Management of Femoral Shaft Fracture in Klippel-Trenaunay Syndrome with External Fixator

Gupta, Yogendra; Jha, Ranjib Kumar; Karn, NK; Sah, Sanjaya Kumar; Mishra, Bibhuti; Bhattarai, Manoj

doi:10.1155/2016/8505038

Cited by 8 publications

(16 citation statements)

References 5 publications

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“…In this case (a 12-year-old girl), the elastic intramedullary nail was quite difficult to insert due to poor bone quality and risk of cortical perforation. The fracture eventually united 4.5 months after surgery [18]. Although the present case lacked the diagnostic criteria for KTS, a more difficult situation existed in that the skin incision involved the AVMs, the femoral shaft was very thin, and closed reduction was difficult.…”

Section: Discussionmentioning

confidence: 96%

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Successful treatment of pathologic femoral shaft fracture associated with large arteriovenous malformations using a 3-dimensional external fixator and teriparatide: a case report

et al. 2019

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Background Arteriovenous malformations (AVMs) are rare congenital vascular lesions associated with early quiescence, late expansion, and, ultimately, infiltration and destruction of local soft tissue and bone. The extremities are a common location. Incidence of bony involvement by AVM has been reported as high as 31%. However, there are few reports on management of pathologic fracture associated with AVM. Teriparatide is a recombinant parathyroid hormone (PTH) analogue consisting of the 1–34 fragment of PTH. Recently, some reports have shown the ability of teriparatide to improve fracture healing. Here, we present a case of pathologic femoral shaft fracture associated with large AVMs that was treated successfully by external fixation and teriparatide. Case presentation A 68-year-old Japanese woman, previously diagnosed as having large AVMs, sustained a right femoral shaft fracture due to a fall. At the time of admission, she presented with massive swelling and venous varicosities of the right thigh. Plain radiography of the right thigh revealed femoral shaft fracture with bony erosion and calcification of soft tissue. We planned closed reduction and intramedullary nailing with a unilateral external fixator following embolization of the feeding artery. However, closed reduction using the fracture table was difficult. When we attempted open reduction, massive bleeding (1000 mL) after incision of subcutaneous tissue occurred. Finally, we carefully applied a Taylor Spatial Frame. Fracture displacement was corrected successfully and bony union was obtained with administration of teriparatide 15 months after the initial surgery. The patient is able to walk using 1 cane. Conclusion We present the first report of pathologic fracture associated with large AVMs that achieved bony union using a 3-dimensional external fixator and teriparatide.

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Section: Discussionmentioning

confidence: 96%

“…There are some reports of patients with Klippel-Trenaunay syndrome (KTS) who suffered femoral shaft fracture [17, 18]. KTS is characterized by capillary malformations and venous anomalies with bony and soft tissue hypertrophy in 1 or more limbs [19].…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of pathologic femoral shaft fracture associated with large arteriovenous malformations using a 3-dimensional external fixator and teriparatide: a case report

et al. 2019

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“…Nahas et al 4 reported a case of a 21-year-old male patient with a femoral fracture in 2014 who was managed with open reduction and locking compression plating. Also, Gupta et al 5 reported a 12-year-old child with KTS with femoral fracture who was managed with external fixation. The only other reported patient was from Zhu et al, 8 in whom limb salvage was not possible and amputation was done.…”

Section: Discussionmentioning

confidence: 99%

“…3 KTS occurs at a rate of one in 40,000 of the general population and is one out of nine vascular anomalies known to affect the extremities without significant arteriovenous shunting. 4,5 A diagnosis of Klippel-Trenaunay-Weber syndrome (or Parkes-Weber) can be made if signs of arteriovenous shunting are seen. 6 Clinical presentation can vary from merely asymptomatic disease to massive bleeding and embolism, which can be life threatening.…”

Section: Introductionmentioning

confidence: 99%

Klippel-Trenaunay syndrome and femoral fracture: a literature review and case report

Deshpande

Chauhan

Agrawal

et al. 2022

Current Orthopaedic Practice

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“…A fracture in the affected limb in patients with KTS can be difficult to manage; bones are abnormal, osteoporotic, and often narrow leading to a significant risk of profuse bleeding. There are only a few case reports in the literature on the management of fractures in this rare condition [3][4][5][6] with no consensus on management techniques [3,4].…”

Section: Introductionmentioning

confidence: 99%

Novel Management of a Femoral Fracture in Klippel-Trenaunay Syndrome

Patel

Hussain

Fuad

et al. 2022

Cureus

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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.

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Management of Femoral Shaft Fracture in Klippel-Trenaunay Syndrome with External Fixator

Cited by 8 publications

References 5 publications

Successful treatment of pathologic femoral shaft fracture associated with large arteriovenous malformations using a 3-dimensional external fixator and teriparatide: a case report

Successful treatment of pathologic femoral shaft fracture associated with large arteriovenous malformations using a 3-dimensional external fixator and teriparatide: a case report

Klippel-Trenaunay syndrome and femoral fracture: a literature review and case report

Novel Management of a Femoral Fracture in Klippel-Trenaunay Syndrome

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