Presentation on theme: "MANAGEMENT OF CONGENITAL PSEUDARTHROSIS OF TIBIA"— Presentation transcript:
1 MANAGEMENT OF CONGENITAL PSEUDARTHROSIS OF TIBIA WHITE TEAM
2 ManagementCongenital pseudarthrosis of the tibia (CPT) is a rare pathologyThe 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 spontaneouslyobtain 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 complicationsTreatment goal is to obtain a stable and functional extremity at the completion of treatmentIt is essential to set realistic goals and to adhere to the treatment principles and technical detailsThe 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 functionLower limb-length equalization
5 Modalities of treatment OperativeBone graftingIntramedullary nailMicrovascular fibular graftExternal fixatorCombinationAmputationNon operativeSplintsElectrical stimulation, low intensity pulse ultrasoundBisphosphonatesUltrasoundOkada 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 tissuesStable fixationBiologic bridging of the bone defectCorrection of deformitiesPrevention of refracture and other complications
7 Vascularized fibular graft ComplicationsNew-onset fractures/ non unionMalalignment and valgus deformityValgus deformity on the donor siteThe 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 refractureComplicationsValgus ankle deformity on the donor sideRecurrent nonunion at one end of the graft siteResidual limb-length discrepancyaccording 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 tissueCan exert compression at fracture site
10 Ilizarov device…Allows extension of both tibia and fibular to bridge defect and prevent ankle valgusCorrection of axial deformities, andAllow full support immediately after the intervention
11 Ilizarov device… External fixator can be cumbersome for small children There is high frequency of re-fractureCircular external fixation procedure takes a long timePin tract infectionIf 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 careFollowing successful treatment, limb should be splinted till skeletal maturity
13 Combine pharmacological and surgical Surgical resection of harmatomatous tissuesApplication of periosteal graft, cancellous bone graft and bone morphogenic proteinStabilization with circular frame and intramedullary nailSystemic bisphosphonate
14 AmputationFor 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 remodelThe deformities of the proximal tibia can be corrected with osteotomy if the morphology of the tibia is normal with external fixatorThe 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 beginningProgressive shortening of the leg occurs as long as the pseudarthrosis remains ununited and also associated with repeated unsuccessful operationsResidual limb length discrepancy following successful union is a major problemGrowth 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 PrognosisSeverity of CPTAge at surgeryTreatment
22 Result of treatment Author No of cases Treatment % union Refracture rate (%)Boyd & Sage 195891Bone grafting56Joseph & Mathew 200014Im rod +double onlay tibial graft8621Johnson 200223Im rod + bone graft87Ohunushi 200526Ilizarov10025Vascularized fibular887CombinationPaley et al 199215943517Ilizarov + im nail29
23 Conclusion CPT remain a challenge to both patient and his surgeons Just as the search of its aetiology continued, so also its effective treatmentCombination treatment with ilizarov frame seems to be the most effective form of treatment