1/10. klippel-trenaunay-weber syndrome presenting as massive lymphangiohemangioma of the thigh: prenatal diagnosis.We report a case of klippel-trenaunay-weber syndrome presenting prenatally as a massive congenital lymphangiohemangioma of the thigh. Routine ultrasonographic examination revealed multiple distorted cystic areas extending from the right flank through the right lower extremity of a 30-week fetus. A diagnosis of cystic lymphangioma of the thigh was suspected prenatally. Neonatal evaluation confirmed the prenatal findings. Neonatal color Doppler imaging revealed blood vessels within the tumor. The differential diagnosis is discussed together with available therapeutic procedures.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/10. klippel-trenaunay-weber syndrome with hydronephrosis and vesicoureteral reflux: an unusual association.The klippel-trenaunay-weber syndrome is a rare disorder characterized by congenital vascular hamartomas, limb hypertrophy, cutaneous manifestations, lymphangiomas and atresia of lymph vessels with non-pitting edema. A three-year-old boy was referred to our clinic for progressive hypertrophy of leg and feet with 32-month history. We diagnosed klippel-trenaunay-weber syndrome, and determined vesicoureteral reflux in our patient. To our knowledge, hydronephrosis and vesicoureteral reflux have not been described previously in the KTWS.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/10. Klippel-Trenaunay syndrome with involvement of coecum and rectum: a rare cause of lower gastrointestinal bleeding.Klippel-Trenaunay syndrome (KTS) is a congenital malformation usually presenting limb asymmetry, abnormal development of the deep and superficial veins, and cutaneous capillary malformations. We describe the case of a 56-year-old male KTS patient who suffered from recurrent but life non-threatening lower gastrointestinal bleeding. colonoscopy revealed multiple extensive cavernous hemangiomas in the coecum and the ascending colon as well as the sigmoid colon and the rectum. MR imaging showed numerous dilated vessels within the left gluteal and inguinal region. The mucosal and the submucosal layers particularly of the sigmoid colon and rectum appeared markedly broadened and displayed high signal intensities in the STIR sequences. Due to only moderate oozing at time of admission the patient was treated with oral iron supplementation so far.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/10. Venous angiomata: treatment with sclerosant foam.Venous angiomata, or venous malformations, are often present at birth, although they may not be evident until later. They consist of a spongy tangle of veins, and these lesions usually vary in size. Treatment of venous angiomata is often requested for cosmetic reasons, but painful ulcerations, nerve compression, functional disability can command care. This presentation describes management using sclerosant foam as the treating agent. During a 30-month period ending March 2004, 1,321 patients were investigated for venous disorders at the Vein Institute of La Jolla. Fourteen (incidence 1%) were found to have venous angiomata (: nine women). The age range was 15-76 years (mean 30.8 /- 18.6). Lesions were classified by the Hamburg system and were primarily venous, extratruncular in 12 patients and combined extratruncular and truncular in two patients. Eight patients, three males, had manifestations of lower extremity Klippel-Trenaunay (syndrome; six had only venous angiomas. Only 10 of the 14 patients were treated. All patients were studied by Doppler duplex examination. Selected lesions were chosen for helical computed tomographic studies. Magnetic resonance venography was also used to image the lesions, define the deep circulation, note connections with normal circulation, identify vessels for therapeutic access, and determine infiltration of the lesion into adjacent soft tissue. Foam was produced by the Tessari two syringes one three-way stopcock teclinique, with the air to Polidocanol ratio being 4 or 5 to 1. This was used at 1% or 2% concentration, specific for each patient. The SonoSite 190 plus Duplex Doppler was used for ultrasound guidance, whenever deep access was required and to monitor progress and effects of treatment. A goal was set for each patient before treatment was begun. Ten patients were treated, and four await treatment. The mean number of treatments was 3.6 /- 2.8 (range 1-10). A primary goal of pain-free healing was set in patients with nonhealing, painful ulceration or symptomatic varicose veins. This was achieved in all treated patients. Cosmetically, all of the patients were improved, and symptomatic patients were relieved of pain. The single complication was formation of a cutaneous ulcer following injection of telangiectasias. Sclerosant foam is a satisfactory tool to use in treating venous angiomata including the Klippel-Trenaunay syndrome. Use of foam sclerotherapy in this experience has proven the technique to be effective, essentially pain-free, and durable in the short term.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/10. Klippel-Trenaunay syndrome with small vessel pulmonary arterial hypertension.A patient with Klippel-Trenaunay syndrome and pulmonary arterial hypertension not associated with chronic thrombo-embolic pulmonary hypertension is described. It is hypothesised that pulmonary arterial hypertension is another complication associated with the Klippel-Trenaunay syndrome, possibly due to haemodynamic changes of small vessel abnormalities.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
