Fibrous dysplasia: rare manifestation in the temporal bone
Introduction: Fibrous dysplasia is a benign disorder, in which normal bone is replaced by fibrosis and immature bone trabeculae, showing a similar distribution between the genders, and being more prevalent in the earlier decades of life. Fibrous dysplasia of the temporal bone is a rare condition, an...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-03-01
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| Series: | Brazilian Journal of Otorhinolaryngology |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1808869420300999 |
| _version_ | 1818009683734036480 |
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| author | Thaís de Carvalho Pontes-Madruga Halana Valéria Carneiro Filgueiras Daniel Marcus San da Silva Leonardo Sales da Silva José Ricardo Gurgel Testa |
| author_facet | Thaís de Carvalho Pontes-Madruga Halana Valéria Carneiro Filgueiras Daniel Marcus San da Silva Leonardo Sales da Silva José Ricardo Gurgel Testa |
| author_sort | Thaís de Carvalho Pontes-Madruga |
| collection | DOAJ |
| description | Introduction: Fibrous dysplasia is a benign disorder, in which normal bone is replaced by fibrosis and immature bone trabeculae, showing a similar distribution between the genders, and being more prevalent in the earlier decades of life. Fibrous dysplasia of the temporal bone is a rare condition, and there is no consensus as to whether it is more common in monostotic or polyostotic forms. External auditory meatus stenosis and conductive dysacusis are the most common manifestations, with cholesteatoma being a common complication, whereas the involvement of the otic capsule is an unusual one. Surgical treatment is indicated to control pain or dysacusis, otorrhea, cholesteatoma, and deformity. Objectives: To describe the clinical experience of a tertiary referral hospital with cases of fibrous dysplasia of the temporal bone. Methods: Sampling of patients diagnosed with fibrous dysplasia of the temporal bone, confirmed by tomography, treated at the pediatric otology and otorhinolaryngology outpatient clinics, between 2015 and 2018. The assessed variables were age, gender, laterality, external auditory meatus stenosis, deformity, hearing loss, presence of secondary cholesteatoma of the external auditory meatus, lesion extension and management. Results: Five patients were included, four females and one male, with age ranging from 13 to 34 years. Three had the polyostotic form and two the monostotic form of fibrous dysplasia of the temporal bone. Four patients had local deformity and external auditory meatus stenosis, two of which progressed to cholesteatoma. All patients showed some degree of hearing impairment. All had preserved otic capsule at the tomography. Two patients are currently undergoing clinical observation; two were submitted to tympanomastoidectomy due to secondary cholesteatoma; one was submitted to lesion resection, aiming to control the dysacusis progression. Conclusion: Five cases of fibrous dysplasia of the temporal bone were described, a rare disorder of which the otologist should be aware. |
| first_indexed | 2024-04-14T05:46:13Z |
| format | Article |
| id | doaj.art-c5a4816294704e3e9b73747ced7e6edf |
| institution | Directory Open Access Journal |
| issn | 1808-8694 |
| language | English |
| last_indexed | 2024-04-14T05:46:13Z |
| publishDate | 2022-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Brazilian Journal of Otorhinolaryngology |
| spelling | doaj.art-c5a4816294704e3e9b73747ced7e6edf2022-12-22T02:09:17ZengElsevierBrazilian Journal of Otorhinolaryngology1808-86942022-03-01882235242Fibrous dysplasia: rare manifestation in the temporal boneThaís de Carvalho Pontes-Madruga0Halana Valéria Carneiro Filgueiras1Daniel Marcus San da Silva2Leonardo Sales da Silva3José Ricardo Gurgel Testa4Corresponding author.; Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina, São Paulo, SP, BrazilUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina, São Paulo, SP, BrazilUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina, São Paulo, SP, BrazilUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina, São Paulo, SP, BrazilUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina, São Paulo, SP, BrazilIntroduction: Fibrous dysplasia is a benign disorder, in which normal bone is replaced by fibrosis and immature bone trabeculae, showing a similar distribution between the genders, and being more prevalent in the earlier decades of life. Fibrous dysplasia of the temporal bone is a rare condition, and there is no consensus as to whether it is more common in monostotic or polyostotic forms. External auditory meatus stenosis and conductive dysacusis are the most common manifestations, with cholesteatoma being a common complication, whereas the involvement of the otic capsule is an unusual one. Surgical treatment is indicated to control pain or dysacusis, otorrhea, cholesteatoma, and deformity. Objectives: To describe the clinical experience of a tertiary referral hospital with cases of fibrous dysplasia of the temporal bone. Methods: Sampling of patients diagnosed with fibrous dysplasia of the temporal bone, confirmed by tomography, treated at the pediatric otology and otorhinolaryngology outpatient clinics, between 2015 and 2018. The assessed variables were age, gender, laterality, external auditory meatus stenosis, deformity, hearing loss, presence of secondary cholesteatoma of the external auditory meatus, lesion extension and management. Results: Five patients were included, four females and one male, with age ranging from 13 to 34 years. Three had the polyostotic form and two the monostotic form of fibrous dysplasia of the temporal bone. Four patients had local deformity and external auditory meatus stenosis, two of which progressed to cholesteatoma. All patients showed some degree of hearing impairment. All had preserved otic capsule at the tomography. Two patients are currently undergoing clinical observation; two were submitted to tympanomastoidectomy due to secondary cholesteatoma; one was submitted to lesion resection, aiming to control the dysacusis progression. Conclusion: Five cases of fibrous dysplasia of the temporal bone were described, a rare disorder of which the otologist should be aware.http://www.sciencedirect.com/science/article/pii/S1808869420300999Temporal boneCraniofacial dysostosisCholesteatoma |
| spellingShingle | Thaís de Carvalho Pontes-Madruga Halana Valéria Carneiro Filgueiras Daniel Marcus San da Silva Leonardo Sales da Silva José Ricardo Gurgel Testa Fibrous dysplasia: rare manifestation in the temporal bone Brazilian Journal of Otorhinolaryngology Temporal bone Craniofacial dysostosis Cholesteatoma |
| title | Fibrous dysplasia: rare manifestation in the temporal bone |
| title_full | Fibrous dysplasia: rare manifestation in the temporal bone |
| title_fullStr | Fibrous dysplasia: rare manifestation in the temporal bone |
| title_full_unstemmed | Fibrous dysplasia: rare manifestation in the temporal bone |
| title_short | Fibrous dysplasia: rare manifestation in the temporal bone |
| title_sort | fibrous dysplasia rare manifestation in the temporal bone |
| topic | Temporal bone Craniofacial dysostosis Cholesteatoma |
| url | http://www.sciencedirect.com/science/article/pii/S1808869420300999 |
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