Polyostotic fibrous dysplasia: report of a patient

Abstract

Polyostotic fibrous dysplasia is a benign fibro-osseous lesion in which the normal structure of the bone is replaced by fibrous tissue and by nonfunctional bony structures that show a trabecular aspect and have an unknown etiology. It is a very rare and infrequent disease on which there is little information in the literature. This situation has motivated us to report this case. It is hypothesized that there is a gene mutation encoding G alpha protein which is responsible for the condition. The surgical management of these cases is a challenge and it is also controversial. In this patient, it was reported an osteolytic lesion in both femoral necks that prevented proper ambulation and it was decided to conduct an inter-consultation with the Orthopedic Specialist for a better management. The patient underwent a hip computed tomography that confirmed the lesion and, subsequently, he underwent a biopsy that reported a polyostotic fibrous bone dysplasia. The dysplasia increases in size until general body growth ceases at the end of puberty.