- 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;
- reference:
-
Extending intramedullary rods in congenital pseudarthrosis of the tibia.
- Intramedullary fixation for congential pseuarthrosis of the tibia. J Baker et al. JBJS Vol 74-A. 1992. p 161-168.
-
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;
- reference:
-
The Syme amputation in patients with congenital pseudarthrosis of the tibia.
- Spontaneous union of a congenital pseudoarthrosis of the tibia after syme amputation. JT Guille et al. CORR. No 351. p 180-185. 1998.
- Amputation for congential pseudarthrosis of the tibia. Indications and techniques. RE McCarthy. CORR Vol 166. p 58-61. 1982.
Congenital pseudarthrosis of the leg. Late results.
Late-onset pseudarthrosis of the dysplastic tibia.
Surgical treatment of congenital pseudarthrosis of the tibia. Umber JS, Moss SW, Colemann SS: Clin Orthop 1982;166:28.
Pathology and natural history of congenital pseudarthrosis of the tibia. HB Boyd. CORR Vol 166. 1982. p 5-13.
Congential pseudoarthrosis of the tibial. A long term follow up study. HH Murray and WW Lovell. CORR. Vol 166. 1982. p 14-20.
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.
