Management of a femoral diaphyseal fracture in a patient with Klippel-Trenaunay-Weber syndrome: a case report

INTRODUCTION: Klippel-Trenaunay-Weber syndrome is a rare congenital disorder of the peripheral vascular system that is characterized by haemangiomas, soft tissue and/or osseous hypertrophy, venous and lymphatic anomalies as well as arterio-venous malformations. To our knowledge there are no documented cases of surgical fracture management in such patients. CASE PRESENTATION: We present the case of a 42-year-old female patient previously diagnosed with Klippel-Trenaunay-Weber syndrome, who had sustained a left femoral shaft fracture. She was treated with a closed, locked intramedullary nailing procedure. The nail was peripherally locked free-hand with a single screw due to the increased vascularity and intraoperative haemorrhage of the area. The patient was transfused with 7 units of blood intra-operatively and was subsequently transferred to the Intensive Care Unit where 3 more units of blood were transfused. Her post-operative course was uneventful. One year following the operation the fracture had united and the patient remained well. CONCLUSION: The surgical management of long bone fractures in patients with such pathology is associated with increased intra and post-operative risk, mainly due to vascular complications. It is therefore mandatory that high dependency facilities and sufficient quantities of blood products are available prior to the procedure. A less invasive fixation method should be used when possible.