Variants of collateral cerebral circulation in moyamoya disease

Abstract

Moyamoya disease is a chronic progressive cerebrovascular disease with a complex pathophysiology and unique features of neoangiogenesis. These features are still known only to a few specialists, although they determine clinical course and outcomes of disease. To determine the nature and degree of neoangiogenesis in restructuring the natural collateral circulation in patients with moyamoya disease and its effect on cerebral blood flow. The influence of collateral circulation on postoperative results and factors of its effectiveness will be analyzed in the 2nd part of the study. The study included 65 patients with moyamoya disease who underwent preoperative selective direct angiography with separate contrast enhancement of both internal, external and vertebral arteries. We analyzed 130 hemispheres. Suzuki stage of disease, pathways of collateral circulation and their relationship with reduction of cerebral blood flow and clinical manifestations were assessed. Distal vessels of the middle cerebral artery (MCA) were additionally studied. Suzuki stage 3 was the most common (36 hemispheres, 38%). Leptomeningeal collaterals were the most common among intracranial collateral tracts (82 hemispheres, 66.1%). Extra-intracranial transdural collaterals were found in half of the cases (56 hemispheres). We observed certain changes in distal vessels of the MCA (hypoplasia of M3 branches) in 28 (20.9%) hemispheres. Suzuki stage of disease significantly determined degree of cerebral blood flow insufficiency, i.e. more severe perfusion deficit was observed at the later stages of disease. A well-developed system of leptomeningeal collaterals significantly reflected stages of compensation and subcompensation of cerebral blood flow according to perfusion data (χ2=20.394, p<0.001). Neoangiogenesis is a natural compensatory mechanism in moyamoya disease designed to maintain brain perfusion under reduced cerebral blood flow. Predominant intra-intracranial collaterals are associated with ischemic and hemorrhagic events. Timely restructuring on extra-intracranial ways of collateral circulation prevents adverse manifestations of disease. Assessment and understanding of collateral circulation in patients with moyamoya disease create the prerequisites for substantiating the method of surgical treatment.