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  • Title: Anesthetic management of Klippel-Trénaunay syndrome and attendant gastrointestinal hemorrhage. A case report.
    Author: Pereda Marín RM, García Collada JC, Garrote Martínez AI, Miralles Serrano EM, Morales Aguilar JL.
    Journal: Minerva Anestesiol; 2007 Mar; 73(3):187-90. PubMed ID: 17159757.
    Abstract:
    Klippel-Trénaunay syndrome is a congenital malformation characterized by the triad of flat red hemangiomas, soft tissue and bony hypertrophy and varicose veins. In addition, venous malformations or hemangiomas may also be found in the lung, gastrointestinal tract, liver, kidney and bladder, which may result in recurrent hemor-rhage and compromise the affected organs. Involvement of the cerebrum, cerebellum or spinal cord may lead to intracerebral bleeding and compression of neurological structures. Venous thrombosis and pulmonary thromboembolism are both common and may cause pulmonary hypertension and right ventricle failure. In some patients, in addition to flat hemangiomas, cavernous hemangiomas can be seen with a rapid growth rate in the first year of the patient's lifespan, producing high-output congestive heart failure and consumptive coagulopathy (Kasabach-Merritt syndrome). We report the case of a 35-year-old male patient diagnosed with Klippel-Trénaunay syndrome who was scheduled for right hemicolectomy for relapsing gastrointestinal hemorrhage unmanageable with conservative treatment. We also discuss the pathophysiologic and clinical aspects of the Klippel-Trénaunay syndrome. Nowadays, the anesthetic management of these patients remains a matter of controversy.
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