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Femoral Shaft Fracture in Klippel–Trenaunay–Weber Syndrome Patients – What to Do to Reduce Bleeding Risk: A Case Report

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초록

A fracture of the affected extremity in patients with Klippel–Trenaunay–Weber syndrome can be fatal due to massive bleeding and show poor results. A 42-year-old male presented with an old fracture of the right femoral shaft with metal failure. We planned an operation to remove the previously fixed plate and to perform re-fixation using an intra-medullary nail. Preoperative angiography was performed and the arteriovenous malformations were embolized in order to reduce the risk of bleeding. After angiography, the previously fixed plate was removed. After the operation, a second angiography was performed immediately and the venous malformation was embolized. One week after the first operation, a second operation was performed in order to reduce the fracture and to perform re-fixation using an intramedullary nail. The patient is being followed without major complication over a period of seven years after surgery. We recommend careful planning of preoperative and postoperative angiography and embolization in order to reduce the risk of bleeding in patients with Klippel–Trenaunay–Weber syndrome.

Abstract

A fracture of the affected extremity in patients with Klippel–Trenaunay–Weber syndrome can be fatal due to massive bleeding and show poor results. A 42-year-old male presented with an old fracture of the right femoral shaft with metal failure. We planned an operation to remove the previously fixed plate and to perform re-fixation using an intra-medullary nail. Preoperative angiography was performed and the arteriovenous malformations were embolized in order to reduce the risk of bleeding. After angiography, the previously fixed plate was removed. After the operation, a second angiography was performed immediately and the venous malformation was embolized. One week after the first operation, a second operation was performed in order to reduce the fracture and to perform re-fixation using an intramedullary nail. The patient is being followed without major complication over a period of seven years after surgery. We recommend careful planning of preoperative and postoperative angiography and embolization in order to reduce the risk of bleeding in patients with Klippel–Trenaunay–Weber syndrome.

참고문헌 (8)

  1. Klippel M, "Du naevus variqueux osteohypertrophique", Arch Gen Med, 185, pp.641~672 (1900)
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  3. Wang SK, "Diagnosis and management of the venous malformations of Klippel-Trénaunay syndrome", J Vasc Surg Venous Lymphat Disord, 5, pp.587~595 (2017)[DOI]
  4. Mahjoub A, "Treatment of femoral shaft fractures in patients with Klippel-Trénaunay syndrome : a report of 2 cases", JBJS Case Connect, 11, pp.e21~ (2021)[DOI]
  5. Nahas S, "A case of femoral fracture in Klippel Trenaunay syndrome", Case Rep Orthop, 2014, pp.548161~ (2014)[DOI]
  6. Gupta Y, "Management of femoral shaft fracture in Klippel-Trenaunay syndrome with external fixator", Case Rep Orthop, 2016, pp.8505038~ (2016)[DOI]
  7. Deshpande P, "Klippel-Trenaunay syndrome and femoral fracture : a literature review and case report", Curr Orthop Pract, 33, pp.204~207 (2022)[DOI]
  8. Barbara DW, "Anesthesia for surgery related to Klippel-Trenaunay syndrome : a review of 136 anesthetics", Anesth Analg, 113, pp.98~102 (2011)[DOI]

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