Hemodynamic and Hydrodynamic Pathophysiology in Chiari Type 1 Malformations: Towards Understanding the Genesis of Syrinx

Abstract

Background: The pathophysiology of this association of type 1 Chiari malformation (CM1) and syrinxes is still unknown. There is an alteration in the dynamics of neurofluids (cerebrospinal fluid, arterial and venous blood) during the cardiac cycle in CM1. Our objective is to quantify CSF or arterial blood or venous blood flow in patients with Chiari syndrome (CS) with and without syrinxes using phase-contrast MRI (PCMRI). Methods: We included 28 patients with CM1 (9 with syrinxes, 19 without). Morphological MRI with complementary PCMRI sequences was performed. We analyzed intraventricular CSF, subarachnoid spaces CSF, blood, and tonsillar pulsatility. Results: There is a highly significant correlation (p < 0.001) between cerebral blood flow, cerebral vascular expansion volume and venous drainage distribution. Venous drainage distribution is significantly inversely correlated with oscillatory CSF volume at the level of the foramen magnum plane [−0.37 (0.04)] and not significantly correlated at the C2C3 level [−0.37 (0.05)] over our entire population. This correlation maintained the same trend in patients with syrinxes [−0.80 (<0.01)] and disappeared in patients without a syrinx [−0.05 (0.81)]. Conclusion: The distribution of venous drainage is an important factor in intracranial homeostasis. Impaired venous drainage would lead to greater involvement of the CSF in compensating for arterial blood influx, thus contributing to syrinx genesis.