Surgical treatment of polyostotic craniomaxillofacial fibrous dysplasia in adult: a case report and review of the literature.

The lesions of fibrous dysplasia usually stabilize after adolescence, and the surgical treatment of adult patient remains the mainstay. However, the surgical treatment alone may be not enough for patient with polyostotic fibrous dysplasia. We present the case of a surgically treated 36-year-old man with a diagnosis of progressive polyostotic fibrous dysplasia in the craniomaxillofacial region. The patients presented the swelling symptoms originally in the parietal bone at the age of 8 years, and extended gradually to almost all of the craniomaxillofacial bones during the following 28 years without specific treatment for socio-economic reasons. The symptom impelled the patient visit our department in 2009 was the rapidly progressive swelling in the chin during the last 3 years, which severely impacted his speech and feeding. The radiographs showed the typical intramedullary located and ill-defined lesions. The patient was treated with segmental mandibulectomy and reconstruction with vascularized fibular myocutaneous flap; the deformities in other craniomaxillofacial bones were not treated simultaneously. The local recurrence was not present in the chin. The visual acuity of right eye severely deteriorated and the left mandibular ramus continued expanding gradually when the patient was followed up through telephone 5 years later. A combination of surgical and medical treatment may be considered for patients with polyostotic craniomaxillofacial fibrous dysplasia.