Abstract
Introduction: Fibrous dysplasia (FD) is an uncommon bone disease caused by genetic mutations. It may be monostotic or polyostotic. It can be isolated or a part of a syndromic pathology. The purpose of this study is to describe the diagnostic, therapeutic and evolutionary features of craniofacial FD.
Case series: The average age was 20.8 years with a 3/1 sex-ratio. Only one patient had occipital osteoma as medical history. One patient had a frontal cranial deformity and a hearing impairment and the second had an exophthalmia. Two patients were completely asymptomatic. The ENT examination noted a deviated obstructive nasal septum in one case and bilateral painless masses involving the two external auditory canals in the other case. The extra-ENT examination found a right exophthalmia in one case and multiple osteomas in one case. All our patients had a CT scan and MRI examinations. Lesions’ localizations were diverse: sphenoidal sinus, left ethmoidal sinus, right infratemporal and right orbit roof. FD was multifocal in one case (frontal sinus, orbit, spheno-ethmoidal and temporomandibular articulation). Two patients had an anti-TNF injection and a bisphosphonate therapy was planned in collaboration with a rheumatologist. One patient had a surgical treatment.
Conclusion: Craniofacial FD is a rare bone disease causing functional and sensory anomalies. It usually requires multidisciplinary management.
Key-Words: Craniofacial disease, Fibrous dysplasia, Diagnostic, Bisphosphonates, Surgery
