First two cases of craniomaxillofacial fibrous dysplasia from Nepal – case series

Abstract

Neha Mishra,1 Sourav Kumar Rout2 1Department of Oral and Maxillofacial Pathology and Microbiology, Chitwan Medical College and Teaching Hospital, Bharatpur-10, Chitwan, Nepal; 2Department of Oral and Maxillofacial Surgery, Chitwan Medical College and Teaching Hospital, Bharatpur-10, Chitwan, Nepal Abstract: Fibrous dysplasia is a benign fibro-osseous disease of the bone, which is most commonly associated with congenital mutations in cAMP regulating protein Gsα coded by GNAS-1 gene. Often it is seen involving the craniofacial skeleton and can range from an asymptomatic monostotic form to polyostotic variant involving almost all the bones of the skull, thereby leading to functional and esthetic problems. This requires a continuous monitoring of the involved region throughout the life of the patient, even after the surgical interventions. We are presenting two cases of craniofacial form of fibrous dysplasia. One case shows monostotic form, while the other case shows features of polyostotic form of disease. To the best of our knowledge, these are the first two cases of craniomaxillofacial fibrous dysplasia from Nepal, which will be reported and published. Keywords: monostotic, polyostotic, Jaffe–Lichtenstein syndrome, McCune–Albright syndrome, fibro-osseous

Keywords

• Monostotic
• Polyostotic
• Jaffe-Lichenstein syndrome
• McCune Albright syndrome
• Fibro-osseous