1. MANAGEMENT OF CONGENITAL PSEUDARTHROSIS OF TIBIA
WHITE TEAM

2. Management
Congenital pseudarthrosis of the tibia (CPT) is a rare pathology
The natural history of the disease is extremely unfavorable and once a fracture occurs, there is a little or no tendency for the lesion to heal spontaneously
obtain a stable, functional extremity at the completion of treatment, it is essential to set realistic goals and to adhere to the treatment principles and technical details

3. Management…
It is challenging to effectively treat this condition and its possible complications
Treatment goal is to obtain a stable and functional extremity at the completion of treatment
It is essential to set realistic goals and to adhere to the treatment principles and technical details
The treatment is multidisciplinary and multi modal

4. Aims Long term bony union without axial or rotational malalignment
Stabilize the ankle mortise for good foot and ankle function
Lower limb-length equalization

5. Modalities of treatment
Operative
Bone grafting
Intramedullary nail
Microvascular fibular graft
External fixator
Combination
Amputation
Non operative
Splints
Electrical stimulation, low intensity pulse ultrasound
Bisphosphonates
Ultrasound
Okada et al. reported a case of CPT of the tibia (Boyd type IV) successfully treated with low-intensity pulsed ultrasound stimulation (LIPUS) administered for 20 min/day. The treatment was continued for 1 year until solid fusion on radiographs and subsequent full-weight-bearing was achieved.67 The underlying mechanisms of action of LIPUS remain unclear. However, in experimental studies conducted in rats, LIPUS application facilitates union and increase mechanical strength of bone

6. Operative Indication crawford iii and iv CPT Principles
Complete excision of harmatomatous tissues
Stable fixation
Biologic bridging of the bone defect
Correction of deformities
Prevention of refracture and other complications

7. Vascularized fibular graft
Complications
New-onset fractures/ non union
Malalignment and valgus deformity
Valgus deformity on the donor site
The limitations of this technique are cost, technical complexity, poor protection against re-fracture, failure to correct limb length discrepancy, and deformities of leg and ankle simultaneously at time of primary surgery.

8. Vascularized fibular graft/ Nail
The intramedullary rod offers good tibial alignment and prevents refracture
Complications
Valgus ankle deformity on the donor side
Recurrent nonunion at one end of the graft site
Residual limb-length discrepancy
according to the technique described by O’Brien.52 Initial consolidation occurred in every case and no fractures were recorded.53 The goal of this original technique is to obtain bone union, by mixing propitious biological environment with the vascularized bone graft,19,20 and the intramedullary rod is responsible for stability.

9. Ilizarov device Allows total resection of pathological tissue
Ensures stability regardless of the amount of resected tissue
Can exert compression at fracture site

10. Ilizarov device…
Allows extension of both tibia and fibular to bridge defect and prevent ankle valgus
Correction of axial deformities, and
Allow full support immediately after the intervention

11. Ilizarov device… External fixator can be cumbersome for small children
There is high frequency of re-fracture
Circular external fixation procedure takes a long time
Pin tract infection
If the child has been protected with internal splint (intramedullary nailing) and bracing till skeletal maturity, the frequency of the re-fracture can be reduced significantly

12. After care
Following successful treatment, limb should be splinted till skeletal maturity

13. Combine pharmacological and surgical
Surgical resection of harmatomatous tissues
Application of periosteal graft, cancellous bone graft and bone morphogenic protein
Stabilization with circular frame and intramedullary nail
Systemic bisphosphonate

14. Amputation
For resistant pseudarthrosis when other extensive surgical procedures have not achieved a functional extremity, either due to persistent nonunion or due to dysfunctional angular deformity, shortening, atrophy, and stiffness, the amputation is entirely appropriate

15. Complications Re-fracture
The frequency of re-fracture after primary union varies from 14% to 60%
Anatomic alignment of the tibia and fibula minimize the risk of re-fracture.
Intramedullary rod and external bracing must be continued as effective protection against re-fractures

16. Complications… Malalignment of the tibia
Diaphyseal malalignment of the tibia (procurvatum or valgus deformity) are progressive and do not remodel
The deformities of the proximal tibia can be corrected with osteotomy if the morphology of the tibia is normal with external fixator
The deformity correction with osteotomy is contraindicated through dysplastic tibia morphology, as it can lead to fresh pseudarthrosis.

17. Complications… Limb length discrepancy
The affected tibia is slightly shorter than the normal side from beginning
Progressive shortening of the leg occurs as long as the pseudarthrosis remains ununited and also associated with repeated unsuccessful operations
Residual limb length discrepancy following successful union is a major problem
Growth abnormalities of the tibia, fibula, and the ipsilateral femur abnormalities are also noted with CPT, which include inclination of the proximal tibial physis, posterior bowing of the proximal third of the tibial diaphysis, proximal migration of the lateral malleolus.41

18. Complications… Proximal tibial lengthening by distraction osteogenesis
Contralateral epiphyseodesis of the femur and/or tibia can be done for expected limb length discrepancy less than 5 cm at skeletal maturity

19. Complications… Ankle valgus
Tibiofibular metaphyseal synostosis (the Langenskiöld procedure)
Distal tibial medial hemi-epiphysiodesis with a malleolar screw

20. Complications… Ankle stiffness
Ankle stiffness usually progressively regresses once intramedullary rod is removed from ankle.

21. Prognosis
Severity of CPT
Age at surgery
Treatment

22. Result of treatment Author No of cases Treatment % union
Refracture rate (%)
Boyd & Sage 1958 91
Bone grafting 56
Joseph & Mathew 2000 14
Im rod +double onlay tibial graft 86 21
Johnson 2002 23
Im rod + bone graft 87
Ohunushi 2005 26
Ilizarov
100 25
Vascularized fibular 88 7
Combination
Paley et al 1992 15 94 35 17
Ilizarov + im nail 29

23. Conclusion CPT remain a challenge to both patient and his surgeons
Just as the search of its aetiology continued, so also its effective treatment
Combination treatment with ilizarov frame seems to be the most effective form of treatment