What is Fibrous Dysplasia?

Fibrous Dysplasia, a big word and even bigger disease.

                                                                                                      I'm writing this advice because i thought it would be handy for mum's out there to know and also to have on hand, as it is a disease that is easy mis-diagnosed. Also because my dear  life-time friend  ( Annie) is going through this draumatic disease for the second time, she is booked for surgery (Brain) in July. Most cases  only go through surgery once though, depending where they have the disease, Annie is unlucky as her's  is in the cavity between her brain and forehead. 34 years ago when she was 16 she went through major surgery to have her forehead removed and fibrous tissue, her forehesd was replaced with a titanium plate which over the years has served her well. But now this disease has come back with vengeance over the past couple of years.  She  has suffered  with bad headaches, her eye sight is in jeopardy, her forehead is swollen and red, she has trouble breathing as the fibrous tissue has affected her sinsus.

"What is Fibrous Dysplasia?"

                                                        Fibrous dysplasia is a condition of the skeleton (bones). It is a BIRTH DEFECT that is non-cancerous disease. It is not hereditary so your child did not get it from you nor will he/she pass it along th his/her children.

"How do I recognize this condition in my child?"

                                                         Fibrous dysplasia is usually detected in early childhood as a result of swelling of the jaw. Also in some cases it may cause the teeth to seperate.

"How does the Disease progress?"

                                                      Fibrous dysplasia gets progressively worse from birth until the bones finish growing. As it progresses, normal bone is replaced by various amounts of structurally weak fibrous and osseous (bone-like) tissue. In normal bone formation, woven bone appears first and later matures into lamellar bone. In fibrous dysplasia, bones do not mature and development  and  stops in the woven bone stage. Fibrous dysplasia causes misshappen bones. It can occur in the bones in front of the head and/or sphenoid bones  that are situated at the base of the skull, if this happens, it can eventually lead to deformation of facial features and effect the shape of the skull.

"How many types of Fibrous dysplasia are there?"

                                                     There are three types of fibrous dysplasia;

                                                                                                                                    Monostotic disease, is the most common type of fibrous dysplasia, occuring in 70% of cases. Monostotic simply means involving one bone. It most often occurs on the long bones such as the femur (thigh bone), ribs and skull.

                                                                                                                                   Polyostotic disease, affects 30% of patients. Polyostotic means occuring in more than one bone. The head and neck are involved in half of these patients.

                                                                                                                                 The third is "McCune-Albright syndrome." It only occurs in 3% of cases. It is characterized by polyostotic fibrous dysplasia occuring in more than one bone; skin pigmentation; and in females; early puberty.

"How often does fibrous dysplasia affect the face & head?"

                                                                                                             Skull involvement occurs in 27% of momstotic and up to 50% of polyostic patients. Fibrous dysplasia involving the face and the skull is called "Leontiasis ossea". Without treatment, one or more bones progressively increase in size, and move into the cavities of the eye, mouth, and/or the nose and it's sinuses. Also, abnormal protrusion of the eyeball may develop and eventually cause complete loss of sight because it presses on the optic nerve. In addition there may be interfernce of the nasal passage and with eating.

"What are the effects of fibrous dysplasia of the skull base?"

                                                                                                               When fibrous dysplasia of the frontal (forehead bone) and/or sphenoid (bone at the base of the skull) bones progresses, these bones become thick and dense. This increase in size eventually causes the facial features and skull to become misshapen. In these cases more than one bone is usually involved. It can also result in cranial nerve problems. If the temporal bone is affected, the patient may suffer as much as 80% hearing loss when the inner ear canal narrows. It may also cause facial nerve paralysis or dizziness. However, any of our 12 cranial nerves can be involved with fibrous dysplasia. The more common results could include cranial nerve problems, and sight and hearing loss.

"Are there any other effects of fibrous dysplasia?"

                                                                                            It is estimated that patients with fibrous dysplasia are 400 times more likely than the general population to develop a malignant bone tumor.

"What is the treatment for fibrous dysplasia?"

                                                                                    Physicians decide on treatment options after assesing a patients symptoms. First the doctor observes the patient. Then he will consider conservative treatment such as surgically shaving or removing the fibrous tissue. In more severe cases the doctor may recommend complete removal of the bone.

Surgery is used to return the face to it's normal structure and/or to relieve effects when a cranial nerve is being pinched. In these cases the abnormal bone must be completely removed. It is best to wait until adolescence for surgery. However, if the progression of the disease affects nerve function, a decompressive procedure should be considered early in childhood to keep normal function.

"If surgery is recommended, how many will be neccessary?"

                                                                                                                   Sometimes the fibrous tissue can be completely removed successfully by a single procedure. However. most fibrous tissue can be managed through staged procedured with overall very favourable results and good long term prognosis.

I do hope this advice is helpful.