The collateral circulation in pediatric moyamoya disease

Purpose The descriptions of collateral circulation in moyamoya have so far been a mixture of topography-based and vessels’ source-based analyses. We aimed to investigate the anatomy and systematize the vascular anastomotic networks in pediatric moyamoya disease. Methods From a series of 25 consecutive complete angiographic studies of newly diagnosed children with moyamoya, 14 children had moyamoya disease and 11 were diagnosed with moyamoya syndrome, i.e., moyamoya angiopathy with some additional concomitant systemic disease. We retrospectively analyzed the arterial branches supplying the moyamoya anastomotic networks, their origin, course, location, and connections with the recipient vessels. Results We describe four types of anastomotic networks in children with moyamoya disease, two superficial-meningeal and two deep-parenchymal. As superficial-meningeal, we defined the leptomeningeal and the durocortical networks. Apart from the previously described leptomeningeal network observed in the convexial watershed zones, we report on the basal temporo-orbitofrontal leptomeningeal network. The second superficial-meningeal network is the durocortical network, which can be basal or calvarian in location. We define as deep-parenchymal networks the nonpreviously described subependymal network and the inner striatal and inner thalamic networks. The subependymal network is fed by the intraventricular branches of the choroidal system and diencephalic perforators, which at the level of the periventricular subependymal zone, anastomose with medullary—cortical arteries as well as with striatal arteries. The inner striatal and thalamic networks are constituted by intrastriatal connections among striatal arteries and intrathalamic connections among thalamic arteries when the disease compromises the origin of one or more sources of their supply. Conclusion The previously inexplicitly described “moyamoya abnormal network” in pediatric moyamoya disease can be described as a composition of four anastomotic networks with distinct angioarchitecture. A better understanding of the collateralization in moyamoya may help in defining a new staging system of the disease with clinical relevance.