VCI is present in several monogenic disorders (associated genes in parentheses): cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, CADASIL (NOTCH 3); hereditary variants of CAA (APP, CYSTATIN C, and other genes); sickle-cell disease (HBB and other hemoglobin genes); Fabry disease (GLA); homocystinuria (CBS and other genes) [37]. The gene discussed is NOTCH3; the disease is diffuse lymphatic malformation.