What are the K-T syndrome questions and answers?

1.What is K-T syndrome? Klippel-Trenaunay syndrome, a congenital peripheral vascular disorder, was first reported by French physicians Klippel and Trenaunay in 1900 and named as “varicose veins with hypertrophic vascular nevus of bone”. In addition, there are many other names: Klippel-Trenaunay-Weber syndrome, Weber’s syndrome, Ollierklippel’s syndrome, Trenaunay’s syndrome, Parkes-Weber’s syndrome, vascular bone hypertrophy syndrome, bone hypertrophic capillary hemangioma syndrome, hypertrophic warty nevus, hypertrophic vasodilatation, etc. It is mainly a manifestation of congenital abnormal vascular development and can be generally divided into the following types: ① venous type – mainly venous abnormalities, including superficial varicose veins, venous tumors, deep venous valve insufficiency, deep venous valve defect or deep venous defect; ② arterial type -The arterial type – including arterial blockage, ischemia or abnormal hyperplasia, etc.; arteriovenous fistula type – mainly based on the abnormal arteriovenous fistula of the affected limb; ③ mixed type. 2, the onset of the disease, if congenital, can it be detected at birth? The disease is a congenital vascular malformation, the onset of which is not related to the living environment. some manifestations of K-T syndrome, such as wine-colored spots can be found in infancy, often considered as birthmarks, and only when the lesion is aggravated, the growth and thickening of one side of the limb is seen. Foreign statistics on such diseases show that the average interval from the first detection of lesions to the first examination is 12 or 7 years. 3. Is there any way for parents to observe the disease at home? The main manifestations of the disease are in the extremities, especially in the lower extremities, with some lesions involving the buttocks, waist, lower abdomen or shoulders, usually involving one limb. The main manifestations are: ① superficial varicose veins of the lower limbs, the occurrence of which is different from the general superficial varicose veins of the lower limbs, mainly concentrated in the outer part of the thighs or buttocks, and the veins are usually not obviously elevated; ② wine-colored spots, a kind of slightly elevated light red or purplish-red nevus in the shape of a map, which can fade when pressed, is actually an intradermal vascular nevus, which is the characteristic manifestation of the disease and is often mistaken by parents as a birthmark. ③Growth or thickening of one limb, as the child grows, the limb on the affected side will gradually grow and thicken. ④Increased skin temperature of the affected limb. By comparing the limb temperatures of both sides, it can be found that the temperature of the affected limb is slightly higher. Parents can touch the child’s limb with the back of their hand and feel the slight difference in skin temperature. 4, K-T syndrome harm due to vascular malformation, resulting in excessive blood supply to the affected limb, development than the opposite limb, can appear soft tissue and bone hypertrophy, limb growth thickening, serious will be because of the two sides of the lower limb length, resulting in children lameness, long-term lameness will affect the development of the spine, the formation of scoliosis, bilateral hip joint force imbalance, may cause hip joint strain. In individual cases, because the arteriovenous fistula is close to the trunk, it leads to ischemic changes in the distal limb, which is manifested as coldness, pallor or purple at the end of the limb, and in severe cases, it can lead to blackness and necrosis at the end. The varicose veins can develop thrombosis secondary to slow blood flow, forming thrombotic superficial phlebitis, which manifests as redness, swelling, pain, increased local skin temperature and pain when touched along the venous line. 5, K-T syndrome treatment methods, the approximate cost, there is no domestic authority? There is no special treatment method yet, but mainly symptomatic reduction treatment. If the limb length difference exceeds 1,5cm, the heel of the healthy side can be padded to prevent secondary lesions caused by long-term limping. For the thickening of the affected limb, medical elastic stockings or elastic bandages should be used for compression therapy, which can control varicose veins, reduce the heaviness and swelling of the lower limbs caused by venous stasis, and prevent the occurrence of thrombotic superficial phlebitis. The price of imported medical elastic stockings for treatment is about 300~500 Yuan. For superficial varicose vein with open deep veins and normal venous valve function, local varicose superficial vein stripping or laser treatment can be performed. For patients with deep venous defects, this method is not applicable. For children with arteriovenous fistula or bilateral limbs of unequal length, bilateral arteriograms of lower limbs can be performed first, and if abnormal vessels are found, they can be embolized or surgically ligated in order to retard the development of the affected limbs and reduce claudication; for children who have not stopped development, epiphyseal clips can be used to limit the development of the longer limb so as to reduce the length difference between the two limbs; for children with cavernous hemangioma, hemangioma excision or sclerotherapy can be performed. For children with cavernous hemangioma, hemangioma excision or sclerotherapy can be performed in stages. The cost of surgical treatment is about 10,000 yuan. Currently, many tertiary hospitals with vascular surgery in China are able to provide treatment.