Klippel-Trenaunay-Weber Syndrome (cont.)

Medical Author:

David Perlstein, MD, MBA, FAAP

David Perlstein, MD, MBA, FAAP

Dr. Perlstein received his Medical Degree from the University of Cincinnati and then completed his internship and residency in pediatrics at The New York Hospital, Cornell medical Center in New York City. After serving an additional year as Chief Pediatric Resident, he worked as a private practitioner and then was appointed Director of Ambulatory Pediatrics at St. Barnabas Hospital in the Bronx.

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Medical Editor:

William C. Shiel Jr., MD, FACP, FACR

William C. Shiel Jr., MD, FACP, FACR

Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

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In this Article

• What is Klippel-Trènaunay-Weber (KTW) syndrome?
• What is a port-wine stain?
• What is asymmetrical limb hypertrophy?
• Are there other abnormalities in Klippel-Trènaunay-Weber syndrome?
• How is intelligence affected?
• What is the basic defect in Klippel-Trènaunay-Weber syndrome?
• What causes Klippel-Trènaunay-Weber syndrome?
• What is the prognosis (outlook) with Klippel-Trènaunay-Weber syndrome?
• Why is it called Klippel-Trènaunay-Weber syndrome?
• Alternative names for Klippel-Trènaunay-Weber (KTW) syndrome
• Klippel-Trenaunay-Weber Syndrome Index

In 1900 the French physicians Maurice Klippel and Paul Trènaunay reported the case of a patient with a port-wine stain, asymmetrical overgrowth (hypertrophy) of soft tissue and bone together with hemangiomas of the skin In 1907, the eminent London physician F. Parkes Weber reported three more cases, and noted the varicosities. As is often the case in medicine, those who describe a syndrome are often given credit by having the syndrome named after them. Thus, in 1918 it became know as Klippel-Trènaunay-Weber syndrome.

Alternative names for Klippel-Trènaunay-Weber (KTW) syndrome include angio-osteohypertrophy syndrome, congenital dysplastic angiectasia, elephantiasis congenita angiomatosa, hemangiectatic hypertrophy, Klippel-Trenaunay- Parkes-Weber syndrome (Parkes was Weber's middle name), Klippel Trenaunay syndrome, KTS, and osteohypertrophic nevus flammeus.