Surgical treatment of congenital pseudarthrosis of the tibia in children: CPAM-LRC consensus and guidelines.

PURPOSE

Congenital pseudarthrosis of the tibia (CPT) is a rare condition typically manifesting within the first decade of life. The primary objectives of surgical intervention for CPT include achieving long-term bony union of the tibia, preventing or minimizing limb length discrepancies (LLD), avoiding mechanical axis deviations of the tibia and adjacent joints, and preventing refracture. This study aims to conduct a systematic review of current treatment methods for CPT to determine the most effective non-surgical and surgical management strategies for pediatric patients with this condition.

METHODS

A review of the literature was performed according to the PRISMA guidelines. A comprehensive search of PubMed, Medline, CINAHL, Cochrane, Embase, and Google Scholar databases was performed over the years 1989 to 2024. Randomized controlled trials, cohort studies and case-control studies on the surgical treatments of CPT in children were included. Random-effects models were used to estimate the pooled primary union rate, primary union time and refracture rate. Then a consensus statement of surgical treatment of CPT in children was achieved based on Delphi methodology which included 2 rounds of electronic questionnaires and 1 round of virtual consensus meeting.

RESULTS

Seventy-four studies were included, 23 of which on intramedullary rods (IMR) involving 364 patients, 15 on Ilizarov methods with 230 patients, and 23 studies combining IMR with Ilizarov involving 458 patients. The pooled primary union rate for IMR, Ilizarov method, and Ilizarov combined with IMR was 69% (95% CI: 0.55-0.82), 89% (95% CI: 0.77-0.98) and 85% (95% CI: 0.74-0.94), respectively. The pooled primary union time for IMR, Ilizarov method, and Ilizarov combined with IMR was 8.95 months (95% CI: 6.53-11.37), 7.14 months (95% CI: 5.53-8.74), and 5.62 months (95% CI: 4.89-6.35), respectively. The pooled refracture rate among primary unions for IMR, Ilizarov method, and Ilizarov combined with IMR was 85% (95% CI: 0.60-1.00), 81% (95% CI: 0.56-0.98), and 60% (95% CI: 0.26-0.90), respectively. After 2 rounds of electronic questionnaires and 1 round of virtual consensus meeting, a consensus statement and guidelines of surgical treatment of CPT in children was provided.

CONCLUSION

The CPAM-LRC consensus panel provided recommendations to improve management of pediatric patients with CPT in clinical practice. Combined techniques for pediatric patients after fracture or the formation of pseudarthrosis commonly include excision of the pseudarthrosis site, external fixation, intramedullary fixation, and autogenous bone grafting. Vascularized fibular graft and cross-union can be considered a viable alternative to corticocancellous autograft. Use of recombinant bone morphogenic protein (rhBMP) is not currently conclusive.