What is the treatment for congenital pseudoarthrosis of the tibia?

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Treatment of Congenital Pseudoarthrosis of the Tibia

The optimal treatment for congenital pseudoarthrosis of the tibia (CPT) involves a combined approach of complete excision of the pseudarthrosis site, autogenous bone grafting, and fixation with both external fixation (Ilizarov) and intramedullary fixation to achieve the highest union rates and lowest refracture rates. 1

Surgical Management Algorithm

Timing of Intervention

  • Operative management is indicated for all patients over 2 years old (100% consensus agreement) 1

Primary Surgical Approach

  1. Complete excision of the pseudarthrosis site (100% consensus agreement) 1
  2. Sufficient autogenous bone grafting (100% consensus agreement) 1
  3. Combined fixation technique (84% consensus agreement) 1
    • External fixation (Ilizarov) combined with intramedullary fixation
    • This combination prevents refractures and axial deformities
    • Achieves 84% primary union rate and 93.3% final union rate 1
    • Mean primary union time: 5.62 months 1
    • Refracture rate: 22.3% 1

Alternative Approaches (Based on Specific Scenarios)

Cross-Union Technique

  • Shows excellent results with 100% primary union rate 1
  • Mean union time: 4.5 months 1
  • Refracture rate: 22.5% 1
  • Creates tibiofibular fusion to increase stability 1
  • Particularly beneficial for younger patients or those with high-risk anatomical features 1

Ilizarov Method Alone

  • Not recommended as primary treatment (only 21% consensus agreement) 1
  • Can be considered as a secondary option when other methods have failed 1
  • Useful for patients with angulation, impending refractures, or limb length discrepancies >5cm 1
  • Primary union rate: 89% 1
  • Mean union time: 7.14 months 1
  • Refracture rate: 81% 1

Vascularized Fibular Graft (VFG)

  • Should be reserved for cases where other techniques have failed 2
  • Primary union rate: 65.3% 1
  • Final union rate: 88.2% 1
  • Refracture rate: 45% 1
  • Higher donor site morbidity 2

Fixation Methods to Avoid as Standalone Treatments

  • Intramedullary rods (IMR) alone - 74% disagreement with using alone 1

    • Primary union rate: only 69% 1
    • Mean union time: 8.95 months 1
    • Refracture rate: 85% 1
    • Non-union rate: 16.4% 1

  • Fixed nails (Rush rods) - 74% disagreement with using alone 1

  • Telescopic nails (Fassier-Duval) - 53% disagreement with using alone 1

    • Though recent studies show improved primary union rates (85.7%) with no refractures 1

  • Locking compression plate (LCP) - Not recommended alone (only 21% agreement) 1

Adjunctive Treatments

  • Recombinant human BMPs (rhBMP7 and 2) - Not recommended (89% disagreement) 1

  • Bisphosphonates - Insufficient evidence (84% neutral) 1

  • Electric stimulation/pulsed electromagnetic stimulation - Insufficient evidence (74% neutral) 1

  • Low-intensity pulsed ultrasound stimulation - Insufficient evidence (63% neutral) 1

Monitoring and Follow-up

  • Long-term follow-up is essential as refractures can occur up to 18 years after fusion 1
  • Monitor for:
    • Ankle function
    • Limb length discrepancies
    • Deformity correction
    • Signs of refracture

Complications to Anticipate

  • Refracture - Most common complication, varies by technique:

    • IMR alone: 85% 1
    • Ilizarov alone: 81% 1
    • Combined techniques: 60% 1
    • VFG: 45% 1
    • Cross-union: 22.5% 1

  • Non-union - Varies by technique:

    • IMR alone: 16.4% 1
    • Ilizarov alone: 12.3% 1
    • Combined techniques: 6% 1
    • VFG: 7.3% 1

  • Limb length discrepancies - Common, especially with IMR alone 1

  • Ankle stiffness - More common with IMR alone 1

Clinical Pearls and Pitfalls

  • The repaired bone in CPT remains biologically and mechanically inferior even after remodeling and skeletal maturity 1
  • Success should be measured not just by radiographic union but by functional outcomes 1
  • Avoid using single fixation methods alone, as they have significantly higher refracture rates 1
  • Combined techniques offer the best balance of union rates and complication profiles 1, 3
  • Consider the cross-union technique for severe cases due to its superior union rate and lower refracture rate 1, 4

References

1
Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

2
Guideline

Bone Grafting in Young Females

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

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Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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