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.

Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, April 25, 2018 9:37 am