Section Editor: Sandy Cheng-Yu Chen, M.D.
Taipei Medical University Hospital, Taipei, Taiwan
Sturge-Weber Syndrome (SWS) is a congenital phakomatosis characterized by vascular malformations involving the forehead cutaneous capillaries (“port wine stain”), choroid (resulting in glaucoma) and pia mater. SWS is thought to be due to abnormal venous development causing chronic venous ischemia resulting in CNS and cutaneous vascular malformations. Patients most commonly present with seizures which are often refractory to medical therapy. CT reveals calcifications (A, white arrows). There is also associated ipsilateral choroid plexus enlargement (A and B, yellow arrow). MRI additionally reveals atrophy of the adjacent brain parenchyma and prominent pial angiomatosis which is enhanced with contrast injection (A and C, red arrows). The calcification can also be shown by the susceptibility-weighted image (D, arrow).