Patients with Klippel-Trenaunay syndrome

Abstract

Klippel-Trenaunay syndrome is the most frequent systemic venous angiodysplasia. It is characterized by cutaneous capillary malformations -naevus fl ammeus, excessive growth of soft and bone tissue, venous and lymphatic malformations. Investigative methods include: clinical examinations, venography as the evidence of dysplastic changes of superfi cial and / or deep venous system, the Moyne obstruction and venous insuffi ciency perforator. Treatment is conservative, related to that of chronic venous disease. The surgery is aimed at removing the varices and insuffi cient perforators (Fig. 9, Ref. 8 Clinical study 139-years old woman patient was admitted to the Clinic of vascular surgery, East Slovak Institute of Cardiovascular Diseases, Inc. in Kosice for persistent swelling of the left tibia, progression of relapsing varicose veins of the left tibia with the tendency to deterioration. In anamnesis she overcame usual childhood diseases, surgery of varicose veins in the left lower limb at the local surgical department, in the dispensary of angiologist and orthopaedist for spine scoliosis. For a long time she used venotonics and anticoagulant therapy with low molecular heparin (LMWH).Within the family there were no problems with varicose veins. At admission, on the shin on the right there were extensive stem varicosities, pre-tibial and perimalleolar oedema.Pulsations were bilaterally well tangible at full range, trophic changes of the skin and adnexes were not present. In the sacral region there was haemangioma of the size of 10x10 cm (Fig. 1).The whole left lower limb was thicker by 3-4 cm. The left foot was signifi cantly smaller than the right one, with skeletal malformation (Figs 2 and 3).In the control ultrasound of the lower limb vein system the fi nding on the left lower limb was following: v. saphena magna as well as v. saphena parva were extirpated, without any signs of recurrence, perforators on the tibia were suffi cient. On the dorsal side of the thigh the cranial extension protruding from the stump of the extirpated v. saphena parva was confi rmed by examination. It was signifi cantly dilated with the luminal diameter of 13.5 mm.Extension was drained by dilated perforator in the middle of dorsomedial side of the thigh running into v. femoralis l.sin.In the preoperative examination the normochromatic anaemic syndrome of slight degree v.s. sideropenic, leucopaenia of slight degree, and slight hypercholesterolemia dominated in the laboratory picture. According to the examination conclusion, the patient was cardio-pulmonary compensated without any contraindications to surgery.Under the total anaesthesia in the position on the abdomen, the stump of v. saphena parva l.sin was anastomized at its distal and proximal extension. The distal extension was fi brotically changed, probably after previous surgery. The proximal one was about 0.7 cm, fi brotically changed after previous infl ammations. After its skeletinization in the middle third of the back half of the thigh we ligated its tertiary part in the...

Diversity and frequency of symptoms in Klippel–Trénaunay syndrome

“…Malformations of this type can result in other distortions of bone and cartilage tissue, such as scoliosis, osteoporosis and osteopenia, limb axis disorders, joint contractures [5], lower [11] and upper [19] limb syndactyly. Soft tissue overgrowth and connected deformations can sometimes affect the exterior genital area, where it can result in cryptorchidism, phimosis and problems with micturition [12].…”

“…to help affected patients and raising their quality of life. Further studies on the clinical manifestation of syndrome help in relieving their complaints, for example, by surgical interventions in varicose veins treatment [19], as well as compensation of limb disparities by epiphysiodesis [9]. Large disproportions and development remain in the literature data collected over the years of research of KTS, perhaps pointing to a need of more detailed, uniform studies on the topic.…”