A Case of Klippel-weber Syndrome

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概要

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• The symptom complex, consisting varicosity, hemangioma and hypertrophy confined to one side of the extremity is generally called "Klippel-Weber Syndrome"The clinical features, however, are full of varieties.An unusual case of Klippel-weber Syndrome in 9-year-old boy is reported. He has had hypertrophies in his right fingers and both lower extremities, hemangioma in trunk and legs, and naevus verrucosus in right side of the body since his birth. The association of hemangioma and naevus verrucosus is corresponded to Adamson-Best type in Phacomatosis Pigmentovascularis (Ota-Kawamura), and very rare in occurence. Angiographic examination in extremities showed venous varicosities, dilatation of veins and probably arteriovenous fistulae. Histologically naevus verrucosus, hemangioma simplex and lymphangioma were seen in right leg. This case, which had hypertrophies of both extremities is thought to be corresponded to "form alterne S. double" by Klippel & Trenaunay.

• 西日本整形・災害外科学会の論文

著者

• 井上 喜博

  国立大村病院整形外科

• 岩崎 勝郎

  国立大村病院整形外科

• 笹岡 和夫

  国立大村病院皮膚科

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