6/10. Identification and successful treatment of congenital microfistulas with the aid of directional Doppler.Hitherto unidentifiable and therefore untreatable congenital microfistulas were detected with the aid of continuous-wave directional Doppler and a fine-beam pencil probe. The microfistulas formed part of the Klippel-Trenaunay (K-T) syndrome. The management of one case is reported in detail and in three others salient features are touched upon. Careful clinical and radiological examination failed to demonstrate any arteriovenous microfistulas. When Doppler ultrasound scanning was carried out, two discrete fistulas were discovered. Their extent and direction were mapped out accurately. Incisions were made directly over the markings displaying a pulsating capillary tuft of vessels. Further dissection exposed a feeding arteriole which was less than 1 mm. in diameter. Excision of the vascular malformations resulted in the cure of the patient. In another patient with the K-T syndrome in whom a cutaneous hemangioma involved the whole lower limb, in spite of a thorough and systematic search with a Doppler, no microfistulas could be demonstrated. It is suggested that all patients suffering from the K-T syndrome should be examined by Doppler ultrasound in the hope that microfistulas which elude radiodiagnostic techniques might be detected and treated surgically.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/10. klippel-trenaunay-weber syndrome with spinal arteriovenous malformation--case report.A 28-year-old female with klippel-trenaunay-weber syndrome associated with an arteriovenous malformation (AVM) of the spinal cord is reported. She was admitted to our hospital with a 4-month history of steadily progressive weakness and dysesthesia of the legs. A nevus flammeus, varices, hypertrophy and elongation of the left leg had been present since her infancy. These symptoms progressed and she became unable to walk and pass water by herself. myelography disclosed a spinal AVM extending from Th11 to L2. angiography confirmed the presence of stretched and tortuous vessels as well as an arteriovenous shunt in the left leg. magnetic resonance imaging showed a high-intensity area extending from Th10 to L2. Following laminectomy from Th10 to L2 and resection of the AVM, her motor and bladder functions worsened. However, 6 months later, her motor function improved to the preoperative state and the bladder dysfunction disappeared. The coexistence of klippel-trenaunay-weber syndrome with spinal AVM, considered to be rare, is discussed, and the pertinent literature is reviewed. The case presented here is the first to have been surgically treated in japan.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/10. Klippel and Trenaunay's syndrome. 768 operated cases.Since 1945, we have operated on 786 patients with Klippel and Trenaunay's syndrome. Elongation of the impaired limb was invariably found while edema was present in 84%, varicose veins in 36%, and flat angiomata in 32%. Venography and surgical exploration have demonstrated malformation of the deep veins involving the popliteal vein in 51%; superficial femoral vein, 16%; both popliteal and superficial femoral veins; 29%; iliac veins, three per cent; and lower vena cava, one per cent. Good clinical results have been achieved following the surgical release of these deep veins in the lower limb. During childhood, when the difference in limb length is noteworthy, ligature of the popliteal vein of the shorter limb induces a compensating elongation. Klippel and Trenaunay's syndrome may be associated with lymphatic malformations, including lymphedema and malformation of the lymph vessels. knowledge of the pathophysiology of these malformations of the deep veins enables a better understanding of the clinical manifestations of the condition, as well as the improved treatment of the serious vesical or rectal hemorrhage which occurs in one per cent of these patients.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/10. The Klippel Trenaunay Weber Syndrome presenting with cutaneous bleeding.A patient with the Klippel Trenaunay Weber syndrome presented with prolonged bleeding from the finger tips during strenuous exercise. Tissue fibrinolysis studies showed increased fibrinolytic activity suggesting that the blood vessels in the affected limb may be functionally as well as anatomically abnormal.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/10. Simultaneous occurrence of aneurysm and multiple meningioma in Klippel-Trenaunay patients: case report.Klippel-Trenaunay syndrome is a rare neurocutaneous disorder in which skeletal hypertrophy, vascular nevi, and vessel anomalies coexist. Involvement of the cranial bone is rare, and intracranial anomalies associated with features of this syndrome are exceptional. We report a case of an adult Klippel-Trenaunay woman with a huge cranial hypertrophy harboring of the same time multiple meningiomas and a fusiform intracranial aneurysm. Although meningioma and intracranial aneurysms have been described in Klippel-Trenaunay patients, such an association has not been reported previously in patients with this syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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