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1/59. Recurrent pulmonary embolism associated with klippel-trenaunay-weber syndrome.

    klippel-trenaunay-weber syndrome (KTWS) is a rare, congenital disorder characterized by the triad of varicose veins, cutaneous hemangiomas, and hypertrophy of soft tissue and bone. We present the case of a woman with KTWS, cor pulmonale, and death due to recurrent pulmonary embolism (PE). The risk of deep venous thrombosis and PE in patients with KTWS is evaluated, and treatment recommendations are made with emphasis on the role of early, aggressive management in the subset of patients with KTWS known to have thromboembolic disease.
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ranking = 1
keywords = venous thrombosis, thrombosis, vein, deep
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2/59. Epidural analgesia in an obstetric patient with Klippel-Trenaunay syndrome.

    We describe the use of epidural analgesia for vaginal delivery of a parturient with Klippel-Trenaunay syndrome in whom the use of repeated magnetic resonance imaging during her obstetric care allowed us to see deep haemangiomata. This also allowed the safe sitting of an epidural catheter at L1-2 to provide analgesia for labour and delivery. Klippel-Trenaunay syndrome and the anaesthetic implications of the congenital vascular abnormalities and potential coagulopathy are discussed.
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ranking = 0.023822818980971
keywords = deep
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3/59. Right iliac vein agenesis, varicosities, and widespread hemangiomas: report of a rare case.

    We present a probable variant of the Klippel-Trenaunay syndrome with the clinical features of capillary hemangiomas, varicosities, and agenesis of the right iliac venous system, but without limb hypertrophy. To our knowledge, this is the 1st such case reported in the medical literature.
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ranking = 1.0853582945864
keywords = vein
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4/59. Endovascular treatment of a cervical paraspinal arteriovenous malformation via arterial and venous approaches.

    We describe a cervical congenital paraspinal arteriovenous malformation (AVM) drained by paraspinal and epidural ectatic veins, which caused massive erosion of the C6 and C7 vertebral bodies, threatening the cervical stability and necessitating treatment. During the first session, six arterial embolizations were performed to reduce the size and the flow of the AVM. Two months later, a venous approach was used to occlude the remnant venous exit of the AVM and achieve a complete cure. All embolizations were performed using N-butylcyanoacrylate.
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ranking = 0.27133957364659
keywords = vein
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5/59. Naevus varicosus osteohypertrophicus. An early diagnostic approach.

    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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ranking = 1.2951623926276
keywords = venous thrombosis, thrombosis, vein, deep
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6/59. Successful pulmonary thromboendarterectomy in a patient with Klippel-Trenaunay syndrome.

    Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a 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 is the development of chronic thromboembolic pulmonary hypertension (CTEPH) and subsequent death from right ventricular failure. We report the first patient with KTS to undergo a successful pulmonary thromboendarterectomy for CTEPH.
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ranking = 0.27133957364659
keywords = vein
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7/59. An unusual association of intracranial aneurysms and oesophageal duplication in a case of Klippel-Trenaunay syndrome.

    The Klippel-Trenaunay syndrome (KTS) is a congenital disorder resulting from a mesodermal abnormality, characterised by cutaneous capillary haemangiomas, hypertrophy of bone and soft tissues and varicose veins. The presence of intracranial aneurysms has rarely been described, while oesophageal duplication has not been reported previously. We describe a patient with the KTS with both there additional abnormalities, which could be explained by a postulated mosaic gene abnormality.
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ranking = 0.27133957364659
keywords = vein
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8/59. klippel-trenaunay-weber syndrome with hemimegalencephaly.

    There are many documented cases of klippel-trenaunay-weber syndrome characterized by the triad of port wine stain, varicose veins, and hypertrophy of bones and overlying tissue. A few cases have even included the additional findings of hemimegalencephaly. We report a case of klippel-trenaunay-weber syndrome with hemimegalencephaly and calvarial hypertrophy, but no evidence of limb or trunk hypertrophy.
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ranking = 0.27133957364659
keywords = vein
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9/59. Squamous cell carcinoma of the oesophagus in a patient with Klippel-Trenaunay syndrome.

    The Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by a triad of port-wine stains, varicose veins, and bony or soft tissue hypertrophy of one extremity. Digestive bleeding is the most commonly observed gastrointestinal manifestation. In rare cases, the syndrome is associated with malignancies. We report the case of a 38-year-old woman with KTS and dysphagia caused by an oesophageal squamous cell carcinoma.
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ranking = 0.27133957364659
keywords = vein
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10/59. Persistence of the embryonic lateral marginal vein: report of two cases.

    PURPOSE: Congenital venous malformations of the lower limbs represent a particular challenge for the vascular surgeon. Persistence of fetal veins is a rare malformation, and the most common is the persistence of the lateral marginal vein usually observed in patients with Klippel-Trenaunnay Syndrome. The persistence of this embryonic vein as an isolated venous malformation without the other characteristics of the Klippel-Trenaunnay Syndrome has not yet been reported. This paper describes two cases. methods: Two patients, a 17-year-old male patient and a 16-year-old female, have had since their birth a large venous trunk in the lateral aspect of the right leg and thigh. The limbs underwent duplex scanning and phlebography. The surgical removal of the lateral marginal vein was performed. RESULTS: Surgical treatment resulted in very good functional and aesthetic results. Follow-up at 26 months showed no evidence of varicose vein recurrence. CONCLUSIONS: To achieve good results, surgical intervention may be indicated in cases of orthopedic deformity, hemorrhage, symptomatic, and unaesthetic lesions.
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ranking = 2.4420561628193
keywords = vein
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