Functional aspects on the Klippel-Trénaunay and related syndromes.

Eight patients with two or three symptoms of the Klippel-Trénaunay triad were examined. All patients had varices, increased chloride concentration of sweat and, with one exception, hemangiomas. Four patients showed osteohypertrophy and one patient revealed signs of a-v fistulas. Venous outflow impairment as in deep venous occlusion is known to produce osteohypertrophy in children. The authors are of the opinion that a hemangioma actually is a functional a-v fistula. A hemangioma located in the vicinity of an epiphyseal zone may well cause a localized outflow impairment of the veins draining the epiphyseal zone, leading to an increased growth of the bone. Although patients with the Klippel-Trénaunay triad, Parkes-Weber syndrome and agenesis of the deep veins constitute an etiologically heterogeneous group, they do have some pathophysiological features in common. Treatment would profit by a classification in terms of the pathophysiology rather than the syndrome. The following scheme is therefore suggested: I: Infantile varices with a-v shunts, (a) true arteriovenous shunts, (b) functional arterio-venous shunts (hemangioma) II: Infantile varices with venous obstruction; (a) venous agensis, (b) deep venous phlebitis.

Hemodynamic influence of multiple congenital arteriovenous fistulas. Report of a case.

A 16-year-old girl complained of sensations of weight, swelling warmth and freqeunt sweating from her right arm and hand. Varicose veins were seen on the dorsal aspect of her right hand, her right arm was longer than the left. Since birth she had a capillary hemangioma involving her right shoulder. Right arm and aortocervical arteriograms disclosed large arteriovenous fistulas. Cardiac output was markedly increased. No cardiac enlargement was seen at X-ray examination. The prognosis and future management of the patient are discussed.

Temporary arterio-venous shunts to dilate saphenous crossover graft and maintain graft patency.

A modification of the Palma operation is described in a 25-year-old man with impaired venous outflow of the right leg. After a phlebitic occlusion of the right superficial femoral and external iliac veins he had been operated on twice for varicose veins. The result of these operations was a serious outflow stasis of the right leg during exercise. A saphenous cross-over graft to the right popliteal vein was constructed. Preoperatively a temporary arterio-venous shunt between the left posterior tibial artery and the great saphenous vein had been made in order to increase the diameter of the saphenous vein. Three months later the dilated saphenous vein was resected at the level of the sapheno-tibial artery shunt and anastomosed to the popliteal vein of the right leg. The cross-over graft occluded several times during this operation. A temporary popliteo-popliteal arterio-venous shunt was established distally to the sapheno-popliteal anastomosis to keep the vein graft patent. This second arterio-venous shunt was resected after three months. Venography one month later showed that the vein graft was patent. The patient's complaints had disappeared one month after the operation and a normalization of his venous outflow was recorded plethysmographically. The graft has remained patent during an observation time of eighteen months.