What is fibrous dysplasia?

Fibrous dysplasia is an abnormal condition of bone which can affect any of the bones in the body.  When this involves the skull, this can solely involve the skull bone or be associated with other bones in the body.  It is characterised by replacement of the normal bony architecture by abnormal bone which invariably leads to thickening of the bone.  It is not a tumour but due to thickening of the bone, it can produce certain problems, particularly when it involves the skull.  These vary from affecting the cosmetic appearance of the contours of the skull, through to narrowing of the bony channels through the skull which convey nerves such as the optic nerve for vision. 

How is it diagnosed?

Fibrous dysplasia can be diagnosed as an incidental finding when it is very minor.  If this is the case then almost certainly we will advise you that nothing further is required beyond simple monitoring.  In those more extensive cases, this monitoring can involve surveillance of your vision to ensure that this is not affected. 

Fibrous dysplasia tends to be more active in people in their teens and twenties and will largely stop progressing by the age of 30. 

How is it treated?

The vast majority of people with fibrous dysplasia of the skull can be managed without any particular surgical intervention.  The key part of treatment is often simply monitoring the condition to ensure that it does not produce symptoms or cause threat to neurological function due to progression.
It is very rare any surgical treatment needs to be considered for fibrous dysplasia. Overall, the sort of problems that can merit consideration of surgery in fibrous dysplasia fall into 2 broad categories.

  1. Cosmetic (if the deformity of the skull causes marked facial asymmetry).  There are various ways to restore the symmetry of the face with surgery.  This can include re-contouring of the skull through specialised techniques which your surgeons have experience of.  Alternatively, parts of the skull can be resected and replaced with titanium or other implants to restore a normal contour.  Clearly, the need for such significant surgery for a cosmetic indication needs careful consideration but your surgeons will very gladly go through this carefully with you.

  2. Threat to neurological function.  Whilst this is rare, it can occur if the fibrous dysplasia involves the skull base and in particular may cause narrowing of the apertures in the skull base through which various nerves run.  Professor King and Mr Rutherford have experience of such cases including loss of vision or impairment of the facial sensory nerve causing numbness and pain.  In these instances, surgery can be done to decompress the respective neurological structures. 

Other than surgery, treatment for fibrous dysplasia is largely unproven.  It is likely your management will also involve an endocrine expert and they can use medical therapy particularly if the bone changes in fibrous dysplasia are causing localised bony pain.  However, this sort of treatment is not routinely prescribed in fibrous dysplasia.