Figure 2. Imaging of sporadic VMs secondary to mutations in MAPK pathway genes demonstrating involvement of all blood vessel sizes.

(A) Lateral image from a digitally subtracted angiogram showing a leash of small, abnormal vessels shunting through a dense capillary bed to early filling veins. (B) Axial contrast-enhanced fat-saturated T1 weighted MRI image showing a grossly enlarged right pinna and thickened posterior auricular soft tissues. The abnormal tissue is filled with multiple signal voids, representing enlarged abnormal vessels. The pinna enhances avidly. (C) Lateral image from a digitally subtracted angiogram, with the catheter tip in the grossly enlarged left internal maxillary artery, which supplies a leash of abnormal high-flow vessels in the face. (D) Thermography of low-flow VMs with overgrowth of the left chest wall and arm demonstrates increased temperature (shown in magenta) compared with the right-sided structures (E), and in the right forearm and thumb compared with the left (F). 3D reconstruction of an abdominal CT angiogram demonstrating multifocal vascular disease, with severe stenoses of the descending aorta, coeliac axis, superior mesenteric artery origin, and right renal artery (E).