Speaker
Description
Background: PHACES syndrome is regarded as a rare condition with associated disorders that affects multiple parts of the child's organism. The presence of this syndrome can be identified through a range of manifestations, including cardiac and arterial anomalies, ocular anomalies or even dermatological issues. The risk of cerebrovascular accident (CVA) resulting from PHACES syndrome is multifactorial, although the mechanistic principles remain unclear. The current literature lacks information on the potential influence of arteriopathy on the risk of CVA. Consequently, the primary objective of this research was to assess the impact of arteriopathy present in PHACES syndrome in order to identify a possible reason for CVA.
Methods: A spatial model of an arterial system was prepared on the basis of biomedical imaging data. Subsequently, the reference geometry was modified to reflect three scenarios of the internal carotid artery: a) PHACES syndrome (severe tortuosity and high hypoplasia), b) severe tortuosity, c) high hypoplasia. The authors then conducted numerical investigations utilising the fluid-structure interaction (FSI) method to accurately predict flow hemodynamics under pulsatile flow conditions. Additionally, a blood washout study was incorporated into the analysis.
Results: The results of the numerical simulations demonstrated that the simultaneous combination of severe tortuosity and high hypoplasia can result in the creation of a thrombogenic environment, with hypoplasia appearing to be the dominant factor. A notable reduction in blood flow intensity was observed in the affected artery, accompanied by an increase in blood viscosity and a rise in blood stagnation. Furthermore, a considerable increase in the proportion of "non-washed-out" blood was evident in the PHACES syndrome case study.
Conclusions: It has been demonstrated that numerical simulations can facilitate statistical analyses and provide insights into the mechanistic underpinnings of potential cerebral venous sinus thrombosis (CVST) associated with PHACES syndrome. This research elucidated the potential for thrombus formation in multiple regions of the PHACES syndrome geometry, which could potentially detach and obstruct further cerebral arteries, leading to CVST.
