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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital disorder characterized by vascular nevus formation, deep venous thrombosis, varicosities, and hypertrophy of affected tissues. A patient with known thrombosis of his splanchnic circulation and visceral KTWS presented with life-threatening hemorrhage from rectosigmoid varices. Portosystemic shunting was not feasible. Endoscopic sclerosis, variceal ligation, and proctocolectomy were not possible due to the size and number of the varices. Previous treatment with epsilon-aminocaproic acid had been unsuccessful and complicated by thrombophlebitis. Conservative treatment with blood transfusions, cryoprecipitate, fresh frozen plasma, vitamin K, propanolol, and somatostatin analog failed to stop the bleeding. The patient was given the antifibrinolytic agent, tranexamic acid, with cessation of his hemorrhage. Serial thromboelastograms confirmed improved reaction time, coagulation time, clot formation rate, and maximum amplitude. We conclude that tranexamic acid may be a useful adjunct in the medical treatment of high-risk patients with KTWS and other vascular nevi complicated by coagulopathy.
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PMID:Successful management of visceral Klippel-Trenaunay-Weber syndrome with the antifibrinolytic agent tranexamic acid (cyclocapron): a case report. 954 37

Naevus varicosus osteohypertrophicus (synonym Klippel-Trenaunay Syndrome KTS) is relatively rare circumscribed, usually quadrant-related gigantism with vascular hyperplasia or malformations based on the embryonic development. The authors observed an 18- and a 30-year-old female with a triad of symptoms: cutaneous nevus flammeus, varicose and dilated veins, and bony and soft tissue hypertrophy of the low limb. The second patient also had two venous ulcers as a dominant clinical feature--a rare manifestation of Klippel-Trenaunay syndrome. A diagnosis of Klippel-Trenaunay syndrome was made by clinical observations, laboratory findings, dermoscan, radiological examination of the bones of the limb, Doppler ultrasonography, photopletismography and venoscan. A bone isotope scan was also done to the first patient. Making an early diagnosis of this sporadic congenital disease with unknown aetiology is important in order to be able to provide early prophylactic and therapeutic measures. Klippel and Trenaunay in 1900 were the first to describe a patient with the simultaneous appearance of osteohypertrophy, hemangiomas and varicose veins involving one extremity [1]. In 1907 Parkes and Weber reported a similar syndrome--they described a patient who had dilated and pulsatile arteries in the affected region including the presence of arterio-venous communications. In 1918 they used the compromise term "haemangiectatic hypertrophy" to embrace all conditions which were associated with congenital vascular malformations including A-V anastomoses associated with bone and soft tissue hypertrophy. Most authors are agreed that Klippel-Trenaunay syndrome and the syndrome of multiple congenital arterio-venous fistulae are two separate features of the Parkes Weber hypertrophy. KTS is manifesting with a triad of symptoms: cutaneous vascular nevus (more frequently nevus flammeus type), superficial venous varicosities and hypertrophy of the affected limb. Usually one quadrant of the body is involved: quite often a leg, an arm, lateral side of the trunk, very rarely the face. More than one quadrant and bilateral involvement are rarely affected. Naevus flammeus appeared at birth. It is extremely variable both in extent and in color--the latter ranging from pale pink to deep purple. Veinous varicosities appear in childhood and adolescence. They are painful and may be complicated by superficial or deep venous thrombosis and rarely, ulceration. Hypertrophy of the affected extremities is due to bone and soft tissue hypertrophy. KTS can be associated with other developmental anomalies such as: polydactyly, syndactyly, oligodactyly [2] macrocephaly, blue nevus, epidermal naevus, venous malformations.
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PMID:Naevus varicosus osteohypertrophicus. An early diagnostic approach. 1059 95

Klippel-Trenaunay syndrome is a congenital disorder characterised by the triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and varicose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported but rare is the development of trophic changes. Herein, we report the case of a male with Klippel-Trenaunay syndrome, deep vein thrombosis, venous ulceration, and death due to recurrent pulmonary embolism.
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PMID:Klippel-Trenaunay syndrome with a life-threatening thromboembolic event. 1269 62

Proteus syndrome is caused by an activating AKT1 mutation (c.49G>A, p.Glu17Lys). Many variable features are possible in this mosaic disorder, including: (i) disproportionate, asymmetric, and distorting overgrowth; (ii) bone abnormalities different from those observed in other disorders; (iii) a characteristic cerebriform connective tissue nevus made up of highly collagenized connective tissue; (iv) epidermal nevi in early life, consisting of acanthosis and hyperkeratosis; (v) vascular malformations of the capillary, venous, or lymphatic types; (vi) dysregulated adipose tissue including lipomas, lipohypoplasia, fatty overgrowth, and localized fat deposits; (vii) other unusual features, including bullous lung alterations; specific neoplasms; a facial phenotype associated with intellectual disability and/or seizures, and/or brain malformations; and (viii) deep vein thrombosis, resulting in premature death. Concluding remarks address diagnostic criteria, natural history, management, psychosocial issues, and differential diagnosis.
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PMID:Proteus syndrome review: molecular, clinical, and pathologic features. 2399 99

We report a 25 year old mole admitted to a critical care unit for fever, lung opacities and acute respiratory failure. A chest angio-CAT sean showed multiple pulmonary artery aneurysms. A deep venous thrombosis of both lower limbs was also documented. A Hughes-Stovin syndrome was postulated. An inferior vena cava filter was placed. The patient received antimicrobial therapy subsiding fever and respiratory failure. Subsequently, he was treated with intravenous and oral steroids and one dose of cyclophosphamide. The patient was discharged in good conditions fifteen days after admission.
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PMID:[Hughes-Stovin syndrome: report of one case]. 2435 42