Surgery for congenital tibial pseudoarthrosis remained difficult across a 12-year pediatric cohort
Congenital pseudoarthrosis of the tibia is one of pediatric orthopedics’ most stubborn problems: a fragile bone segment, poor vascularity, and fibrous tissue at the fracture site can turn a childhood tibial bend or fracture into a long-term mechanical failure. In a 12-year retrospective cohort from Nepal’s only specialized center for children with physical disabilities, surgical management produced uneven results and repeated operations were common. Among 70 children with congenital pseudoarthrosis of the tibia, the average age at presentation was 3.84 years and the average age at first surgery was 5.38 years, a delay that likely reflected both referral patterns and the practical difficulty of treating a rare disorder in a resource-limited setting. Boys made up 57.1% of the cohort, and most lesions were classified as Boyd type 5 and Crawford type 4, pointing to advanced, complex disease in many of the children studied.
The clinical challenge begins long before the operating room. CPT is not simply a broken tibia that refuses to heal; it is a dysplastic segment with abnormal periosteum, impaired perfusion, and hamartomatous fibrous tissue that resists normal remodeling. Progressive anterolateral bowing, ankle valgus, fibular migration, and limb shortening can compound the functional burden and raise the stakes for any reconstructive plan. In practice, the surgeon is trying to restore stability in bone that behaves unlike normal tibia, while also anticipating refracture after initial union. The cohort’s mean limb-length discrepancy of 2.51 cm, with a range extending to 14 cm, reflects how much deformity can accumulate before definitive reconstruction.
Among the 35 patients who underwent surgery, the overall union rate reached 41.93% among 31 evaluable surgical cases, and 23.08% of those who united later refractured. Those numbers are sobering, but they fit the biology of the disease. Even after radiographic bridging appears, the repaired segment may remain vulnerable if the abnormal local environment is not fully corrected. Radiologic union in this study required bridging of the fracture gap, disappearance of the fracture line, and at least three of four cortices bridged; clinical union also required absence of pain, no tenderness, and ability to bear weight. This dual definition matters in CPT, where apparent stability on imaging may still fail under load if the construct and biology are not strong enough.
Periosteal grafting and Masquelet reconstruction produced similar midrange results
The center used several reconstructive strategies over time, including periosteal grafting, the Masquelet technique, Ilizarov-based stabilization, and intentional cross-union concepts adapted for local feasibility. Periosteal grafting achieved a 50% union rate in the treated subgroup, while the Masquelet technique produced a 41.67% union rate. Neither result solves the problem, yet both show that biologically oriented reconstruction can still generate healing in a disease marked by persistent pseudarthrosis. Periosteal grafting, especially when paired with intramedullary stabilization and ring fixation, aims to replace diseased tissue and provide osteogenic stimulus from the iliac wing. The Masquelet approach, used in two stages, attempts to create a more favorable membrane environment before definitive bone reconstruction, a strategy that makes sense when the local tissue bed is hostile to consolidation.
The cohort also shows how much the chosen operation is shaped by age, size, and anatomy. Children younger than four years were sometimes observed rather than immediately reconstructed because some had not yet fractured, while others were waiting for surgery. Ten children were lost to follow-up after receiving elective dates, a reminder that rare-disease care often depends on long travel distances, repeated visits, and family resources as much as on surgical theory. Eighty-one children were identified in the electronic database, but 11 were excluded because radiographs could not be retrieved. That attrition did not erase the central message: in CPT, the pathway to union is narrow, and the path back from refracture is even narrower.
Repeated operations, long immobilization, and retained fixation defined postoperative care
Postoperative management was intensive. Except for children stabilized with Ilizarov rings, patients were immobilized in a posterior slab until suture removal on day 14, then placed in an above-knee cast that was changed at follow-up. Non-weight-bearing ambulation with crutches was advised for all cases. When union was achieved, external bracing was added for protection and internal fixation was retained to support consolidation. Those steps reflect the underlying problem: even when the tibia bridges, it often needs prolonged mechanical support because the diseased segment has not fully normalized biologically.
A few elements of the operative strategy are especially relevant for orthopedic surgeons working with similar patients. The team used intramedullary rods to stabilize several constructs, supplemented some cases with Ilizarov fixation, and adapted cross-union surgery by using a plate on the tibia rather than circular external fixation. Bone morphogenetic protein was not used because of cost and availability. Bisphosphonate infusion was given two weeks before and two weeks after surgery in an effort to reduce osteoclastic activity, which fits the disease model of excessive bone resorption and failed remodeling. Each of these decisions reflects an attempt to compensate for weak local biology with mechanical support and targeted adjuncts.
Counseling families is as important as selecting the operation
CPT management rarely ends with a single successful operation. The mean number of surgeries per patient in this cohort was 2.84, showing how often children require iterative correction, revision, or protection before the limb becomes functionally reliable. Families need clear guidance about the possibility of nonunion, the risk of refracture after apparent healing, and the likelihood of prolonged bracing or additional procedures. In a disorder where the tibia can bow again, fracture again, or fail to consolidate despite technically sound surgery, informed counseling is part of treatment rather than an afterthought.
The Nepal experience also reinforces the value of individualized surgical planning. Age at presentation, limb-length discrepancy, lesion classification, and the state of the fibula all influence the operative choice, the durability of fixation, and the likely need for future procedures. The study does not identify a single superior technique, and it does not need to. Its more practical message is that congenital pseudoarthrosis of the tibia remains a high-failure, high-revision condition in which union rates can be modest even in specialized hands. The cohort’s 41.93% union rate and 23.08% refracture rate among unions make that reality difficult to ignore, especially in children who may face years of follow-up before a stable, functional limb is secured.