Mixed vascular deformities of the lower limbs, with particular reference to lymphography and surgical treatment

A series of patients with congenital blood and lymph anomalies of the lower limb investigated and treated at St Thomas's Hospital, London, are reviewed. They fell into three classes: (1) those in which the venous element predominated (Klippel and other syndromes), (2) those with arteriovenous shunts and (3) those with angiomas of blood or lymph vessels scattered through the limb (diffuse mixed angiomas). Most of the patients were investigated by angiography (of blood or lymph systems) as well as by plethysmography, dermal temperature measurements and other techniques in the thermal laboratory. Phlebography showed most abnormalities in the Klippel group and was useful in delineating them before operation. The importance of confirming the existence of an adequate deep venous circulation prior to ablation of abnormal superficial vessels is emphasized. Arteriography showed most abnormalities in the group with suspected arteriovenous shunts. The most commonly performed operations in this group were for control of overgrowth of the limb or for ulceration. Lymphography showed many of the Klippel group to suffer from insufficiency of the main pathways, either aplasia or hypoplasia. In addition many had vesicles, fistulas and lymph cysts. Patients in the arteriovenous shunt group had large hyperplastic lymph pathways, which were possibly either congenital or a hypertrophic response. One hundred and thirty-eight operations were performed in 46 patients for a variety of lesions and disabilities. These are reviewed. The scope and benefit of surgery in these children are greater than has been accepted in the past. Three patients required amputation of a limb. There were 5 deaths in the series, 4 of these being in the scattered angioma group and in patients in whom the deformities extended beyond the limb into the trunk.