Abstract
Klippel-Trenaunay syndrome (KTS) is characterized by a cutaneous vascular nevus of the involved extremity, as well as bone and soft tissue hypertrophy of the extremity and venous malformations. We present the case of a 52-year-old man with a femoral fracture and a history of haemangiomas, limb bone hypertrophy and varicosity. The patient was finally diagnosed with KTS and treated in mini-invasive surgery by endomedullary fracture nailing in general anaesthesia. Clinical management was particularly demanding, not only because of the need to monitor the risk of haemorrhage and thrombosis but also because of the onset of a rare picture of neuropathic pain with hyperalgesia and allodynia, never previously reported in patients affected by KTS, that required the administration of major opioid drugs. According to our knowledge, this is the second case of KTS managed for femoral fracture. Unlike the previous report in literature, in this case a severe disabling neuropathic pain complicated the clinical management.
References
Tsaridis E, Papasoulis E, Manidakis N, Koutroumpas I, Lykoudis S, Banos A, Sarikloglou S (2009) Management of a femoral diaphyseal fracture in a patient with Klippel-Trenaunay-Weber syndrome: a case report. Cases J 26(2):8852
Bouhassira D, Attal N (2011) Diagnosis and assessment of neuropathic pain: the saga of clinical tools. Pain 152(3 Suppl):S74–S83
Lee A, Driscoll D, Gloviczki P, Clay R, Shaughnessy W, Stans A (2005) Evaluation and management of pain in patients with Klippel-Trenaunay syndrome: a review. Pediatrics 115(3):744–749
Franz RW, Prok A (2009) Klippel-Trénaunay syndrome: treatment of lower extremity pain with a spinal cord stimulator. Vascular 17(5):293–295
Van Gompel JJ, Griessenauer CJ, Scheithauer BW, Amrami KK, Spinner RJ (2010) Vascular malformations, rare causes of sciatic neuropathy: a case series. Neurosurgery 67(4):1133–1142
Dispenza K (2011) Severe pain related to Klippel-Trénaunay-Weber syndrome: a case study. Pain Manag Nurs 12(2):112–114
Barbara DW, Wilson JL (2011) Anesthesia for surgery related to Klippel-Trenaunay syndrome: a review of 136 anesthetics. Anesth Analg 113(1):98–102
Gundogan TG, Jacquemyn Y (2010) Klippel-Trenaunay syndrome and pregnancy. Obstet Gynecol Int 2010:706850
Holak EJ, Pagel PS (2010) Successful use of spinal anesthesia in a patient with severe Klippel-Trénaunay syndrome associated with upper airway abnormalities and chronic Kasabach-Merritt coagulopathy. J Anesth 24(1):134–138
Zea MI, Hanif M, Habib M, Ansari A (2009) Klippel-Trenaunay syndrome: a case report with brief review of literature. J Dermatol Case Rep 3(4):56–59
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Notarnicola, A., Pesce, V., Maccagnano, G. et al. Klippel-Trenaunay syndrome: a rare cause of disabling pain after a femoral fracture. Arch Orthop Trauma Surg 132, 993–996 (2012). https://doi.org/10.1007/s00402-012-1504-9
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DOI: https://doi.org/10.1007/s00402-012-1504-9