Klippel‐Trenaunay Syndrome with a Life‐Threatening Thromboembolic Event

Aggarwal, Kamal; Jain, Vijay Kumar; Gupta, Sanjeev; Aggarwal, Himanshu; Sen, Jyotsna; Goyal, Vishal

doi:10.1111/j.1346-8138.2003.tb00378.x

Cited by 24 publications

(22 citation statements)

References 14 publications

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“…Paradoxically, KTS patients are also at risk of thromboembolic complications, with a reported incidence of venous thromboembolism as high as 22%. 12 Deep vein hypoplasia of the lower extremities, along with the increased blood volume of pregnancy and relative obstruction of venous return by the gravid uterus, can lead to edema and vascular sludging. Additionally, as the varicosities are at risk of minor trauma, and most KTS patients would be expected to be in a hypercoagulable state secondary to pregnancy, the classic predisposing factors for venous thrombosis -stasis, hypercoagulability, and vascular injury -are often met in these patients.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic and obstetric considerations in a parturient with klippel-trenaunay syndrome

Sivaprakasam

Dolak

2006

Can J Anesth/J Can Anesth

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Purpose: To explain the considerations governing the anesthetic management of pregnant patients with Klippel-Trenaunay syndrome (KTS). Klippel-Trenaunay syndrome is a congenital vascular disease characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy; and is associated with both hemorrhagic and thrombotic complications. The importance of this diagnosis, including the presence of neuraxial vascular anomalies, is often under-appreciated by both obstetric and anesthesia providers. While regional anesthetic management of patients with KTS has been discussed by others, we present a case in which regional anesthesia presented an unwarranted risk to the patient. Clinical features:An obese, 18-yr-old parturient with a fetus in the breech position underwent Cesarean delivery at 35 weeks gestation secondary to evolving preeclampsia. Unfortunately, no neurovascular imaging of this patient's spine was available. The patient underwent an attempted external cephalic version, a failed obstetric induction, and, ultimately, a Cesarean delivery under general anesthesia. The resulting infant was without any stigmata of KTS. Both mother and infant did well during the course of their hospitalization, and were discharged home without incident. Conclusions:The posterior cutaneous hemangiomas of KTS may be associated with underlying epidural and subdural vascular malformations. Disruption of these vascular anomalies during regional anesthesia may lead to neuraxial hematoma formation, which may be further compounded by a consumptive coagulopathy observed in some cases of KTS. If neuraxial vascular anomalies cannot be ruled out radiographically, regional anesthesia should be avoided. Additionally, regardless of the anesthetic technique chosen, the coagulation profile of these patients should be verified for signs of coagulopathy. Objectif

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Section: Discussionmentioning

confidence: 99%

Anesthetic and obstetric considerations in a parturient with klippel-trenaunay syndrome

Sivaprakasam

Dolak

2006

Can J Anesth/J Can Anesth

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“…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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“…Des cas d'embolies pulmonaires sévères ont été décrites chez l'adulte [8][9][10]. Les épisodes d'embolies pulmonaires répétés peuvent également entraîner des hypertensions artérielles pulmonaires chroniques.…”

Section: Autresunclassified

Syndrome de Klippel-Trenaunay

Samimi

Lorette

2010

La Presse Médicale

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Klippel‐Trenaunay Syndrome with a Life‐Threatening Thromboembolic Event

Cited by 24 publications

References 14 publications

Anesthetic and obstetric considerations in a parturient with klippel-trenaunay syndrome

Anesthetic and obstetric considerations in a parturient with klippel-trenaunay syndrome

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)

Syndrome de Klippel-Trenaunay

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