Novel Management of a Femoral Fracture in Klippel-Trenaunay Syndrome

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder with a clinical triad of capillary malformations, vascular abnormalities, and bone/soft tissue hypertrophy. This is the first case of closed femoral shaft fracture in a patient with KTS managed by flexible intramedullary nails.

A 34-year-old patient sustained a right femoral mid-shaft spiral fracture after slipping on the grass. Due to a very narrow femur and large venous malformations, nail or plate fixation was impossible. Surgery was conducted using flexible intramedullary (TENS) nails with good reduction but significant bleeding which was controlled with tranexamic acid and CELOX. The patient required 4 units of red blood cells, 3 units of fresh frozen plasma, and 900 mL of cell saver intraoperatively with a further 2 units of RBC post-op. Fracture union was achieved 14 months after the initial fracture with additional pulsed ultrasound therapy.

Bleeding from vascular malformations during surgery makes operative management challenging in KTS patients. Previous studies have reported a variety of management strategies to achieve fracture fixation and union including IM nailing, plate fixation, and external fixators, but encountered significant bleeding of up to 10 units and 15 units, respectively. Ultrasound therapy has been utilized as a useful adjunct in lower limb fracture with delayed therapy.

Management of fractures in patients affected by KTS is extremely challenging despite extensive workup and planning to evaluate the optimal fixation method and explore strategies to reduce the risk of intra-operative bleeding. Management strategies should be tailored to the patient with close follow-up to assess fracture union.