Abstract

Treatment of congenital pseudarthrosis of the tibia remains a major challenge in pediatric orthopedics. Ideal timing and preference of surgical procedures are discussed controversially. A variety of reconstructive treatment strategies have been described in literature, but so far none has proven its superiority. The aim of treatment is to obtain long-term bone union, to prevent refracture, and to correct angular deformities and leg length discrepancies. This study retrospectively evaluates the outcome of different reconstructive strategies. Sixty-nine patients were identified who presented to our outpatient department between 1997 and 2019. Twenty-six of these patients underwent reconstructive surgical treatment and were included in this study. The study cohort was divided into three groups. Excision of the pseudarthrosis was performed in all patients in Group A and B, and in two patients of Group C. Group A (six/26 patients) received subsequent bone transport through external fixation maintaining original length. In Group B (15/26 patients), patients underwent either previous, simultaneous, or subsequent extrafocal lengthening through external fixation to reconstitute length. In Group C (five/26 patients), internal fixation with intramedullary nails was applied. Radiological and clinical evaluation was performed to assess bone union and complication rates. Results varied considerably between the study groups. Overall, the primary bone fusion rate was 69.2%. There were four refractures, all occurring in Group B. The long-term bone union rate without refracture was 53.8%. The overall complication rate was 53.8% and 23.1% showed persistent pseudarthrosis. Two secondary amputations were performed due to failed bone fusion. In conclusion, excision of the pseudarthrosis and extrafocal lengthening achieves a satisfying bone union rate and limb reconstruction, while bone transport does not offer significant advantages but shows higher complication rates. Intramedullary stabilization should be applied to maintain bone union, but shows lower bone union rates when used as a stand-alone treatment regimen. Regardless of the primary bone fusion rates, the probability of long-term bone union remains unpredictable.

Highlights

  • Congenital pseudarthrosis of the tibia (CPT) is a rare condition of unclear etiology [1]

  • This study evaluates the outcome of three different reconstructive strategies for treatment of CPT focusing on bone fusion rates, secondary complications, and outcome

  • Primary bone union was achieved in three patients (50% primary bone union rate)

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Summary

Introduction

Congenital pseudarthrosis of the tibia (CPT) is a rare condition of unclear etiology [1]. In most cases the pseudarthrosis is not yet present at birth, but the tibia shows a characteristic anterolateral bowing which deteriorates during growth (Figure 1). A fracture frequently develops spontaneously or after a minor trauma. This leads to a pseudarthrosis with atrophy of the bone ends and a surrounding soft tissue hamartoma impeding physiological healing [7,8,9]. The loss of remodeling potential and the growth inhibition of the tibia lead to further deterioration of the deformity and to leg length discrepancy (LLD) [7]

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