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

Abstract

IntroductionKlippel-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 presentationWe 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.ConclusionThe 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.

Klippel-Trenaunay syndrome: a rare cause of disabling pain after a femoral fracture

“…In literature, only one previous case of surgical treatment of a diaphyseal femoral fracture in a patient aVected by KTS has been described [1]. In this case, too, the authors opted for mini-invasive treatment, although seven units of blood transfusion were required during the procedure and three postoperatively [1], while four were suYcient in our patient.…”

“…In this case, too, the authors opted for mini-invasive treatment, although seven units of blood transfusion were required during the procedure and three postoperatively [1], while four were suYcient in our patient.…”

“…Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder of the vascular system, characterized by the presence of two of the following triad of clinical signs: (1) haemangiomas due to cutaneous capillary dysplasias; (2) soft tissue and/or bone hypertrophy; (3) venous and lymphatic anomalies [1]. We report a case of a picture of severe disabling neuropathic pain emerged in KTS patient that complicated the post-traumatic and peri-operative clinical management.…”

Carpal Tunnel Syndrome Associated with Klippel-Trénaunay-Weber Syndrome

“…MRI demonstrated an abnormally large nerve but showed no other causes of extrinsic or intrinsic compression. Surgical treatment has been described for the relief of a range of symptoms in patients with Klippel-Trénaunay-Weber syndrome 15 , but these procedures remain controversial because they are associated with limited benefits and high rates of complications 16,17 . Surgical treatment of such patients is also known to become more difficult as these patients get older and the hypertrophy and vascular anomalies continue to develop 18 .…”

“…Surgical treatment was debated for this patient given the known high-risk nature of surgery in patients with Klippel-Trénaunay-Weber syndrome 16,17 as well as the duration and severity of the symptoms, which brought into question the degree of benefit that could be achieved. It is likely that permanent nerve damage had already been done.…”