The Klippel–Trenaunay syndrome: Clinical, radiological and haemodynamic features and management

Abstract

Forty-nine patients with the Klippel-Trenaunay syndrome have been studied. Sixty-eight per cent have a superficial, embryological venous channel on the lateral aspect of the limb, 25 per cent have had one or more severe spontaneous haemorrhages from dilated varices and 22 per cent have suffered a venous thrombo-embolism. Twenty-nine per cent have had episodes of rectal bleeding or haematuria associated with pelvic angiomas. Foot volumetry (n = 31) showed that calf emptying was decreased in only five patients and only one of these had phlebographic evidence of deep venous obstruction, which conflicts with previous findings. The rate of calf refilling was increased in 81 per cent and phlebography demonstrated incompetent communicating veins in 45 per cent of patients. No patient had clinical evidence of an arteriovenous fistula, and arteriography (n = 22) and calf blood flow (n = 33) were normal, but lymphangiography (n = 14) showed lymphatic hypoplasia in 55 per cent of limbs. Eighty-eight operations have been performed on 38 patients but symptoms persist in 90 per cent. We suggest that surgery should be limited to the excision of localized symptomatic abnormalities and that the best form of control is provided by graduated compression stockings.

Klippel Trenaunay Syndrome, Inverse Klippel Trenaunay Syndrome: Hypertrophy of Lower Limbs and Atrophy of the Upper Limbs and Facial Muscles: Case Report and Literature Review

“…However, others reported prominent superficial varicose veins are present in a majority of patients with Klippel Trenaunay Syndrome [9,26]. In over two-thirds of patients, a characteristic incompetent lateral venous channel arises near the ankle and extends a variable distance up the extremity to the infrainguinal or pelvic deep venous system [27]. This venous malformation frequently present as persistent embryonic veins, of which the lateral marginal vein (the vein of Servelle) has been the most typical finding, which found in 68-80% of patients [28].…”

“…The prevalence of deep venous aplasia or hypoplasia, as detected with venographic techniques (ascending venography and varicography), ranges from 18% [27] to 40% [26]. Lymphatic malformations have also been common in up to 70% of cases [1], that include primary lymphaedema, cystic hygroma or lymphangiectasia associated with reflux of chyle [29].…”

“…The bony abnormalities may affect all bones in an extremity or limited to one or two bones. The enlargement of the extremity consists of bone elongation or circumferential soft-tissue growth of the affected limb [9,27]. Hypertrophy is caused by local hyperemia and secondary venous stasis [32] which are attributed to arteriovenous shunting and venous anomaly [33].…”

“…The cutaneous vascular lesion is generally a capillary malformation and usually involves the enlarged limb, although involvement of the whole side of the body or of the contralateral limb may be seen [9,27].In addition, other bone deformities can be seen such as macrodactyly, syndactyly, split hand deformity, phalangeal agenesis and hip joint dislocation [35]. Soft tissue hypertrophy may be limited to a localized mass on the back or chest, or it can be diffuse involving an entire arm or leg [29].…”

“…Local complications include Extremity pain, stasis dermatitis, cellulitis, ulceration and spontaneous cutaneous hemorrhage. Chronic venous insufficiency, coagulopathy, thrombosis or thrombophlebitis are commonly encountered in KTS [9,27,32]. Gangrene can occur as a result of thrombophlebitis.…”