Treatment of Femoral Shaft Fractures in Patients with Klippel-Trénaunay Syndrome: A Report of 2 Cases. Issue 3 (July 2021)

• Record Type: Journal Article
• Authors: Mahjoub, Adel; Gilbert, Grace; Turkula, Stefan; Mendez, Gregory; Graf, Kenneth; Kim, Tae Won B.
• Is Part Of: JBJS case connector. Volume 11:Issue 3(2021)
• Publication Date: 2021-07
• Subjects: endoprosthetic reconstruction -- KTS -- Klippel-Trénaunay syndrome -- Parkes-Weber syndrome -- PWS -- orthopaedic oncology -- intramedullary nail -- femoral shaft fracture -- diaphyseal femur fractures -- fracture nonunion -- internal tourniquet -- adult -- female -- IM nail -- external fixator -- balanced traction -- proximal femoral replacement -- radical resection -- total hip arthroplasty -- intercalary replacement -- ambulatory dysfunction -- hemorrhage -- open reduction internal fixation -- capillary hemangioma -- venous varicosities; Orthopedic surgery -- Case studies -- Periodicals; Orthopedic surgery -- Periodicals; Orthopedics; Orthopedic Procedures; Orthopedic surgery; Periodicals; Electronic journals; Case studies; Periodicals; 616.7
• DOI: 10.2106/JBJS.CC.21.00136
• Languages: English
• ISSNs: 2160-3251

Abstract

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.