What is KT syndrome? Characteristics and manifestations of KT syndrome

KT syndrome is a genetic disease characterized by abnormal vascular development in patients, which is mainly manifested in three aspects: nevus-like telangiectasia, varicose veins, and hyperplasia of soft and bone tissue. The symptoms of KT syndrome mainly manifest in the child's lower limbs. Below, I will give you a detailed introduction to the relevant knowledge of KT syndrome for your reference!

1. What is KT syndrome?

Klippel-Trenaunay syndrome is a congenital peripheral vascular disease. It is mainly a manifestation of congenital vascular dysplasia.

2. Three major characteristics of KT syndrome:

1. Nevus-like telangiectasia;

2. Varicose veins;

3. Hyperplasia of soft tissue and bone tissue

This disease is a congenital vascular malformation and its onset has nothing to do with living environment. Some manifestations of KT syndrome, such as port-wine stains, can be found in infancy and are often considered birthmarks. Medical treatment is not sought until the lesions worsen and one side of the limb grows and thickens.

3. KT syndrome manifestations

It mainly manifests in the limbs, especially the lower limbs. Some lesions involve the buttocks, waist, lower abdomen, or shoulders, usually involving one limb. The main manifestations are:

1. The occurrence site of superficial varicose veins in the lower limbs is different from that of general superficial varicose veins in the lower limbs. The veins are mainly concentrated on the outer side of the thigh or buttocks, and the bulge is generally not obvious.

2. Port wine stains are a kind of slightly raised reddish or purple-red nevus in the shape of a map. It can fade when pressed. In fact, it is an intradermal vascular nevus, which is a characteristic manifestation of the disease and is often mistaken for a birthmark by parents.

3. Growth or thickening of one limb As the child grows, the limb on the affected side will gradually grow and thicken

4. The skin temperature of the affected limb is increased. By comparing the temperature of the limbs on both sides, it can be found that the temperature of the affected limb is slightly higher. You can touch the limb with the back of your hand to feel the slight difference in skin temperature.

Due to vascular malformation, the affected limb is supplied with too much blood, and the limb may grow longer and thicker than the opposite limb due to soft tissue and bone hypertrophy. In severe cases, the child may have limp due to the different lengths of the lower limbs on both sides. Long-term limp will affect the development of the spine and form scoliosis. The imbalance of force on both hip joints may cause hip strain. In some cases, the arteriovenous fistula is close to the trunk, which leads to ischemia in the distal limbs, manifested as cool, pale or purple limb extremities. Severe cases may cause blackening and necrosis of the extremities. Varicose veins may develop thrombosis due to slow blood flow, resulting in thrombosis of superficial thrombotic phlebitis, manifested as redness, swelling and pain along the course of the veins, increased local skin temperature and pain when touched.