Fibrous dysplasia of bone is a benign, a congenital, and a non-communicable disease caused by the GNAS mutation encoding the Gsα protein. Its prevalence is less than 1/2,000. It affects both sexes equally and is characterized by a benign proliferation of fibrous tissue in the bone marrow. Bone lesions are usually unique or multiple and can affect all osseous structures with a high frequency of maxillofacial lesions.The CT scan showed a diffuse thickening of the cranial vault with an osteocondensation of "frosted glass appearance". The osseous frontal abnormalities were both condensing and lytic with multifocal cortical ruptures in the endocranium, periosteal reactions, and extradural intracranial extension of increased tissue density after injection of contrast medium. The mixed imaging features suggested a malignant transformation of the disease. The diploe was thickened. The considerable osseous frontal hypertrophy was responsible for a compression of the frontal cerebral parenchyma underscored by the deletion of the cortical furrows. The maxillary bone, mandible, temporal bones, facial sinuses, and skull base bones had lytic lesions with a blown appearance of the bones.

The significant bone hypertrophy had almost led to the total stenosis of the facial sinuses. The ethmoid cells were normal with no tooth extraction. Three dimensional reconstructions have been useful in assessing craniofacial dysmorphia. The cervical spine and the osseous structures of both shoulders also showed similar diffuse osteolysis features. This polyostotic fibrous dysplasia associated with skin patches and endocrinopathy was diagnosed as McCune-Albright syndrome within this patient. Unfortunately, she died of cardiac arrest in the following days after the performance of imaging. Her sudden death hampers us from performing a bone biopsy to confirm the malignant transformation of bone dysplasia.

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Mishita
Jornal co-ordinator
Journal of Bone Research and Reports