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

Head Neck. 2024 Jan;46(1):E1-E5. doi: 10.1002/hed.27546. Epub 2023 Oct 12.

Abstract

Background: 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.

Methods: We present a case of medically and surgically resistant FD that required nine optic nerve decompressions.

Results: The condition was ultimately controlled with the use of the denosumab agent.

Conclusion: The case highlights the importance and potential efficacy of denosumab in resistant FD management, particularly in cases involving sensitive organs.

Keywords: McCune-Albright syndrome; craniofacial; denosumab; fibrous dysplasia; optic decompression.

Publication types

  • Case Reports
  • Research Support, N.I.H., Intramural

MeSH terms

  • Bone and Bones
  • Craniofacial Fibrous Dysplasia* / drug therapy
  • Decompression, Surgical
  • Denosumab
  • Fibrous Dysplasia, Polyostotic* / diagnostic imaging
  • Fibrous Dysplasia, Polyostotic* / drug therapy
  • Fibrous Dysplasia, Polyostotic* / surgery
  • Humans
  • RANK Ligand / antagonists & inhibitors

Substances

  • Denosumab
  • RANK Ligand