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.