RESUMEN
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.
RESUMEN
Vascular malformations are developmental anomalies occurring due to defective vasculogenesis. Depending on the number of vessels involved, they are subgrouped into simple or combined types. Combined vascular malformations are a rare clinical entity with two or more vascular malformations (capillary, venous, arteriovenous, lymphatic) present in one lesion. Due to the complexity of these lesions, clinicians should employ an interdisciplinary approach with multi-staged treatment for the proper management. Here, we report a case of a lady with a combined vascular malformation in the right side of the neck who presented to our department with a complaint of progressively increasing swelling for three months. A multidisciplinary team of vascular surgeons, interventional radiologists and physiotherapists were involved in its treatment. Initially, Doppler ultrasonography was performed, which revealed a vascular lesion with arterial and venous components and a flow void region suggestive of lymphatic malformation, further confirmed by magnetic resonance imaging (MRI). A multistage treatment modality was employed where intralesional steroid was initially administered in the lesion area to shrink the vessel's size. Next, venous malformation targeted sclerotherapy was performed preoperatively, followed by intraoperative ligation of the feeding vessel and excision of remaining malformation. Postoperatively the patient had no wound site complication and was discharged on the fourth postoperative day with advice to follow rehabilitative neck exercises. One month follow-up revealed complete resolution of the malformation. Thus, in the case of combined vascular malformation, multistage treatment modalities with a multidisciplinary team should be employed for proper treatment.
