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Head Neck ; 46(1): E1-E5, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37823398

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.


Subject(s)
Craniofacial Fibrous Dysplasia , Fibrous Dysplasia, Polyostotic , Humans , 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 , RANK Ligand/antagonists & inhibitors
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