RANK-L inhibitor as a promising agent for refractory extensive craniofacial fibrous dysplasia: A case report.

Abstract

McCune-Albright syndrome is a rare disorder characterized by polyostotic fibrous dysplasia (FD), café-au-lait skin pigmentation, and endocrine dysfunction. Extensive FD in the craniofacial region can present significant challenges in terms of disease control and carries a high risk of permanent visual impairment. We present a case of medically and surgically resistant FD that required nine optic nerve decompressions. The condition was ultimately controlled with the use of the denosumab agent. The case highlights the importance and potential efficacy of denosumab in resistant FD management, particularly in cases involving sensitive organs.