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 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Klippel-Trenaunay syndrome presents with a vascular nevus extending over an entire extremity, ipsilateral varicose veins dating from infancy and hypertrophy of all the tissues of the affected limb. This paper illustrates the problems that can be encountered in the management of this disorder. Congenital angiodysplasias should be suspected in all young individuals presenting with unilateral varicose veins. We recommend that modern methods of investigation be used in these cases to precisely define the anatomic pathology. In this manner, the appropriate treatment may be tailored to the patient's specific disorder.
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PMID:The Klippel-Trenaunay syndrome. A case report and review. 17 66

Nineteen patients with vascular deformities of the lower extremity were studied and were classified into three groups based on their clinical and angiographic findings. 1) The Klippel or Venous Dysplasia Group (n = 8) demonstrated: cutaneous nevi of metameric distribution, congenital varices (many of which were anomalous by phlebography), hypertrophy of bone and other tissues, but no evidence of arterial involvement by arteriography. 2) Congenital Arterio-Venous Fistulae Group (n = 7) showed: cutaneous angiomata, hypertrophy of bone and soft tissues, but arteriographic findings of single or multiple arterio-venous fistulae. 3) Scattered Angiomata Group (n = 4) had single or multiple angiomata involving the lower limb in particular. Arteriography was normal, while phlbography revealed venous abnormalities. Lymphography was performed on all patients. Six of 8 of the Klippel group demonstrated aplastic or hypoplastic lymphatics by lymphography. By contrast, all 7 of the patients in the Congenital Arterio-Venous Fistulae Group showed in their lymphographic findings-three of 4 of these patients had aplasia or hypoplasia. Fourteen of the 19 patients complained of painful swelling as a major symptom. Lymphatic fistulae and lymphoceles developed in several patients.Four patients underwent reduction operations for lymphedema. Lymphatic abnormalities as demonstrated by lymphography play a significant role in mixed vascular deformities of the lower extremities.
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PMID:Congenital mixed vascular deformities of the lower limb: the relevance of lymphatic abnormalities to their diagnosis and treatment. 18 10

Benign tumors and "dark lesions" accounted for 22% of vulvar disease seen in the Vulvar Clinic at the Milwaukee County Medical Complex over an 8-year period. Biopsy confirmation was obtained for 269 lesions. The order of frequency of lesions in this study was as follows: epidermal inclusion cyst, lentigo, Bartholin's duct obstruction, carcinoma in situ, melanocytic nevi, acrochordon, mucous cyst, hemangiomas, postinflammatory hyperpigmentation, seborrheic keratoses, varicosities, hidradenomas, verruca, basal cell carcinoma, and, last, unusual tumors such as neurofibromas, ectopic tissue, syringomas, and abscesses. The variability in clinical appearance of vulvar tumors suggests that biopsy confirmation should be obtained on all lesions for which there is the least doubt regarding the diagnosis.
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PMID:Benign vulvar tumors. 131 12

Klippel-Trenaunay syndrome is a rare congenital malformation characterized by a large angiomatous nevus; hypertrophy of soft tissue or overgrowth of bone, or both; and venous varicosities. The cases of 108 patients who had a diagnosis of this syndrome between 1956 and 1990 were reviewed. One hundred and twenty-six anomalies were found in twenty-nine patients, each of whom had one to thirteen malformations of the fingers or toes, or both. The ratio of female patients to male patients was approximately two to one. Twenty-six patients had macrodactyly affecting one to six digits; nine, syndactyly involving two or more digits; five, metatarsus primus varus; two, clinodacytly; two, polydactyly; one, camptodactyly; and one, a congenital trigger finger. Thirty-three of the 126 anomalies were in extremities that had no nevi, varicose veins, or generalized hypertrophy.
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PMID:Anomalies of the fingers and toes associated with Klippel-Trenaunay syndrome. 166 Aug 97

Klippel-Trenaunay syndrome is an angio-osteohypertrophy syndrome without deep arteriovenous malformations. Clinical manifestations consist of vascular nevi, varices, and bony and soft-tissue hypertrophy. On skeletal imaging, there was extensive radionuclide uptake in a massively enlarged right lower extremity.
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PMID:Musculoskeletal findings in Klippel-Trenaunay syndrome. 166 14

The Klippel-Trenaunay Syndrome is a triad of congenital anomalies characterized by a vascular naevus, varicose veins and hypertrophy of soft tissue and bone. A number of patients affected with this rare syndrome need amputation. In this paper the systemic problems, stump complications and prosthetic difficulties of four amputees with Klippel-Trenaunay syndrome are outlined. The period of follow-up ranged from 10 to 24 years after amputation.
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PMID:The Klippel-Trenaunay syndrome. 184 65

Eight cases (9 limbs including 8 lower extremities and 1 upper extremity) of the Klippel-Trenaunay Syndrome (KTS) are reported Among 8 cases, 7 cases were proved by surgery KTS is basically a sort of congenital venous dysplasia including venous atresia, aplasia, valvular anomalies, and external compression by a fibrovascular band or surrounded by a fibrous sheath. The basic clinical triad (vascular nevus, varicosities and limb hypertrophy) suggests the characteristic clinical diagnosis. KTS may be divided into four types. Orthodromic, deep venography is the most important examination in diagnosing KTS. Selective arteriography and direct puncture venography may be also used if necessary. The clinical features, angiographic characteristics, surgical problems, and the nomenclature, type and differential diagnosis of KTS were discussed.
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PMID:[The clinical feature and angiographic diagnosis in Klippel-Trenaunay syndrome]. 196 92

A complete check-up of vascular morphology and haemodynamics in patients resulted in a differentiation of three clinical entities: (I) type F. P. Weber: multiple active arteriovenous fistulas inducing overgrowth of the skeleton, (2) type Klippel-Trenaunay: venous angiodysplasia with the triad localized gigantism, varicose veins, naevus flammeus, (3) type Servelle-Martorell: systemic haemangiomatosis including the bone and soft tissues resulting in hypoplasia of the skeleton. Early diagnosis and differentiation of these different clinical types are needed for the assessment of the spontaneous course of the disease and choice of an optimal therapeutical approach. In the presence of active arteriovenous fistula (type F. P. Weber) the therapeutic principle should be focused on the normalisation of the shunt volume by surgery (skeletonisation) or catheter embolisation. In patients with venous angiodysplasias (type Klippel-Trenaunay or Servelle-Martorell) the therapy is aimed at prevention or reduction of deep venous insufficiency and is basically conservative by external compression bandages.
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PMID:[Congenital angiodysplasia of the limbs; diagnosis and therapy]. 215 9

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.
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PMID:Klippel-Trenaunay-Weber syndrome with spinal arteriovenous malformation--case report. 247 29

The Klippel-Trenaunay-Weber (KTW) syndrome is a rare congenital syndrome of unknown etiology consisting of the triad: a large cutaneous naevus, congenital varicosities and hypertrophy of bones and soft tissues. A heterogenous group of vascular malformations may also occur. The case record of acute myelopathy in a patient aged 42 years with recognized KTW syndrome is presented. It is concluded that magnetic resonance imaging is indicated in cases of suspected intramedullary haemorrhage in patients with congenital vascular malformations.
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PMID:[Klippel-Trenaunay-Weber syndrome. Magnetic resonance imaging diagnosis of medullary involvement]. 255 37


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