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August 1974

Picture of the Month

Author Affiliations

From the Clinica Pediatrica Universita, Catania, Italy.

Am J Dis Child. 1974;128(2):213-214. doi:10.1001/archpedi.1974.02110270087017

Denouement and Discussion 

Klippel-Trenaunay-Weber Syndrome (Angioosteohypertrophy) 

Manifestations  Major manifestations include cutaneous hemangiomas, varicosities, and soft tissue and bony enlargement (Fig 1-3). The cutaneous vascular lesions may be present at birth or occur later in life. They are usually unilateral and involve the lower limb, although they occasionally appear in other areas. They consist of hemangiomas or may have a port-wine stain appearance. The varicose veins generally are not present at birth but appear within the first few years of life. They vary in size from small ectasias of the veins to very large thickened varicosities that may show phleboliths.Other vascular abnormalities may include arteriovenous fistulas and absence of the deep venous channels. The osteohypertrophy is also usually not present at birth but appears within the first few months or years of life and is almost always in the same area as the vascular abnormalities. There may be periods of

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