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INTRODUCTION AND IMPORTANCE: Klippel-Trenaunay syndrome (KTS) is characterized by a triad of port-wine stain, varicose veins and soft tissue or bony hypertrophy of lower limb. Varicose veins in Klippel Trenaunay syndrome are mostly distributed in the lateral aspect of the lower limb. The exact etiology of KTS is not known, and the treatment usually starts with conservative management- limb elevation, compression stockings and physiotherapy. However, some cases are severe enough to warrant surgical management. CASE PRESENTATION: Here we present a case of a 3-year-old male child with clinical features suggestive of Klippel Trenaunay Syndrome managed successfully with sclerotherapy of persistent lateral marginal vein of servelle. At one month follow-up the vein was sclerosed and there was a significant reduction in varicosities of leg. CLINICAL DISCUSSION: Starting treatment of varicose veins in Klippel Trenaunay Syndrome in children is effective in preventing long-term complications in adults. Thus, treating venous malformation with sclerotherapy is warranted in early childhood to prevent venous hypertension and chronic venous insufficiency. CONCLUSION: Varicose veins in KT syndrome can be managed successfully by sclerotherapy of lateral marginal vein of servelle resulting in significant reduction in varicosities of leg.
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BACKGROUND: Antiphospholipid syndrome patients have antiphospholipid antibodies (aPLs) that promote thrombosis, and they have increased cardiovascular disease risk. Although the basis for the thrombosis has been well delineated, it is not known why antiphospholipid syndrome patients also have an increased prevalence of nonthrombotic vascular occlusion. The aims of this work were to determine if aPLs directly promote medial hypertrophy or neointima formation in mice and to identify the underlying mechanisms. METHODS AND RESULTS: Medial hypertrophy and neointima formation invoked by carotid artery endothelial denudation were evaluated in mice administered normal human IgG or aPLs. While aPLs had no effect on medial hypertrophy, they caused exaggerated neointima development. This was related to an aPL-induced impairment in reendothelialization post denudation, and scratch assays in cell culture revealed that there are direct effects of aPLs on endothelium that retard cell migration. Further experiments showed that aPL antagonism of endothelial migration and repair is mediated by antibody recognition of ß2-glycoprotein I, apolipoprotein E receptor 2, and a decline in bioavailable NO. Consistent with these mechanisms, the adverse impacts of aPLs on reendothelialization and neointima formation were fully prevented by the NO donor molsidomine. CONCLUSIONS: APLs blunt endothelial repair, and there is related aPL-induced exaggeration in neointima formation after endothelial injury in mice. The initiating process entails NO deficiency mediated by ß2-glycoprotein I recognition by aPLs and apolipoprotein E receptor 2. The modulation of endothelial apolipoprotein E receptor 2 function or NO bioavailability may represent new interventions to prevent the nonthrombotic vascular occlusion and resulting cardiovascular disorders that afflict antiphospholipid syndrome patients.
