Within the syndrome there are bone fractures and deformity of the legs, arms and skull, different pigment patches on the skin, and early puberty with increased rate of growth. Polyostotic fibrous dysplasia is usually caused by mosaicism for a mutation in a gene called GNAS1.

Polyostotic Fibrous Dysplasia has different levels of severity. For example one child may be entirely healthy with no outward evidence of bone or endocrine problems, enter puberty at close to the normal age and have no unusual skin pimgentation. The complete opposite of that would be children who are diagnosed in early infancy with the obvious bone disease and obvious increased endocrine secretions from several glands.

Approximately 20-30% of fibrous dysplasias are polyostotic and two thirds of patients are polyostotic before the age of ten.

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FIBROUS DYSPLASIA: 64 CASES
From Journal of Bone and Joint Surgery, 1/1/04 by Karray, N

Purpose: Fibrous dysplasia is a rare condition accounting for about 1% of benign bone tumours. Both mono-and polyostotic dysplasia is described. Treatment of small tumours is not particularly difficult, but extensive or complicated tumours are another problem. The purpose of this work was to present the results of surgical treatment of a consecutive series of 64 patients. Particular locus was placed on complications.

Material and methods: This retrospective series included 64 patients with fibrous dysplasia. Mean age was 32 years. There were 37 women and 27 men. Monostotic dysplasia was noted in 58 cases. The femur was the most frequent localisation (n=19). Seven patients had a fracture. Histological proof was obtained in 61 patients. In three patients, fibrous dysplasia was associated with another tumour (aneurysmal cyst or adamantinoma). Several therapeutic strategies were proposed: abstention for three patients, biopsy alone for 13, resection for six, curettage for 34. The curettage was filled in 23 cases with osteosynthesis in 11. Eight patients were given other surgical treatments (prosthesis, osteosynthesis, amputation).

Results: We had five postoperative complications. At mean follow-up of 45 months. 14% of the patients still suffered from generally mild to moderate pain. Radiologically, there were four recurrences. 12 unchanged, 18 regressions, and 19 remissions (generally after resection or curettage with filling). Long-term follow-up revealed malignant transformation in two patients.

Discussion: Lesions treated by biopsy alone cured or regressed in 30% of the cases while curettage with tilling led to cure or regression in 70% of the cases. We did not include patients treated by bisphosphonates in this series because of insufficient follow-up. Nevertheless, this treatment appears to have a promising effect on pain.

Conclusion: Results of treatment for fibrous dysplasia are not as good as might be suspected for this benign disease. The role of surgery appears to be on the decline, to be replaced by bisphosphonates whose long-term efficacy must be evaluated.

N. Karray, A. Babinet, B. Tomeno, P. Anract

Service de Chirurgie Orthopedique B. Hopital

Cochin, 27, rue du Faubourg-Saint-Jacques, 75014

Paris, France

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