Wound-Healing Problems Associated with Combined Vascular Malformations in Klippel-Trenaunay Syndrome.

In Klippel-Trenaunay syndrome (KTS), management of a wound in the affected limb can be difficult because of the underlying vascular malformations present. This review describes the characteristics of KTS with wound complications in light of the genetic and molecular mechanisms of the disease. KTS is a slow-flow combined vascular malformation characterized by the triad of capillary malformation, venous malformation with or without lymphatic malformation, and limb overgrowth. KTS is encompassed within the phosphatidylinositol-4,5-bisphosphate3-kinase catalytic subunit alpha ()-related overgrowth spectrum (PROS), having recently been linked to activating mutations in the gene. This clearly has implications for both molecular diagnosis and potential treatment strategies for the disease. KTS should be distinguished from Parkes Weber syndrome, a fast-flow-type combined vascular malformation with limb overgrowth. Individualized management is needed for KTS and should be focused on the treatment of symptoms. Targeted therapies that inhibit the phosphoinositide 3-kinase signaling pathway are a potential treatment option for PROS.