Review of moyamoya disease and syndrome with special consideration of associations with sickle cell disease

Abstract

Moyamoya, a pattern of progressive stenosis of the ICA associated with secondary formation of collateral vessels at the base of the brain, is a rare but well described cerebral vasculopathy. Moyamoya can be idiopathic but is also associated with several genetic disorders, inflammatory conditions and radiation exposure. Despite extensive research on the topic, the underlying mechanisms for how and why Moyamoya develops are not well understood. Moyamoya syndrome in sickle cell disease is one of the more studied subsets of the disease and recent experience with this subtype may have implications for understanding of all types of Moyamoya. Treatment options for Moyamoya are limited, with the mainstays of therapy restricted to surgical revascularization procedures.

Keywords

Moyamoya - Sickle cell disease - hemoglobin SS - vasculopathy