Abstract
Congenital Pseudoarthrosis of the Tibia (CPT) is a rare condition with a reputation for recurrent fractures and failure to achieve union. A large variety of surgical procedures have been attempted for the treatment of fractured cases of CPT with an average rate of union without refracture of only 50%. Intentional cross-union between the tibia and fibula has been reported to improve these results to 100% union with no refractures. This is a retrospective study of 39 cases of CPT in 36 patients treated by the Paley cross-union protocol with internal fixation, bone grafting, zoledronic acid infusion and bone morphogenic protein 2 (BMP2) insertion. All 39 cases of CPT united at the tibia and developed a cross-union to the fibula. Two patients had a persistent fibular pseudarthrosis, one that was later treated at the time of planned rod exchange and one that has remained asymptomatic. There were few postoperative complications. There were no refractures during the up to 7-year follow-up period. The most common problem was the Fassier-Duval (FD) rod pulling through the proximal or distal physis into the metaphysis (66.7%). This did not negatively affect the results and was remedied at the time of the planned rod exchange. The Paley Cross-Union Protocol is very technically demanding, but the results have radically changed the prognosis of this once sinister disease.
Highlights
Congenital Pseudoarthrosis of the Tibia (CPT) is a rare condition with an incidence between 1:140,000 to 1:250,000 live births [1]
Beyond the physical and mental trauma that results from the interruption of childhood with repeated surgery, the likelihood of failed treatment may lead to the recommendation for amputation as a primary or secondary treatment [9]
In 2011, Choi et al [10] and in 2012, Paley [11] independently published their respective methods of treating patients with CPT by creating an intentional cross-union between the from the interruption of childhood with repeated surgery, the likelihood of failed treatment may lead to the recommendation for amputation as a primary or secondary treatment [9]
Summary
Congenital Pseudoarthrosis of the Tibia (CPT) is a rare condition with an incidence between 1:140,000 to 1:250,000 live births [1]. CPT is most commonly associated with neurofibromatosis (NF) in. Non-operative management with protective bracing is considered the standard of care in cases that have not yet broken. Once the bone has fractured, surgical management is indicated to achieve union and prevent refracture. A wide variety of surgical treatments have been tried for the management of CPT, with variable success. The average rate of union without refracture for all of these methods is approximately 50% [2,7]. Persistent nonunion or refracture leads to additional surgery and secondary changes in the leg, including ankle and knee contractures, malalignment, leg length discrepancy, calcaneocavus foot deformity, foot length shortening, calf muscular atrophy, gait alterations, coxa valga, and even hip dysplasia [8]. Beyond the physical and mental trauma that results from the interruption of childhood with repeated surgery, the likelihood of failed treatment may lead to the recommendation for amputation as a primary or secondary treatment [9]
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