Congenital Psueudoarthrosis- it is a rare pathology with various clinical presentation ranging from simple anterolateral tibial angulation to complete nonunion with extensive bone defects. More than 50% cases are associated with neurofibromatosis, ten percent with fibrous dysplasia and remaining as idiopathic. It is the most challenging and dreaded condition. CPT has tendency to refracture until skeletal maturity fracture can occur in even in adults. Refracture incidence is highest in younger age. Neurofibromatosis & younger age at treatment is considered negative prognostic factor.
Pathology: pathology is still poorly understood. Many theory has been given for this problem but most suitable one are
1 Diseased periosteum
- Increases osteoclastic activity
Investigation- xray and assessment of multilevel neurofibroma should be rule out by detail history and examination.
Treatment= Drohrpaley approach
X-union protocol –
Mechanical modalities- correction of angular deformity, stability of fixation using external and internal fixation, increasing width at the cpt site by cross union of tibia and fibula, reinforce the bone strength with IM nail of tibia and fibula
Biological fixation- resection of the fibrous hamartoma & any hypo-vascular bone, improving the blood supply to the CPT site, autogenous bone and periosteal grafting of the CPT site.
Use of intravenous zolendronic acid, calcium and vit d 2 week prior to operation
Use of BMP at CPt site
All the patient limb will be protected by brace for years.
Inspite of all these measure chances of refracture cant be denied. But till now by this technique original author has achieve 100 % outcome with no refracture till date. Intramedullary rod has to be changed as child grows.