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August 1966

The Angioosteohypertrophy Syndrome: Klippel-Trenaunay-Weber Syndrome

Author Affiliations

From the Birth Defects Center and Department of Pediatrics, University of Texas, Dallas.

Am J Dis Child. 1966;112(2):161-164. doi:10.1001/archpedi.1966.02090110105012

THE ASSOCIATION of vascular abnormalities and trophic changes in soft tissue and bone was described in 1900 by the French authors Klippel and Trenaunay.1 In 1907 Weber2 named the syndrome "Naevus varicosus osteohypertrophicus," or "Hemangiectasia hypertrophicans." Therefore the symptom-triad (1) nevi, usually unilaterally located, (2) varicose veins, and (3) hypertrophy of the soft tissue and bone is referred to as the Klippel-Trenaunay-Weber syndrome. Recently, Tobler (quoted by Stellmach)3 suggested the descriptive term "angioosteohypertrophy."

Aside from the classical triad, different variations exist. They are called "formes frustes" and are divided into neviform, osteohypertrophic, and avaricose types. Servelle4 stated that varicose veins are infrequently present at birth. These occur within the first few years of life and show varying degrees of enlargement. There may be only small ectasias of the veins of the skin, but sometimes these veins may become finger-thick varicosities and may show phleboliths. Fegeler et al5