DOI QR코드

DOI QR Code

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

  • Byung-Chan Cho (Department of Orthopedic Surgery, Dongsan Hospital, Keimyung University School of Medicine) ;
  • Byung-Woo Min (Department of Orthopedic Surgery, Dongsan Hospital, Keimyung University School of Medicine) ;
  • Kyung-Jae Lee (Department of Orthopedic Surgery, Dongsan Hospital, Keimyung University School of Medicine)
  • 투고 : 2022.07.04
  • 심사 : 2022.08.09
  • 발행 : 2022.12.31

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.

키워드

참고문헌

  1. Klippel M, Trenaunay P. Du naevus variqueux osteohypertrophique. Arch Gen Med. 1900;185:641-72. 
  2. Viljoen DL. Klippel-Trenaunay-Weber syndrome (angioosteohypertrophy syndrome). J Med Genet. 1988;25:250-2. https://doi.org/10.1136/jmg.25.4.250 
  3. Wang SK, Drucker NA, Gupta AK, Marshalleck FE, Dalsing MC. Diagnosis and management of the venous malformations of Klippel-Trenaunay syndrome. J Vasc Surg Venous Lymphat Disord. 2017;5:587-95. https://doi.org/10.1016/j.jvsv.2016.10.084 
  4. Mahjoub A, Gilbert G, Turkula S, Mendez G, Graf K, Kim TWB. Treatment of femoral shaft fractures in patients with Klippel-Trenaunay syndrome: a report of 2 cases. JBJS Case Connect. 2021;11:e21.00136. https://doi.org/10.2106/JBJS.CC.21.00136 
  5. Nahas S, Wong F, Back D. A case of femoral fracture in Klippel Trenaunay syndrome. Case Rep Orthop. 2014;2014:548161. https://doi.org/10.1155/2014/548161 
  6. Gupta Y, Jha RK, Karn NK, Sah SK, Mishra BN, Bhattarai MK. Management of femoral shaft fracture in Klippel-Trenaunay syndrome with external fixator. Case Rep Orthop. 2016;2016:8505038. https://doi.org/10.1155/2016/8505038 
  7. Deshpande P, Chauhan R, Agrawal S, Rivi S, Nandan B, Dhawan M. Klippel-Trenaunay syndrome and femoral fracture: a literature review and case report. Curr Orthop Pract. 2022;33:204-7. https://doi.org/10.1097/BCO.0000000000001090 
  8. Barbara DW, Wilson JL. Anesthesia for surgery related to Klippel-Trenaunay syndrome: a review of 136 anesthetics. Anesth Analg. 2011;113:98-102. https://doi.org/10.1213/ANE.0b013e31821a03c2