Abstract
BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterized by a combination of capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations. The syndrome predisposes patients to hypercoagulable states, including venous thromboembolism and pulmonary embolism (PE). CASE SUMMARY: A 12-year-old girl with KTS was scheduled excision of verrucous hyperkeratosis in the left foot and posterior aspect of the left leg and left thigh and excision of a cutaneous hemangioma in the right buttock. After induction, the surgeon elevated the patient's leg for sterilization, whereupon she experienced a massive PE and refractory cardiac arrest. Extracorporeal membrane oxygenation (ECMO) was performed after prolonged resuscitation, and she had a return of spontaneous circulation. After this episode, the patient was discharged without any neurologic complications. CONCLUSION: The mechanism of PE, a lethal disease, involves a preexisting deep vein thrombosis that is mechanically dislodged by compression or changing positions and travels to the pulmonary artery. Therefore, patients predisposed to PE should be prescribed prophylactic anticoagulants. If the patient has unstable vital signs, resuscitation should be started immediately, and extracorporeal cardiopulmonary resuscitation should be considered in settings with existing ECMO protocols, expertise, and equipment. Awareness of PE in patients with KTS while leg raising for sterilization is critical.