What exactly is “K-T” syndrome?

The full name of K-T syndrome is “Klippel-Trenaunay-Syndrome”, which was first reported by French doctors Klippel and Trenaunay in 1900 and named as “Venous Hyperplasia Bone Hypertrophy Syndrome”. In 1900, French doctors Klippel and Trenaunay firstly reported this disease and named it as “venous hyperplasia osteohypertrophy syndrome”, therefore, people called it as “K-T” syndrome by using the abbreviation of their surnames; in 1907, Parker-Weber found that there was congenital arteriovenous fistula on the basis of the above mentioned lesions, and since then, people called the cases with arteriovenous fistula as “K-T-W” syndrome. “K-T-W syndrome. Nowadays, the above two categories are collectively referred to as “K-T” syndrome. It is a congenital limb-oriented vascular disease characterized by developmental malformations of the superficial and deep veins, capillary hemangiomas (nevi) and/or cavernous hemangiomas, with or without the formation of arteriovenous fistulae, and soft-tissue and osteoarticular hyperplasia. Its main manifestations are: superficial vein walking abnormalities and superficial varicose veins; thickening and growth of the limbs due to overgrowth; multiple cutaneous hemangiomas (nevi) and/or cavernous hemangiomas. It may also be accompanied by manifestations of lymphangiomas, heart failure, gangrene of the limbs, intractable skin ulcers, vaginal or colonic bleeding, and thromboembolism. At present, this kind of disease is still an international therapeutic problem, the existing treatment modalities are: a. Conservative physical therapy: mainly used for light, too young, can not implement the operation or postoperative effect is not good, the main way to wear medical compression stockings or elastic bandage, reduce the affected limb bruising state, may make the child patient’s limb to delay excessive growth, so that the adult patient’s affected limb intractable ulceration incidence of reduced; b. Conservative drug therapy: for children patients, the limb is gangrene, skin ulcers, vaginal or intestinal bleeding, thrombus, etc., Conservative drug treatment: oral hormone therapy is mainly applied to children when the lesion develops, and some patients can effectively inhibit the development of the lesion, and adult patients can take oral drugs such as calcium hydroxybenzenesulfonate, MaiZhiLing, AiXingLang, and Triclabrutin to reduce the swelling of the symptomatic treatment; c. Surgical treatment: since deep vein malformation is one of the characteristics of the disease, and the abnormal mesodermal vascular development is the basic background of the disease, there is not a single surgical procedure that can target the cause of the disease, and there are no other surgical procedures that can be applied to the disease. c. Surgery: since deep venous malformation is one of the characteristics of this disease, mesodermal vascular development abnormality is the basic background of this disease, so there is not a kind of surgery that can target the cause of this disease, and the surgical methods applied in the clinic at present are only for rebuilding the deep venous return paths, and are not recommended to be the first choice of the treatment. The location and distribution of vascular malformations and arteriovenous fistulas can be clearly shown under angiography, which is like drawing a map for the operator, guiding the guide wire manipulated by the operator to reach the abnormal part along the blood vessel, and then interrupting the traffic between arteriovenous veins and sealing off abnormal malformed veins through local injection of embolizing agent or placing steel wire ring embolization or placing overlay stent, etc., so as to achieve the therapeutic goal, and it has been proved clinically that this emerging surgical method is safe, minimally invasive, effective, and gives maximum preservation of function to the affected limbs. Clinically, it has been proved that this emerging surgical method is safe, minimally invasive, effective, with maximum preservation of the function of the affected limb, and can be operated repeatedly, which makes it a more respected treatment method at present. However, due to the histoembryological and pathological characteristics of this disease, the medium- and long-term efficacy of various treatment modalities is not yet very satisfactory, and all of them have a very high recurrence rate. Regrettably, the current medical means can not completely cure this disease, but can only alleviate the symptoms, delay its development and spread, and minimize the impact on the quality of life of the patients.