Adamsbaum Catherine, Moutard Marie Laure, André Christine, Merzoug Valérie, Ferey Solène, Quéré Marie Pierre, Lewin Fanny, Fallet-Bianco Catherine
Department of Radiology, St Vincent de Paul Hospital, 82 avenue Denfert Rochereau, 75674 Paris Cedex 14, France.
Pediatr Radiol. 2005 Feb;35(2):124-40. doi: 10.1007/s00247-004-1316-3. Epub 2004 Nov 23.
MRI is a useful tool to complement US for imaging of the fetal posterior fossa (PF). In France, the discovery of a PF malformation in the fetus frequently leads to termination of pregnancy (80% in a personal series). However, despite improved accuracy in the diagnosis of PF abnormalities, prognosis remains uncertain. The first objective of this review is to document the normal MRI landmarks of the developing fetal PF. Because of their thinness, the visibility of the cerebellar fissures is dramatically delayed on MRI compared to macroscopic data. An important landmark is identification of the primary fissure of the vermis, normally seen at around 25-26 weeks' gestation (WG) on the sagittal slice, separating the larger posterior lobe from the anterior lobe (volume ratio around 2:1). The prepyramidal and secondary fissures are usually only identifiable after 32 WG and the hemispheric fissures are difficult to see until the end of pregnancy. Considering the signal changes, high signal on T2-weighted (T2-W) sequences is seen from 25 WG in the posterior part of the brain stem (tegmentum and ascending sensory tracts) related to myelination. The low signal intensities seen within the cerebellum on T2-W images correspond to high cellularity of grey matter (deep nuclei), as there is no myelination within the white matter before 38 WG. The second objective is to highlight the signs highly predictive of a poor neurological prognosis. Lack of pontine curvature or vermian agenesis without a PF cyst (small volume of PF) is greatly associated with poor neurological status. The third objective is to propose a diagnostic strategy in difficult cases where prognosis is important, e.g. the Dandy Walker continuum. Analysis of the cerebellum is often impossible if a PF cyst is present (whatever its nature) as the mass effect usually blurs the foliation and even impairs evaluation of the normal ratio between the posterior and anterior lobes of the vermis. Isolated cerebellar hypoplasias raise the question of prognosis and genetic counselling. Such uncertainties require an amniocentesis and a careful search for other anomalies (cerebral and extracerebral). Unilateral abnormalities of a cerebellar hemisphere can be associated with good neurological status if they are isolated. The final objective is to discuss other rare PF fetal abnormalities, such as vascular malformations and tumours.
磁共振成像(MRI)是一种辅助超声对胎儿后颅窝(PF)进行成像的有用工具。在法国,胎儿后颅窝畸形的发现常常导致终止妊娠(个人系列研究中为80%)。然而,尽管后颅窝异常的诊断准确性有所提高,但其预后仍然不确定。本综述的首要目的是记录发育中胎儿后颅窝的正常MRI标志。由于小脑裂很薄,与宏观数据相比,其在MRI上的显示明显延迟。一个重要标志是蚓部初级裂的识别,在矢状面上通常在妊娠25 - 26周左右可见,它将较大的后叶与前叶分开(体积比约为2:1)。锥体前裂和次级裂通常在妊娠32周后才能识别,而半球裂直到妊娠末期才难以看清。考虑信号变化,在T2加权(T2 - W)序列上,从妊娠25周起,脑干后部(被盖和上升感觉束)出现高信号,这与髓鞘形成有关。T2 - W图像上小脑内的低信号强度对应于灰质(深部核团)的高细胞密度,因为在妊娠38周前白质内没有髓鞘形成。第二个目的是强调高度预示神经预后不良的征象。脑桥曲度缺失或无后颅窝囊肿(后颅窝体积小)的蚓部发育不全与神经功能不良密切相关。第三个目的是针对预后重要的疑难病例提出诊断策略,例如丹迪 - 沃克连续体。如果存在后颅窝囊肿(无论其性质如何),通常由于占位效应会使小脑叶片模糊,甚至影响对蚓部后叶与前叶正常比例的评估,因此往往无法对小脑进行分析。孤立性小脑发育不全引发了预后和遗传咨询的问题。这些不确定性需要进行羊水穿刺并仔细寻找其他异常(脑内和脑外的)。小脑半球的单侧异常如果是孤立的,可能与良好的神经功能状态相关。最后一个目的是讨论其他罕见的胎儿后颅窝异常,如血管畸形和肿瘤。