Klippel-Trenaunay syndrome

What is Klippel-Trenaunay syndrome?

Klippel-Trenaunay syndrome (KTS) is a rare congenital (present at birth) vascular anomaly that results in your child having a large number of abnormal blood vessels. Doctors can sometimes see evidence of KTS in a prenatal ultrasound, and the capillary malformation (in the form of a "port-wine stain" birthmark) is almost always apparent at the baby’s birth. KTS is equally common in boys and girls.

How we care for Klippel-Trenaunay syndrome

The Vascular Anomalies Center (VAC) at Boston Children's specializes in innovative, family-centered care for children with KTS. From your first visit, you’ll work with a team of professionals who are committed to supporting all your family’s physical and psychosocial needs. KTS is a progressive condition, which means that it will grow as your child grows. That said, many kids — especially those with mild cases of KTS — go on to live normal, healthy lives. Treatment for your child’s symptoms is the most effective way to manage the disease.

Children with KTS sometimes have legs so large that it becomes debilitating, forcing them to use a wheelchair. Surgeons at Boston Children's are leading the charge in removing the extra tissue that grows as a result of KTS. In what's called a debulking procedure, our surgeons remove a great deal of the extra tissue, which can allow your child to regain leg function.

Our areas of innovation for Klippel-Trenaunay syndrome

KTS is caused by errors that occur when blood and lymphatic vessels are forming. The Vascular Anomalies Center conducts research that may lead to the development of new, more effective treatments and perhaps ultimately result in ways to prevent KTS.

Our investigators are currently probing the genes and molecules that regulate the formation and growth of blood and lymphatic vessels. We hope that understanding the genes that control these molecular events will result in new therapies for vascular malformations.