Congenital Pseudarthrosis of the Tibia

Abstract

Background: This study assessed the outcomes, at skeletal maturity, for 34 patients in whom congenital pseudarthrosis of the tibia (CPT) had been treated with intramedullary (IM) rod fixation. Methods: The results in skeletally mature patients in whom type-4 CPT had been treated with an IM rod at an average of 11.9 years earlier were reviewed. The rod procedures varied according to whether both the tibia and the fibula were resected and both bones (type A) or just the tibia (type B) were fixed with an IM rod or whether only the tibia was resected and the fibula received no surgery (type C). Outcome grading ranged from unequivocal union with brace-free function (grade 1) to a functional limb with residual angulation or cortical defects (grade 2) to a severely impaired extremity with insufficient union or refracture (grade 3). Results: Thirty-four patients were evaluated at a mean age of 16.9 years, a mean of 11.9 years after their initial surgical procedure. Seventeen patients had a grade-1 result; 11 patients, grade 2; and 6, grade 3. Thus, 82% (28) of the 34 patients had a functional extremity at maturity. All patients with a final grade-3 outcome eventually requested amputation. The final outcomes were not affected by the age at the initial fracture or surgery, the presence of neurofibromatosis-1, or cross-ankle fixation. A total of 58 IM rod procedures were performed in the 34 patients. Twenty-four (73%) of the 33 type-A procedures produced grade-1 or 2 outcomes, as did 14 (88%) of the 16 type-B procedures. Of the 9 type-C procedures, none produced a grade-1 result and 4 produced a grade-2 outcome. The results of types A and B combined were superior to those of type-C procedures (p = 0.03). Refracture occurred in 13 of 33 patients with initial stability/union after rod fixation, with 3 of those fractures remaining ununited at the latest follow-up. A dystrophic fibula had no effect on the eventual achievement of a grade-1 or 2 outcome. Conclusions: This review, in which all patients had reached skeletal maturity, documents functional (grade-1 or 2) outcomes in 82% of cases of IM rod fixation for CPT. This finding was almost identical to the result in our earlier report and confirms the long-term value of permanent IM rod fixation in maintaining union and function in patients with CPT. Procedures not addressing the fibula produced inferior results. Although 13 patients had a refracture following initial union, only 3 of these fractures failed to heal with additional treatment. Level of Evidence: Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.

Surgical treatment options for congenital pseudarthrosis of tibia in children: cross-union versus other options: a systematic review

“…Association with NF1 is considered to be a negative predictor for bone union, refractures and final outcome in CPT by certain studies [46,60,62,65,66]. However, others have reported no effect on final results with comparable rates of union in NF and non-NF groups [13,18,24,26,27,30,46,52,53,60,64].…”

“…The following information was extracted and recorded into excel sheets: number of patients/tibias with CPT, number of patients/fibulas with fibular pseudarthrosis (FPA), presence/absence of NF1, mean age at surgery (years), mean duration of follow-up, type of surgical intervention, type of union, mean time to union (months), number of non-unions, number of refractures, bone graft (BG) source and adjuvants used (Tables 1–5) [1–57]. The type of union was categorized as primary (cases which united after index surgery), secondary (cases requiring additional intervention after index surgery for union) and final (overall unions including primary and secondary unions).…”

Clinical study of a new type of telescopic rod for the treatment of congenital pseudarthrosis of the tibia in children

“…Dustin Singer et al . [10] reported that 34 children with CPT were treated with the intramedullary rod, with an average follow-up of 11.9 years. Although 13 cases had re-fracture (38%), most of them obtained tibial pseudarthrosis healing through bone grafting and other operations.…”

“…The etiology of congenital pseudarthrosis of the tibia (CPT) is unknown [1], which is one of the refractory diseases in children's orthopedics [2][3][4][5][6][7][8][9][10]. With the improvement of surgical technology, the healing rate of CPT has improved significantly.…”

Outcome of Congenital Pseudoarthrosis Tibia Treated with Illizarov Fixator in Children

“…Type of surgery to achieve union and prevent refracture remains controversial. Initial management is resection of entire pseudoarthrosis and surrounding hamartomatous tissue [6] . Atrophic type of CPT is more challenging.…”