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Congenital Pseudarthrosis of Tibia


- See:
        - Anterolateral Bowing
        - Neurofibromatosis

- Discussion:
    - is rare, occurring equally in girls and boys;
    - disease is almost always unilateral;
    - acute frxs occur during first 2 yrs of life, usually shortly after birth.
    - associated w/ anterolateral bowing and neurofibromatosis;
    - neurofibromatosis is present in approx 40-50% of pts w/ pseudarthrosis, while about 10% of pts w/ neurofibromatosis will have
           pseudarthrosis of tibia;
           - dysplastic type is characterized by narrowing, sclerosis, and obliteration of the medullary canal.
           - cystic type shows no narrowing but instead has cyst-like areas which resembles fibrous dysplasia microscopically;
                  - in this type, leg appears normal early in course, w/ frx and pseudarthrosis occurring after 5 years of age;


- Non Operative Treatment:
    - patellar tendon bearing - total contact orthosis, which is used to try to prevent frxs or to control developing ones;


- Surgical Treatment Options:
    - once frx ensues, treatment includes open reduction, internal fixation, & bone grafting w/ autogenous graft with or w/o electrical stimulation;
    - intramedullary rodding + bone grafting: (see osteogenic proteins)
           - has mixed results;
           - references:
               - Extending intramedullary rods in congenital pseudarthrosis of the tibia.
               - Intramedullary fixation for congential pseuarthrosis of the tibia.  
               - Treatment of Congenital Pseudarthrosis of the Tibia with Recombinant Human BMP-7 (rhBMP-7). A Report of 5 Cases.
    - limb lengthening:
    - vascularized fibular transfer:
         - may still leave the involved leg w/ potential for frx, angulation, & growth disturbance;
         - references:
               - Congenital pseudarthrosis of the tibia. Long-term followup of 29 cases treated by microvascular bone transfer.
               - Vascularized fibular grafts in the treatment of congenital pseudarthrosis of the tibia.
    - syme's amputation:
         - indicated for congential pseudoarthrosis with limb length inequality greater than 5 cm and or severe foot deformity;
         - presence of pseudoarthrosis by itself is not an indication for amputation;
         - note that amputation thru pseudoarthrosis may leave scars over amputation stumps, and over growth phenomena may end up requiring
                  additional revision amputations;
         - in some cases, spontaneous union of pseudarthrosis will occur (due to total contact of prosthesis and beneficial effects of wt bearing;
         - references:
               - The Syme amputation in patients with congenital pseudarthrosis of the tibia.
               - Spontaneous union of a congenital pseudoarthrosis of the tibia after syme amputation.  
               - Amputation for congential pseudarthrosis of the tibia.  Indications and techniques.  

                     



Congenital pseudarthrosis of the leg. Late results.

Late-onset pseudarthrosis of the dysplastic tibia.

Surgical treatment of congenital pseudarthrosis of the tibia.      

Pathology and natural history of congenital pseudarthrosis of the tibia.      

Congenital pseudarthrosis of the tibia. A long-term follow-up study.

Congenital Pseudarthrosis of the Tibia. Results of Technical Variations in the Charnley-Williams Procedure

Residual challenges after healing of congenital pseudarthrosis in the tibia.

Management of congenital pseudarthrosis of the tibia in children under 3 years of age: effect of early surgery on union of the pseudarthrosis and growth of the limb.