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Genitourinary hemangiomas in 2 children with Klippel-Trenaunay syndrome are reported. The presence of cutaneous hemangiomas should alert urologists to the possibility of urinary tract hemangiomas inpatients with gross hematuria. Transurethral manipulation of bladder hemangiomas should be avoided because of the danger of excessive hemorrhage, the treatment of choice being partial cystectomy.

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.

Between January 1956 and July 1981, 40 patients with Klippel-Trenaunay syndrome were seen at the Mayo Clinic. Twenty male and 20 female patients presented with the classic triad of soft tissue and bony hypertrophy of the extremity, hemangioma, and varicosity without evidence of functional arteriovenous fistulae. The lower extremity was involved in 38 patients (95%), the upper extremity in six patients (15%). In four of these cases (10%), both the upper and lower extremities were affected. The disease was unilateral in 34 patients (85%), bilateral in five cases (12.5%), and crossed-bilateral in one case (2.5%). Surgery was done in 13 patients (32.5%), nine of whom were operated on at the Mayo Clinic and four of whom had had previous surgery elsewhere. Excision and stripping of varices were performed in three patients; of these three, a deterioration of symptoms was observed in one, but the procedure was beneficial in the other two. Partial varicectomy was performed in four cases, while resection of the angioma was attempted in eight cases, with good results in three cases of small angiomas. Femoral and tibial epiphysiodesis stopped the overgrowth and produced an excellent result in one case. In four cases of epiphysiodesis at the foot level and two cases of derotational tibial osteotomy, moderate improvement was achieved. The indication for vascular or orthopedic surgery should be carefully considered in each patient who has this syndrome. One patient (2.5%) died of a severe form of the disease, but the relatively benign course is documented by the 21 patients (52.5%) who are free of complaints without any treatment or with elastic support only.

The angiographic, phlebographic and lymphographic findings in a 3-year old girl with mixed angiodysplasia (Klippel-Trenaunay-Weber syndrome) combined with unilateral congenital choanal atresia are described.

A patient with intravascular coagulation caused by the Klippel-Trenaunay syndrome is presented; this is a rare phenomenon. The patient was treated with fast electrons. Sonography is a suitable method for determining the extension of the haemangiomas occurring in Klippel-Trenaunay's syndrome. This is important for therapy planning. With colour-coded Doppler sonography, arteriovenous fistulas can be ruled out, this allowing to distinguish the Klippel-Trenaunay syndrome from the Parkes-Weber syndrome.

A woman aged 46 years, an epileptic, with no family history of hereditary disorders consulted a doctor in 1972 because of an ulcer on the right foot. She presented varices, lengthening of the right leg, and an angioma. The three components of the Klippel-Trenaunay syndrome were present. The angioma also involved the trunk, the right arm and the head where there was predilection for the trigeminal region. Radiograms of the cranium show a "pumice-stone" appearance of the dome and deformation of the sella turcica. The electroencephalogram showed slow waves appearing during hyperpnoea in the right temporo-rolandic region. These different elements made it possible to diagnose the Sturge-Weber syndrome. This association of the Klippel-Trenaunay and Sturge-Weber syndromes did not appear clinically to have been due to chance but appeared to be one disease akin to the phakomatoses. Thus the two syndromes that were associated can each be considered as phakomatosis.

The authors have tried to show the close link between the lymphatic and venous systems. They describe the clinical aspects which cause us to note that one or other of these systems is the more affected, and they bring to mind aetiological theories on the origin, either congenital or acquired, of the affection. This leads to considerations clarifying the clinical and anatomo-pathological data.

A case of nonimmune hydrops fetalis in association with angioosteohypertrophy (Klippel-Trenaunay) syndrome is reported for the first time.

Defects in angiogenesis occurring in utero produce a broad spectrum of clinical abnormalities. Some regress in infancy, some persist and may be allied with abnormalities of other structures. The possible interrelationships between abnormalities of the arterial, venous and lymphatic systems and skeletal development are explored in this paper.

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.

We have reviewed a wide spectrum of disorders producing a swollen limb, some common, others uncommon. Cutaneous signs are often seen in association with these conditions and careful attention to them by the dermatologist may be helpful in determining an accurate diagnosis and appropriate therapy.

The present paper describes two patients with Sturge-Weber and Klippel Trénaunay-Weber syndromes. Some etiopathogenic factors are analyzed. We suggest that the association of both diseases in the same patient may be due to a single autosomal dominant gene.

It is reported on a 21-year-old patient with Klippel-Trenaunay's syndrome and simultaneous intestinal haemorrhage. Anamnesis and histological findings are discussed and the problems of therapy are explained. In the discussion the knowledge of the peripheral angiodysplasias and their differential diagnosis in literature are entered. Comparative considerations concerning the own case are made.

We report a bladder hemangioma in a patient with the Klippel-Trenaunay syndrome. Ultrasound findings consisted of a markedly thickened bladder wall with multiple serpiginous, anechoic spaces. Computerized tomography demonstrated diffuse bladder wall thickening with foci of calcification.

The association of soft tissue and bony overgrowth, varicose veins and port-wine nevus is known as Klippel-Trenaunay-Weber syndrome. An unusual case of a young woman with this syndrome is presented. In addition to the classic features she had face swelling, bilateral limb involvement and an infected cutaneous lymphangioma. No evidence of arteriovenous fistula was demonstrated. In contrast to an abnormal lymphatic system the deep veins of the limbs were normal. Unilateral lung lesions were demonstrated which have not been described previously in such cases.

A 23-year-old woman with factor XIII deficiency was presented. The patient had no consanguinity, but familial traits were present. A bleeding tendency and poor wound healing had been noted in the patient since birth. She had hemangiomas in the leg and vulva (Klippel-Weber disease). Hematologic studies revealed platelet dysfunction, cryofibrinogenemia and mild chronic disseminated intravascular coagulation with prolonged PT and PTT, hypofibrinogenemia, a high turnover rate of 125I-fibrinogen and mild elevation of fibrinogen-fibrin degradation products, beta-thromboglobulin and platelet factor 4. A decrease in clot retraction and a marked reduction in maximal amplitude of thrombelastogram were also found. The assay of the factor XIII level was 10% by the antiserum inhibition method, and the assay of subunits A and S were 16 and 29%, respectively, by the electroimmunoassay method. Transamidase activity of factor XIII was 26%. The level of factor XIII of her sister was low, similar to that of the patient. The concentration of cold-insoluble globulin in EDTA-plasma was 36.5 mg/dl.

The triad of varicose veins, hypertrophy of soft tissue and bone, and cutaneous hemangiomas (The Klippel-Trenaunay syndrome) often results in major malformations. A review of eleven children treated for this condition showed that the overgrowth of the limb was a major problem in management. In three patients the altered hemodynamics resulted in cardiac complications. Venous stripping, excision of the arteriovenous fistulae, and radiation therapy should be avoided.