Treatment of Femoral Shaft Fractures in Patients with Klippel-Trénaunay Syndrome

Abstract

Cases: Long-bone fractures in patients with Klippel-Trénaunay syndrome (KTS), a rare disorder of the venous, lymphatic, and capillary system, are difficult to treat with many complications. Two patients diagnosed with KTS presented with closed femoral shaft fractures after low-energy falls. Conservative treatment, open reduction internal fixation, and intramedullary nailing resulted in painful nonunions. Ultimately, both patients achieved pain relief and the ability to ambulate after en bloc resection and reconstruction. Conclusions: These cases demonstrate the challenges in achieving bony union when treating long-bone fractures in KTS. The feasibility of undergoing extensive resection and reconstruction to regain function is best approached with a multidisciplinary team.

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

“…There are only a few cases involving treatment of a femoral shaft fracture in KTWS and KTS patients and poor results were reported in these cases. Mahjoub et al 4) reported that conservative treatment such as skeletal traction and operative treatment such as open reduction and internal fixation (ORIF) or IM nail showed poor results in two cases of femoral shaft fracture in KTS patients. They eventually performed en bloc resection and reconstruction and insisted that it is a viable option 4) .…”

“…Mahjoub et al 4) reported that conservative treatment such as skeletal traction and operative treatment such as open reduction and internal fixation (ORIF) or IM nail showed poor results in two cases of femoral shaft fracture in KTS patients. They eventually performed en bloc resection and reconstruction and insisted that it is a viable option 4) . Nahas et al 5) reported that ORIF for treatment of femoral shaft fracture in KTS patients resulted in delayed union, and management with low intensity pulsed ultrasound was subsequently administered.…”

“…Barbara and Wilson 8) reported that mean estimated bleeding was 740±2,739 mL (range, 20 to 18,000 mL) in 82 patients. Mahjoub et al 4) estimated intraoperative blood loss of 3 L during performance of resection and reconstruction procedures. Nahas et al 5) reported that 15 units of blood were required during the operation.…”

“…Excessive and uncontrollable bleeding can lead to poor results and intensive care. Many surgeons used an intraoperative common iliac artery balloon as a tourniquet for control of intraoperative bleeding 4 8) . However, a complicated surgical procedure and extended operative time may be required.…”