Angiomatosis osteohypotrophica

Based on a clinical and radiological study of seven 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, for which we suggest the name angiomatosis osteohypotrophica, is reported. Angioma, varicose veins and/or arteriovenous fistulae with resulting increase in limb have been widely known since the beginning of this century as Klippel-Trénaunay syndrome. Although the patients in this series present all other features of Klippel-Trénaunay syndrome, thinning and shortening of the long bones occurred, resulting in an overall reduction in limb length. Intraosseous hemangiomas in the metacarpal, respective metatarsal bones were common characteristic findings. The arteriograms undertaken in this study showed, in addition to hemangiomas, presence of arteriovenous shunts within the area of the tumour. Their influence on skeletal growth is discussed.