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.
D000328:Adult; D000368:Aged; D000798:Angiomatosis / Q000503:physiopathology*; D000886:Anthropometry; D001132:Arm / Q000473:pathology; D002675:Child, Preschool; D005121:Extremities / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D007866:Leg / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010690:Phlebography; D010991:Plethysmography; D012039:Regional Blood Flow; D014690:Venous Pressure