Poliostotic fibrous dysplasia with affectation of cervical rachis.

Abstract

The fibrous dysplasia is a benign although progressive dysfunction, in which a gene mutation originates the production of fibrous disorganized bony matrix. The bony tissue is replaced by bony tissue in expansion (amorph conjuntival tissue) that produces bony deforrnities in sorne patients, pain, pathological fractures or deambulation disorders. The diagnosis is important since ocasionally the first symptom is the fracture. We show up the   case of a 21 year -oid patient with pain clinic in high cervical region. The complementary tests (radiology, bone scintigrraphy and MRI) and anatomo-pathology confirmed the  diagnosis of polyostotic fibrous dysplasia with cranial (occipital esfenoides and right   frontal and temporal bone), iiiac, femoral, tibial and cervical (apophysis of C2) affectation. Our attitude was of canying out a narrow observation by means of periodical strict controls, advising to avoid hard activities or contact sports. To the five years the patient is free of symptomatology. Radiologicaily the injuries have been stabilized. The fibrous dysplasia can affect to a single bone (monostotic) or to several (polyostotic). In occasions it is associated to endocrine dysfunctions and skin pigmentations in McCune- Albright's syndrome (7). We confront a pathology that specifies an anatomo-pathologic diagnosis to be confirmeci, an extension diagnosis to detect asymptomatic focuses and whose treatment is symptomatic in most of the cases only using surgery in frank deformities or when the fracture risk is considerable, although the recurrence is frequent. The malignization is exceptional but possible that's why continuous observation is needed. The radiation therapy  is radically contraindicated (6).