Congenital mixed vascular deformities of the lower limb: the relevance of lymphatic abnormalities to their diagnosis and treatment.

Nineteen patients with vascular deformities of the lower extremity were studied and were classified into three groups based on their clinical and angiographic findings. 1) The Klippel or Venous Dysplasia Group (n = 8) demonstrated: cutaneous nevi of metameric distribution, congenital varices (many of which were anomalous by phlebography), hypertrophy of bone and other tissues, but no evidence of arterial involvement by arteriography. 2) Congenital Arterio-Venous Fistulae Group (n = 7) showed: cutaneous angiomata, hypertrophy of bone and soft tissues, but arteriographic findings of single or multiple arterio-venous fistulae. 3) Scattered Angiomata Group (n = 4) had single or multiple angiomata involving the lower limb in particular. Arteriography was normal, while phlbography revealed venous abnormalities. Lymphography was performed on all patients. Six of 8 of the Klippel group demonstrated aplastic or hypoplastic lymphatics by lymphography. By contrast, all 7 of the patients in the Congenital Arterio-Venous Fistulae Group showed in their lymphographic findings-three of 4 of these patients had aplasia or hypoplasia. Fourteen of the 19 patients complained of painful swelling as a major symptom. Lymphatic fistulae and lymphoceles developed in several patients.Four patients underwent reduction operations for lymphedema. Lymphatic abnormalities as demonstrated by lymphography play a significant role in mixed vascular deformities of the lower extremities.