (PCF) width (p = 0.009), PCF height (p = 0.045), and PCFw/BPDb (p = 0.013). Postnatally, only the clivus' length was significant between CM1 cases and controls.
Pre- and post-natal CM1 cases did not share striking common features, making qualitative prenatal assessment not predictive; however, our preliminary results support the view that some of the pathogenetic basis of CM1 may be embedded to some extent already in intrauterine life.
关于特发性1型Chiari畸形（CM1）发病机制的形态学研究主要基于出生后的神经影像学检查。目前尚缺乏与CM1发育相关的产前线索。我们展示了特发性CM1的产前和产后影像学时间进程，并评估胎儿颅骨和脑测量数据，以确定在胎儿期是否存在关于CM1发育的线索。
对多中心数据库进行筛选，以获取出生后扫描显示具有CM1特征儿童的宫内磁共振成像（iuMR）。排除干扰颅骨-脑生长的综合征。在胎儿期（平均24.4周；范围21至32周）和出生后（平均15.4个月；范围1至45个月）测量22个形态学参数；纳入匹配的对照组。
在7000例iuMR病例中，925例有出生后扫描结果，其中7例报告有出生后CM1特征。所有胎儿均未表现出CM1特征。在所有7例病例中，出生后较晚的扫描显示扁桃体下移明显。CM1组和对照组之间有6个胎儿参数在统计学上存在差异：基底角（p = 0.006）、斜坡-枕骨上角（p = 0.044）、斜坡长度（p = 0.043）、后颅窝（PCF）宽度（p = 0.009）、PCF高度（p = 0.045）以及PCFw/BPDb（p = 0.013）。出生后，仅斜坡长度在CM1病例和对照组之间有显著差异。
产前和产后CM1病例没有明显的共同特征，使得定性的产前评估无法预测；然而，我们的初步结果支持这样一种观点，即CM1的一些发病机制基础可能在某种程度上已经存在于宫内生活中。
