Free fibula flap to achieve bone union after congenital pseudoarthrosis of the forearm in neurofibromatosis: Technical report based on 3 cases, and literature review

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Congenital pseudarthrosis of the long bones (CPFB) is rare with only 106 reported cases and frequently associated with neurofibromatosis (NF). Approximately 5% of patients with NF develop a pseudarthrosis, and 50% of patients with pseudarthrosis have NF.Achieving bone union is difficult in congenital pseudarthrosis. Many methods have been attempted including casting, internal fixation with or without grafting, and electrical stimulation, but failure is frequent. Free vascularised fibular flaps (FVF) have been used to bridge long bone defects since 1975 and in tibial pseudarthrosis since 1979.In CPFB, FVF’s have been found to be more successful than other methods in achieving union and is currently the treatment of choice. Here, we present three cases of forearm pseudarthrosis treated with FVF, review the literature on CPFB and discuss some of the technical aspects of FVF treatment.Three cases of congenital pseudoarthrosis were treated with free fibula flaps, diagnosed aged 7 (ulna); 15 months (radius) and 9 years (radius and ulna). Two flaps were stabilised with IM wires and latterly, one with compression plates. One persistent non-union received revision non-vascularised bone grafting and plating. All patients achieved union by 11 months post index surgery.Reconstruction with vascularised fibula is the treatment of choice as it offers the highest published union rates and good functional results. Complete resection of affected bone and stable fixation, latterly with compression plates are critical to success. Surgery is technically demanding; complications are common and secondary surgery may be required but outcomes are favourable. Level of evidenceIV