[Polyostotic fibrous dysplasia. A clinical case report]

The authors present a severe case of polyostotic fibrous dysplasia in which there was considerable involvement of cranial bone and facial skeleton. Numerous lesions were present at the level of the long bones of limbs. Endocrine dysfunction was also present in the form of a hypophyseal adenoma secreting prolactin and ACTH. The concomitance of acromegaly or gigantism and/or hyperprolactinemia and polyostotic fibrous dysplasia has only been reported to date in a few cases in literature. The authors describe the appearance of the subject, correlating clinical photographs with X-rays. They report the clinical excursus of the patient characterised by the gradual increase in deformities which seriously jeopardized the patient's relational life, in particular the appearance of a bulk on the forehead and checks and the deformation of the symphyseal portion of the mandible with presence of interdental diastemata. The patient also complained diplopia, difficulty in chewing owing to the mobilisation of teeth, and increasing bone pain probably due to nerve compression by exuberant bone. It was not possible to perform corrective surgery owing to the patient's overall poor health conditions. In fact, dilatative cardiomyopathy which continued to worsen in spite of numerous forms of medical treatment resulted in the patient's death owing to cardiac decompensation. Even the attempt to treat the patient's primary endocrine dysfunction using bromocryptine and subsequently octreotide failed to produce positive results owing to the onset of collateral effects which led to the early suspension of treatment.