Dental management of Klippel–Trenaunay Syndrome

Klippel–Trenaunay Syndrome (KTS) is a rare complex congenital disorder, characterized by two or more of the following: (1) capillary malformations (port-wine stains), (2) varicose veins or venous malformations (3) soft tissue or bony hypertrophy (or both). It is usually diagnosed in early childhood and has severe long-term sequelae. The orofacial region is rarely involved; however, a broad scale of dento-orofacial abnormalities may require specialized dental and anesthetic management. We are reporting a case of a 3-year-old Saudi girl with KTS, reviewing the literature, and outlining medical complications and dental considerations of this syndrome.