Approach to Pathologic Fractures in Children

Amy K. Williams; Alexandre Arkader

Disclosures

Curr Orthop Pract. 2013;24(3):260-266. 

In This Article

Fibrous Dysplasia

Fibrous dysplasia is a neoplastic or developmental condition in which the normal bone and marrow are replaced by fibrous-osseous tissue that can result in decreased bone strength and increased risk of pathologic fracture. There can be an association with endocrinopathies and café-au-lait spots, known as McCune-Albright syndrome. Specific endocrinopathies that are associated include hyperparathyroidism, hyperthyroidism, hyperprolactinemia, excess human growth hormone, and hypercortisolism.[37,38]

These lesions are often asymptomatic; however, depending on the size and extent of the lesion, fibrous dysplasia can present with deformity or fracture. Fractures most commonly occur in the proximal femur, followed by the tibia, ribs, and bones of the face. The disease can be monostotic or polyostotic. The fractures associated with fibrous dysplasia usually are incomplete or minimally displaced with a peak between the ages of 6 and 10 years.[39] Chronic stress fracture leading to angular deformity is commonly seen with proximal femoral lesions, particularly in polyostotic disease.

Pathologic fractures associated with monostotic disease often can be treated conservatively. Fractures can be treated with a cast or brace, particularly in young children.[40] In the polyostotic form, internal fixation with a load sharing device often is required because the bone quality is poor and there is high risk of secondary angular deformity. The preferred method of fixation is intramedullary devices to protect the entire bone. It does not seem that treating the lesion directly (i.e. curettage and grafting) is advantageous because there is a tendency for the grafted bone to be transformed into fibrous dysplasia.[40–43]

Given the metabolic abnormalities that can be associated with polyostotic disease, pamidronate has been used to decrease pain, improve bone quality, and decrease the incidence of fractures.[37]

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