What are the symptoms of KTS syndrome

KTS is a rare disease. This disease occurs at birth or in infancy. If the disease occurs at this time, the symptoms are not so obvious. Most children will not show symptoms, which may lead to the possibility of misdiagnosis. It will not be discovered until the disease worsens. At this time, relevant examinations should be carried out in time, so that the type of disease can be determined based on the examination results and disease symptoms.

Symptoms

It usually occurs shortly after birth or when the child begins to walk. In Servelle's report, the main clinical manifestations are limb growth, edema, varicose veins, hemangiomas and nutritional disorders. The KTS triad is as follows:

⑴ Vascular nevus or hemangioma: This is the earliest symptom and is mostly discovered at birth or infancy. The typical one is purple or dark purple, flat, dot-shaped intradermal capillary nevus. However, some patients' hemangiomas can develop deeper and invade subcutaneous tissue, muscles, and even the chest and abdominal cavity. The number of vascular nevus on the affected limb varies, and the distribution range is also very different. Generally, they occupy a part of the affected limb, and some are spread throughout the limb, and can even be spread throughout the limb and trunk of the affected side and the healthy limb.

⑵ Tissue hyperplasia: The soft tissue and bone of the affected limb proliferate, making the affected limb thicker and longer. It can be seen in infancy, childhood, adolescence, youth or adulthood. Lesions in the lower limbs are mostly below the knee joints; those with obvious edema and thickening of the thighs are often accompanied by lymphatic system lesions, and in a few patients, the buttocks on the affected side are also enlarged and thickened.

The circumference of the lower limb on the affected side generally increases by 4-5 cm, and in severe cases it can be >15 cm. The length of the affected limb generally increases by 3-4 cm, and in the largest case it can increase by >12 cm. The affected foot is generally larger than the healthy side. X-rays show cortical thickening of the long bones. Servelle believed that limb growth was caused by venous reflux disorder.

⑶ Superficial varicose veins : The affected limbs often have obvious superficial varicose veins, which usually appear within 1 year after birth and become increasingly severe with age. Meyers et al. once divided superficial varicose veins into the most common lumbar-foot type, that is, obvious varicose veins appeared on the lateral side of the affected lower limb. Dodd et al. called it "lateral venous malformation". Normally this vein closes within the second month of fetal development, but in KTS cases it remains open and forms a prominent varicose vein after birth.

Superficial varicose veins are obviously related to reflux obstruction caused by deep vein lesions. If the lesion is located in the popliteal vein, side branches will form around the knee joint. On the inner side, there is an enlarged great saphenous vein, and on the outer side, there are some large veins extending to the joint surface. In addition, there is a large vein in the middle that runs along with the sciatic nerve and drains part of the blood in the gastrocnemius venous plexus. This vein often enters the pelvic cavity through the sciatic and gluteal notches, and finally flows into the internal iliac vein.

When the femoral vein is occluded or compressed by a fibrous tissue band, the compensatory collateral branches can not only expand but also collect venous blood into the deep femoral vein and expand due to the increased blood flow. The sciatic vein merges with the varicose veins from the outer thigh behind the trochanter to form a venous trunk, which is then divided into two branches, entering the pelvic cavity through the sciatic and gluteal notches respectively, and finally introducing a large amount of venous blood into the internal iliac vein. There are several thick communicating branches between the meridian behind the adductor muscle and the sciatic vein. If they are freed and relaxed together with the femoral vein during surgery, a large amount of blood in the femoral venous system on the back of the thigh can flow into the vein on the front of the thigh, greatly reducing the high pressure state of the internal iliac vein.