Objective
Klippel-Trenaunay Syndrome is a congenital disorder characterized by the cutaneous capillary malformation, deep-seated venous malformation, varicose veins, long bone hypertrophy and other abnormalities. To date, the mainstay of the management of Klippel-Trenaunay Syndrome is medical treatment. Surgical management is only reserved for symptomatic patients refractory to conservative treatment, including recurrent hemorrhage, infections, and repetitive ulcers. We followed up a patient with multiple organ involvement for 10 years, who is currently asymptomatic after several surgical interventions.
Patient and Methods
This is a patient diagnosed with Klippel-Trenaunay Syndrome since her teenage. In the beginning, there was only hemangiolymphangioma of left lower extremity, and she has undergone 4 operations at another hospital before visiting our unit. Due to recurrent infection and bleeding of cutaneous ulcers, we performed 12 times excision of vascular lesions, with the use of tourniquet intraoperatively, of the left lower extremity and bilateral perineum. She started to experience gradual abdominal fullness three years ago because of the splenomegaly, so she received splenectomy with scheduled pneumococcal vaccination at that time. In addition, laparoscopic low anterior resection and loop ileostomy was done last year because of intermittent bloody stool for years, which was caused by multiple mucosal angiodysplasia of the rectum. The loop ileostomy was taken down 4 months later.
Results
So far there was no pulmonary thromboembolism event occurred in the perioperative period, and the pathology results of all operations were compatible with the Klippel-Trenaunay Syndrome. Currently, all lesions in the skin and subcutaneous layer had been removed. Only the minor lesions in the muscles could not be removed completely for the reason to preserve muscle functions. However, the minor lesions in the muscles do not cause any problem of ulcer, bleeding or infection at all.
Conclusions
Surgical management seems to provide satisfactory life quality for the patient with refractory symptoms. To prevent pulmonary thromboembolism event perioperatively, it is important not to exsanguinate the affected limb before the application of tourniquet.