Cariofacila Dysplaisa
Keywords:
fibrous dysplasia, neurosurgery, monostotic, polyostoticAbstract
Introduction: Craniofacial dysplasia is a benign condition in which bone is replaced by structurally defective fibro-osseous tissue. It is caused by a postzygotic activating mutation of the gene encoding the α subunit of the stimulatory G protein. Imaging studies suggest the diagnosis, which must be confirmed through pathology. There is currently no specific drug treatment available.
Objective: To report a clinical case of craniofacial monostotic fibrosis.
Clinical case report: We report the case of a 50-year-old female patient who was treated in the Neurosurgery service at the Neurology and Neurosurgery Institute in October 2022. In 2019, she began to present inflammation of the lower right eyelid, she was first seen by an ophthalmology service where she was treated for a probable allergic condition. Six months ago she noted volume increase in her right eyeball plus a decrease in ipsilateral visual acuity. In the simple skull tomography study, a hyperdense image was observed that could be correlated to a fibrous lesion. The surgical procedure was performed and the anatomopathological study with hematoxycillin and eosin staining revealed curved, disorganized bone trabeculae with background of fibrous tissue.
Conclusions: Craniofacial monostotic fibrous dysplasia is a rare disease with slow progression and low incidence, which requires efficient multidisciplinary diagnostic analysis for its management. Early diagnosis and optimal adjustment of treatment are important to obtain favorable surgical and clinical results.
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References
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