The treatment problems for Fibrous Dysplasia include:
- Angular deformities
- Fractures
- Pseudoarthrosis
- Limb length discrepancy
This can affect both the upper and lower limbs.
Some syndromes are associated with Fibrous Dysplasia: McCune-Albright Syndrome and Campanacci Disease. In McCune-Albright Syndrome, there are often associated endocrinopathies with polyostotic fibrous dysplasia. Limb length discrepancy is common. These require equalization of limb length through epiphysiodesis and/or limb lengthening. They also require correction of angular deformities through epiphysiodesis and/or osteotomy.
For more information, see Limb Lengthening Center
For Campanacci Disease, the involvement is of one or both tibias. This is one of the etiologic associations with Congenital Pseudarthrosis of the Tibia. Fibrous Dysplasia accounts for 10% of all cases of Congenital Pseudarthrosis of the Tibia.
The lesions of Fibrous Dysplasia are often recalcitrant to healing. They are best treated by intramedullary nailing to prevent bending and breaking. That, combined with bone grafting, is often the best way to treat the lesions. Limb lengthening through Fibrous Dysplasia bone can show delayed ossification. Furthermore, limb lengthening through affected bone, even when performed through the normal regions of the bone, may demonstrate new Fibrous Dysplasia lesions in the regenerate bone. This is very different from Ollier’s Disease, in which osteotomy through a lesion leads to healing of the lesion and excellent new bone formation.
Perhaps the most difficult deformity to correct in Fibrous Dysplasia is the Shepherd’s crook deformity of the hip. Previously, complete correction was unthinkable due to the severity of the deformity: the upper femur bends 180 degrees, such that the femoral head rests on the shaft of the femur. Dr. Paley’s SUPERhip procedure is perhaps the best way to treat this severe angular deformity. It allows acute correction of the hip deformity to a normal neck-shaft angle in a single surgery; while shortening of the femur is part of this procedure, no axis length is actually lost since the femur maintains the same hip-to-knee distance.
In very severe cases the strategy should be to correct all the deformities and then prophylactically rod each of the bones, including support of the femoral neck into the femoral head to prevent recurrent deformity.
Severe examples of the various types of monostatic and polystatic Fibrous Dysplasias are shown.
