Congenital pseudoarthrosis is a rare disorder, and mostly occurs in the radius, ulna, tibia, and fibula, but is not reported in the femur. Congenital cases that were reported in the literature, most were associated with neurofibromatosis, cleidocranial dysostosis, congenital constriction band syndrome and fibrous dysplasia and the acquired cases followed fractures of bones or tuberculosis. Since the life span of the patient is affected rarely, clinical examination of these patients and serial radiographs can give us plenty of information about the limb abnormality, its clinical course, and the nature of the underlying disturbance of growth. A 3 year female child presents to the outpatient department of a tertiary care hospital with complaints of pain, swelling and deformity thigh region right side for 5 months. On examination, Swelling was present [2x2 cm] at the junction of the mid and distal thigh, smooth surface, ill defined edges, soft and non tender. There was abnormal mobility which was noted at the right thigh at the junction of the middle and distal 1/3 right femurMRI reported circumferential periosteal reaction with cortical surface irregularity of the right femoral shaft was also noted. The findings were reported to be concerning for chronic osteomyelitis with non-united fracture & cortical resorption/chronic bone loss. Histopathological sections showed multiple bits of viable bony trabeculae with surrounding fibro-collagenous tissue displaying significant proliferation of fibroblasts, slit-like blood vessels, arterioles and moderate mixed inflammatory infiltrate of plasma cells, few neutrophils, lymphocytes, histiocytes and few multinucleated histiocytes.She was managed with resection of pseudoarthrosis and masquelet procedure stage 1 and later masquelet stage 2 (cement spacer removal, ipsilateral ileac crest bone grafting with fixation with 7-hole distal fibular anatomical plate and bone graft substitute placement [ChronOs]). Idiopathic or primary congenital pseudoarthrosis can involve the femur as well as other long bones. It may or may not be associated with hip dysplasia and the outcome may not be as bad as previously reported in the pseudoarthrosis of other long bones.

Congenital pseudarthrosis of the forearm bones (CPFBs) is rare, with only 106 reported cases, and is frequently associated with neurofibromatosis (NF). Approximately 5% of patients with NF develop 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 vascularized fibular flaps (FVFs) have been used to bridge long bone defects since 1975 and in tibial pseudarthrosis since 1979. In CPFB, FVF is more successful than other methods in achieving union and is the current treatment of choice. Here, we presented three cases of forearm pseudarthrosis treated with FVF, reviewed the literature on CPFB, and discussed some technical aspects of FVF treatment. Three cases of congenital pseudoarthrosis were treated with free fibula flaps, diagnosed at ages of 7 years (ulna), 15 months (radius), and 9 years (radius and ulna). Two flaps were stabilized with intramedullary wires and latterly, one with compression plates. One persistent nonunion received revision nonvascularized bone grafting and plating. All patients achieved union by 11 months after index surgery. Reconstruction with vascularized fibula is the treatment of choice because it offers the highest published union rates and good functional results. Complete resection of the affected bone and stable fixation, latterly with compression plates are critical to success. Surgery is technically demanding, and complications are common. Secondary surgery may be required, but outcomes are favorable. LEVEL OF EVIDENCE: IV.

Congenital pseudarthrosis of the forearm poses a considerable challenge because of its rarity. The objective of this report is to introduce a novel surgical technique for its treatment. Here, we document a case of congenital pseudarthrosis of the radius in a 3-year-old boy diagnosed with type-1 neurofibromatosis. The surgical treatment involved the excision of approximately 9 cm of native radial periosteum and a bifocal radius osteotomy, which was supplemented with a vascularized tibial periosteal transplant to facilitate bone healing. Anastomosis between the anterior tibial vessels and radial vessels was performed. No immediate or late postoperative complications were observed. After 3 weeks, a robust callus formation was observed, and during a follow-up examination 3 years and 4 months later, a wide range of active forearm rotation was noted. This report suggests that vascularized periosteal flaps show promise as a viable treatment option for congenital pseudarthrosis of the forearm. They offer an alternative to vascularized fibular grafts or single-bone forearm constructs.

Although congenital pseudoarthrosis of the radius is a rare case, it has been reported many times in the literature in the past and it has been shown that it can be associated with neurofibromatosis type 1. However, traumatic radius pseudoarthrosis has never been reported before. In this case report, all treatment options were applied to the same patient over a four-year period and the results were reported. A two-year-old boy had a left radius diaphysis fracture after a simple fall, and bone union could not be achieved despite the application of cast immobilization, internal fixation grafting, and electrical stimulation in an external center. He was admitted to our hospital when he was six years old. A plain X-ray image and computed tomography scan showed that he had radius pseudoarthrosis and also he was diagnosed with neurofibromatosis type 1 on genetic analysis. The patient underwent wide resection of the segment with pseudoarthrosis and free vascularized fibula grafting. Bone union was achieved in the third postoperative month.