Klippel-Trénaunay-Weber syndrome associated with abdominal aortic aneurysm in childhood

Klippel-Trénaunay-Weber syndrome (KTWS), also known as angioosteohypertrophy syndrome, is a rare congenital malformation with unknown etiology characterized by the combination of capillary malformations (port-wine strain), venous varicosities, and a soft tissue or bony hypertrophy of the affected limb. It is known to be rarely associated with abdominal aortic aneurysm (AAA) in adults. We report the first published case of KTWS and a rapidly progressing symptomatic AAA undergoing open repair in a child. This underlines the importance of AAA screening and treatment rather than surveillance in patients with KTWS.