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

The syndrome of Klippel and Trenaunay remains basically a venous angiodysplasia. Newer investigative methods have however allowed us to distinguish a polyvalence of the vascular morphology. The basic clinical triad: vascular nevus, varicosities and limb hypertrophy remains the main clinical diagnostic characteristic.
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PMID:The syndrome of Klippel-Trenaunay, a polyvalent angiodysplasia. 282 Oct 7

Chronic lower-leg edema in patients with venous disorders was studied by means of lymphoscintigraphy. Lymphatic patterns of flow were evaluated prospectively in 26 patients with technetium 99m antimony trisulfide colloid injected subcutaneously in the interdigital web spaces on the feet. Most patients in this study had postphlebitic syndrome, and all of these patients had abnormal lymphoscintigraphic flow patterns. Nine had evidence of lymphatic obstruction, and one had an enhanced flow pattern. Three patients had veins used for distal arterial bypass, and all these veins showed decreased lymphatic flow. Two patients with Klippel-Trenaunay syndrome (congenital varicose veins associated with limb elongation, a capillary nevus, and an abnormal deep venous system) had obstruction to lymphatic flow, and two others had normal and enhanced patterns. Normal studies were seen in four of five patients who had veins used for coronary artery bypass grafting. The finding of decreased lymphatic flow in patients appears to be the result of the length of time from an episode of deep venous thrombosis, the occurrence and number of episodes of cellulitis and lymphangitis, and mobilization of the vein for use in distal arterial bypass surgery. This study shows that the edema attributed previously to primary venous disorders may have a significant lymphatic component. The degree of lymphatic obstruction can be determined by lymphoscintigraphy with technetium-labeled antimony trisulfide colloid.
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PMID:Abnormalities of lymphatic drainage in lower extremities: a lymphoscintigraphic study. 291 Nov 34

The etiology of the Klippel-Trenaunay syndrome (KTS) remains obscure. Although venous hypertension secondary to deep venous obstruction has been suggested as a cause, recent studies have demonstrated that most patients have unimpeded venous drainage. Calf blood flows have been measured in 33 patients with KTS using venous occlusion plethysmography. Although all flow rates were within normal limits, flow in affected limbs was invariably greater than in normal limbs (p less than 0.001), and this is related to the presence of the nevus. Biopsies of subcutaneous veins demonstrate the histological features of a response to chronically raised flow. The authors suggest that KTS is caused by mesodermal abnormality during fetal development, leading to the maintenance of microscopic arteriovenous communications in the limb bud, as a result of which the triad of nevus, hypertrophy, and superficial varices is produced. Deep venous abnormalities occur pari passu with the triad and are not responsible for its development.
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PMID:The etiology of the Klippel-Trenaunay syndrome. 299 51

The dermatologic component of the first National Health and Nutrition Examination Survey (N = 20,637), conducted from 1971 through 1974, documented the deleterious effect of ultraviolet radiation on selected skin and eye conditions. Actinic skin damage was more frequent in white men with high as compared with low sunlight exposure, 36.7% vs 23.3%, respectively. Among white women, the corresponding figures were 34.1% vs 18.6%, respectively. Actinic damage was found more often in individuals with light eye color. Basal cell epitheliomas were found in 11.3% of white men aged 65 to 74 years who had severely actinic-damaged skin as compared with 1.0% of those with undamaged skin. Sunlight exposure was positively associated with localized hypomelanism, localized hypermelanism, seborrheic keratoses, senile lentigines, freckles, acne rosacea, spider nevi, varicose veins, venus star, dry skin, wrinkled skin, pterygia, arcus senilis, and a variety of minor oral lesions of the tongue, palate, and buccal mucosa. These findings suggest that a large number of dermatologic conditions, which may in part result from overexposure to sunlight, may be preventable.
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PMID:Health effects of sunlight exposure in the United States. Results from the first National Health and Nutrition Examination Survey, 1971-1974. 325 72

We examined an 11-year-old female with symmetric, progressive telangiectatic nevi associated with venous varicosities and angiokeratomas. Clinical bleeding occurred from cutaneous angiokeratomas and mucosal telangiectasias.
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PMID:Symmetric, progressive telangiectatic nevi with hemorrhagic angiokeratomas. 372 97

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.
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PMID:An unusual variant of Klippel-Trenaunay-Weber syndrome. 436 79

Several mechanisms can be invoked in order to explain the appearance in a limb of the characteristic triad : naevus, varices and osteodystrophy. Many cases can be explained by the haemodynamic disturbance associated with venous hypertension caused by a venous malformation combined with arteriovenous microcommunications. These are the true Klippel and Trenaunay Syndromes. In a few rare cases the haemodynamic disturbance is caused by one or more arteriovenous fistulae with major flows. These are the Parkes-Weber-Syndromes. Finally, in the more complex syndromes there is the combined triad of naevus, varices and osteodystrophy, together with other genetic disturbances. These are the Phacomatoses.
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PMID:[Klippel-Trenaunay syndrome and phacomatoses]. 624 51

The Klippel-Trenaunay syndrome is a triad of congenital anomalies characterized by a vascular nevus, varicose veins and bony and soft-tissue hypertrophy. Although the syndrome is uncommon, initially the condition appears as a skin blemish or varicose veins. To avoid errors in management, it is important to recognize the syndrome. In this paper the authors outline the presentation, investigation and management of Klippel-Trenaunay syndrome and describe four cases of their own. One form of Klippel-Trenaunay syndrome, in which all the anomalies of the triad are important, in which direct signs of a large arteriovenous shunt are present and for which the surgical approach is difficult, is Parkes-Weber syndrome. Lack of reports in the world literature on long-term follow-up leaves many unanswered questions regarding prognosis and management.
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PMID:Klippel-Trenaunay syndrome: a review. 631 89

The major clinical features of nine patients (seven women and two men) with cutis marmorata telangiectatica congenita (CMTC) were persistent cutis marmorata, spider nevus-like telangiectasia, and phlebectasia. Three of the patients had persistent ulcers and atrophy. In two patients, atrophie blanche-like lesions developed on the legs with ulcers. No patient had a family history of a similar disorder. Skin lesions were present at birth in seven cases, at the age of eight months in one case, and at eighteen months in one case. Three patients had clinical improvement of the skin lesions with age. Eight of the nine patients had associated physical anomalies. Biopsy specimens showed an increase in the number and size of the blood vessels in the upper dermis. Alkaline phosphatase staining gave negative results in the dilated blood vessels of the single case in which this histochemical study was done.
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PMID:Cutis marmorata telangiectatica congenita: report of nine cases and review of the literature. 638 56

Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular disorder that was first described in 1922 by Van Lohuizen (1). The clinical presentation is one of persistent cutis marmorata, phlebectasia, telangiectasia, and areas of ulcerations. The disorder shows slow clinical improvement over time. Previously, CMTC has also been described as congenital generalized phlebectasia (2-7), congenital phlebectasia (8), nevus vascularis reticularis (9), congenital livedo reticularis (10), and livedo telangiectatica (11). The following is a case report.
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PMID:Cutis marmorata telangiectatica congenita: a case report. 649 67


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