Recurrent Pulmonary Embolism Associated With Klippel-Trenaunay-Weber Syndrome

“…A review of the literature showed that Klippel-Trénaunay syndrome has been associated with venous thromboembolism in 8% to 22% of the cases [3][4][5][6][7][8][9]. The underlying mechanism of hypercoagulability in vascular malformations is still unclear.…”

Section: Discussionmentioning

confidence: 99%

A 39-year-old woman with atypical variant of Klippel-Trénaunay syndrome and progressive thromboembolic pulmonary hypertension, successfully treated by pulmonary thromboendarterectomy (RCD code: II-1A.5)

Poręba

Misztal

Biederman

et al. 2013

Journal of Rare Cardiovascular Diseases

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We present a case of 39-year old female with Klippel-Trenaunay syndrome (KTS) complicated by chronic thromboembolic pulmonary hypertension (CTEPH). Our patient was operated in adolescence by vascular surgeons and severe varicosities of her leg were excised. After second pregnancy she manifested pulmonary embolism (PE) and was treated with vitamin K antagonists (VKA) and implantation of vena cava filter. Despite chronic VKA therapy she developed pulmonary embolism again. We present our TTE studies, X-ray and CT scans confirming diagnosis of pulmonary hypertension. Because of worsening of her cardiopulmonary status she was send to Cardiac Surgery Department and underwent pulmonary thromboendarterectomy. Extensive quantities of chronic thromboembolic material were cut out from both pulmonary arteries. At one year follow-up her functional status returned to NYHA class I. Control TTE demonstrated normal diameter and function of her right cardiac chambers. We conclude that vascular anomalies existing in KTS although present locally are responsible for general hypercoagulability. We also wonder what should be proper treatment and which diagnostic tests should be perform to prevent from severe thromboembolic pulmonary hypertension. JRCD 2013; 1 (2): 61-68

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“…VHs are usually multiple and can be a component of the Klippel-Trenaunay-Weber syndrome (3). Venous thrombosis is usually seen as a complication in this syndrome (4). VHs rarely causes neurological symptoms but this condition should be considered in the differential diagnosis when a patient presents with progressive weakness of extremities.…”

Section: Introductionmentioning

confidence: 99%

Thoracic vertebral hemangioma causing paraplegia in klippel-trenaunay-weber syndrome: case report

Okutan¹,

Yıldırım²,

Işık³

et al. 2011

Turkish Neurosurgery

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Vertebral hemangiomas are the most common tumours of the vertebral column. Generally, these tumours are asymptomatic but some patients complain of back pain and develop neurologic symptoms due to extraosseous extension. Vertebral hemangiomas can extend extradurally causing neurological impairment as a result of compression of the spinal cord and nerve roots. Vertebral hemangiomas may be multiple and detectable as a component of the Klippel-Trenaunay-Weber syndrome. Although this syndrome consists of deep venous thrombosis, lymphatic anomalies, cutaneous capillary malformations, and hypertrophy of soft tissue and bone on extremities, its clinical presentation may be very variable. We present a unique case of vertebral hemangioma causing spinal cord compression due to the extradural extension that also had deep venous thrombosis, hematuria, hypophyseal cyst and ventricle asymmetry, diagnosed as the Klippel-Trenaunay-Weber syndrome.KeywOrds: Thoracic vertebra, Hemangioma, Cord compression, Klippel-Trenaunay-Weber syndrome ÖZVertebral hemanjiyomlar vertebral kolonun en sık görülen tümörleridir. Genel olarak bu tümörler asemptomatikdir ancak bazı hastalarda sırt ağrısı ve kemik dışı yayılıma bağlı nörolojik defisitler görülebilir. Bu olgularda hemanjiyomlar dura dışı olarak yayılır ve sırt ağrısına, radikülopatiye, spinal kord ve sinir köklerinin basısı sonucu ilerleyici veya ani parapareziye neden olur. Vertebral hemanjiyomlar çoklu ve KlippelTrenaunay Weber sendromu ile ilişkili olabilirler. Bu sendromda derin venöz tromboz, lenfatik anomaliler, kutanöz apiller malformasyonlar, ekstremitelerde yumuşak doku ve kemik hipertrofisi bulunsa da klinik görünüm oldukça değişiklik göstermektedir. Dura dışı yayılımına bağlı olarak spinal kordu basılayan aynı zamanda derin venöz tromboz, hematüri, hipofizyel kist ve ventrikül asimetrisi bulunan ve KlippelTrenaunay-Weber sendromu olarak düşündüğümüz oldukça nadir görülen vertebral hemanjiyom vakasını sunmaktayız.

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Recurrent Pulmonary Embolism Associated With Klippel-Trenaunay-Weber Syndrome

Cited by 58 publications

References 7 publications

Pulmonary thromboembolism associated with Klippel Trenaunay Weber Syndrome: A rare case report

Pulmonary thromboembolism associated with Klippel Trenaunay Weber Syndrome: A rare case report

A 39-year-old woman with atypical variant of Klippel-Trénaunay syndrome and progressive thromboembolic pulmonary hypertension, successfully treated by pulmonary thromboendarterectomy (RCD code: II-1A.5)

Thoracic vertebral hemangioma causing paraplegia in klippel-trenaunay-weber syndrome: case report

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