What is K-T syndrome?

Last Thursday, we saw a child with K-T syndrome (Klippel-Trenaunay syndrome) at the Difficult Disease Consultation Center with Professor Daniel Porter. The history was clear: the lower limb on the affected side developed thicker and longer due to congenital anomalous hyperplasia of the arterial vessels in the affected thigh and excessive blood supply. The parents sought help from the orthopedic department with unequal limbs. Usually, unequal limbs in children are caused by hip diseases and epiphyseal injuries, and they appear to be shorter on the affected side. So I almost got it backwards when I translated it at first. However, the father of the child, who has been a doctor for a long time, mentioned K-T syndrome, and Professor Porter understood it immediately. K-T syndrome is a congenital peripheral vascular disease. In this case, the abnormal growth of the femoral artery on one side led to the overgrowth of one limb, for which there is no specific cure. Generally with growth and development, limb inequality will aggravate, the affected side of this child is currently longer 1.8cm, can still shoe inserts correct, does not affect the daily movement. The full-length x-ray of the lower limb should be reviewed every six months, and when the difference is 3cm, surgery should be considered. There are two methods of surgery, epiphyseal fusion or internal fixation to inhibit bone growth. The traditional approach is epiphyseal fusion, which is simple and reliable, but requires the surgeon to grasp the timing of surgery. In recent years, internal fixation to inhibit bone growth has become popular in China, specifically by placing a total of four plates and screws in the distal femur and proximal tibial epiphysis on the affected side to limit bone growth. The reasons for this were threefold: 1. the presence of the epiphyseal plate and the inherent force of continued growth against the screw, which often breaks the nail. 2. failure of inhibition, often requiring a second operation to fuse the epiphysis. 3. the cost and length of the procedure, which may require multiple operations. The child’s father, who had sought help from top specialists at several prestigious hospitals, found Professor Porter’s account very thorough and trustworthy and said he would come for follow-up every six months.