RESUMO
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.
Assuntos
Displasia Fibrosa Craniofacial , Displasia Fibrosa Poliostótica , Humanos , Osso e Ossos , Displasia Fibrosa Craniofacial/tratamento farmacológico , Descompressão Cirúrgica , Denosumab , Displasia Fibrosa Poliostótica/diagnóstico por imagem , Displasia Fibrosa Poliostótica/tratamento farmacológico , Displasia Fibrosa Poliostótica/cirurgia , Ligante RANK/antagonistas & inibidoresRESUMO
Anomalies in the frontal lobe can interfere with visual function by compression of the optic chiasm and nerve. The gyrus rectus is located at the anterior cranial fossa floor superior to the intracranial optic nerves and chiasm. Compression of these structures by the gyrus rectus is often caused by neoplastic or dysplastic growth in the area. We report a rare case of a herniated gyrus rectus impinged on the optic chiasm and nerve without a clear pathological cause for the herniation.