RT Journal Article
SR Electronic
T1 Revisiting the Pathophysiology of Intracranial Hemorrhage in Fetuses with Chiari II Malformation: Novel Imaging Biomarkers of Disease Severity?
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
DO 10.3174/ajnr.A8331
A1 Shi, Hui
A1 Prayer, Daniela
A1 Kienast, Patric
A1 Khalaveh, Farjad
A1 Tischer, Johannes
A1 Binder, Julia
A1 Weber, Michael
A1 Stuempflen, Marlene
A1 Kasprian, Gregor
YR 2024
UL http://www.ajnr.org/content/early/2024/08/22/ajnr.A8331.abstract
AB BACKGROUND AND PURPOSE: Intracranial hemorrhage (ICH) has emerged as a notable concern in Chiari II malformation (CM II), yet its origins and clinical implications remain elusive. This study aims to validate the in utero prevalence of ICH in CM II and investigate contributing factors, and visualize the findings in a network format.MATERIALS AND METHODS: A single-center retrospective review of fetal MRI scans obtained in fetuses with CM II (presenting January 2007 to December 2022) was performed for ICH utilizing EPI-T2* blood-sensitive sequence. Fetuses with aqueduct stenosis (AS) were included as a control group. The incidence of ICH and corresponding gestational ages were compared between CM II and AS cases, and morphometric measurements (inner/outer CSF spaces, posterior fossa, venous structure) were compared among the 4 1:1 age-matched groups: CM II+ICH, CM II-ICH, AS+ICH, and AS-ICH. Additionally, a co-occurrence network was constructed to visualize associations between phenotypic features in ICH cases.RESULTS: A total of 101 fetuses with CM II and 90 controls with AS at a median gestational age of 24.4 weeks and 22.8 weeks (P = .138) were included. Prevalence of ICH in fetuses with CM II was higher compared with the AS cases (28.7% versus 18.9%, P = .023), accompanied by congested veins (deep vein congestion mainly in young fetuses, and cortical veins may also be affected in older fetuses). ICH was notably correlated with specific anatomic features, essentially characterized by reduced outer CSF spaces and clivus-supraocciput angle. The co-occurrence network analysis reveals complex connections including bony defects, small posterior fossa dimensions, vermis ectopia, reduced CSF spaces, as well as venous congestion and venous sinus stenosis as pivotal components within the network.CONCLUSIONS: The high prevalence of ICH—detected by fetal MRI—among fetuses with CM emphasizes the pathophysiologic importance of venous congestion, ICH, and vasogenic edema. As indicators of disease severity, these features may serve as helpful additional imaging biomarkers for the identification of potential candidates for fetal surgery.ASaqueduct stenosisCM IIChiari type II malformationICHintracranial hemorrhagePCAprincipal component analysisSSFPsteady-state free precessionSSSFEsingle-shot fast spin-echo