Angiomatosis Osteohypotrophica

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Abstract
Based on a clinical and radiological study of 7 patients, a syndrome consisting of congenital hemangiomas with arteriovenous fistulae engaging the soft tissues and the bones, and associated with shortening of the involved extremity, is reported. The name angiomatosis osteohypotrophica is suggested for this entity. Angioma, varicose veins and/or arteriovenous fistulae with resulting increase in limb have been widely known since the beginning of this century as Klippel-Trenaunay syndrome. Although the patients in this series present all other features of Klippel-Trenaunay syndrome, thinning and shortening of the long bone occurred, resulting in an overall reduction in limb length. Intraosseous hemangiomas in the metacarpal and respective metatarsal bones were common characteristic findings. Arteriograms showed presence of arteriovenous shunts within the area of the tumor in addition to hemangiomas. The influence of arteriovenous shunts on skeletal growth is discussed.