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SICOT e-Newsletter

Issue No. 6 - March 2009

Case of the Month

A six-year-old girl was presented to the children's orthopaedic department with bone pain in projection to the diaphyseal region of the left tibia. Her parents reported two subsequent fractures of the left tibia one and two years before which were treated by casting (Figs. 1 and 2 below).

 Fig. 1     Fig. 2

The X-ray at time of presentation showed an osteolytic lesion with widening of the diaphysis, cortical thinning and residuals from the previous fractures (Fig. 3).

Fig. 3

Comment:

There are several differential diagnoses to be considered which could be the reason for the pathological fractures: enchondroma, aneurysmal bone cyst, adamantinoma and fibrous dysplasia.

What investigation would you recommend?

  • Physical examination
  • Special blood tests
  • MRI
  • CT-Scan
  • Scintigraphy
  • Bone biopsy

 

 

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Comment:

The suspected diagnosis of fibrous dysplasia makes physical examination and special blood tests necessary to detect birth marks (café-au-lait spots) and endocrine problems such as precocious puberty, hyperthyroidism and others (McCune-Albright syndrome). MRI provides information about the extend of the lesion, the intraosseous matrix and amount of bone loss (Fig. 4). A total body bone scan can rule out polyostotic disease. An open biopsy is reasonable to confirm the diagnosis (here: fibrous dysplasia) and to exclude malignancy.

 Fig. 4

The therapy of choice would be?

  • Conservative treatment
  • Medical therapy with bisphosphonates
  • Wide resection or intralesional curettage
  • Bone grafting (autogenic or allogenic)
  • Internal fixation with plate and screws
  • Intramedullary nailing 

 

 

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Comment:

Some studies indicate that oral bisphosphonates help relieve pain in children and adolescents with severe fibrous dysplasia but surgery remains the mainstay of treatment especially for long bone disease. Intramedullary nailing with UTNs or TENs according to the size of the affected tibia and the age of the patient is recommended. Autogenic cancellous bone graftings are frequently resorbed therefore allogenic material should be preferred (Fig. 5).

 Fig. 5

Fibrous dysplasia is an uncommon disease that can affect any bone in the body. It is caused by a gene defect and there is no cure for this disease. In the treatment of pathological fracture conservative therapy achieves fracture healing, but the pathological nature of tissue at the fracture site causes healing with defective mineralization and consequent risk of deformity and refracture. After confirmation of diagnosis curettage of the lesion, autogenic bone grafting and intramedullary fixation seems to be the treatment of choice.  

Author:
Dr Peter Raab 
Orthop.Klin. König-Ludwig-Haus
Brettreichstrasse 11
97074 WURZBURG
Germany
Fax: +49 (931) 803-1109 

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