Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
Abstract Background McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painle...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2019-08-01
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| Series: | BMC Oral Health |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12903-019-0872-8 |
| _version_ | 1818870577548492800 |
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| author | Theodora Miti Kabali Jeremiah Robert Moshy Sira Stanslaus Owibingire Karpal Singh Sohal Elison N. M. Simon |
| author_facet | Theodora Miti Kabali Jeremiah Robert Moshy Sira Stanslaus Owibingire Karpal Singh Sohal Elison N. M. Simon |
| author_sort | Theodora Miti Kabali |
| collection | DOAJ |
| description | Abstract Background McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. Case presentation This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. Conclusion Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment. |
| first_indexed | 2024-12-19T12:09:14Z |
| format | Article |
| id | doaj.art-e952b39beaa04e1b848289c252d426de |
| institution | Directory Open Access Journal |
| issn | 1472-6831 |
| language | English |
| last_indexed | 2024-12-19T12:09:14Z |
| publishDate | 2019-08-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Oral Health |
| spelling | doaj.art-e952b39beaa04e1b848289c252d426de2022-12-21T20:22:16ZengBMCBMC Oral Health1472-68312019-08-011911710.1186/s12903-019-0872-8Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reportsTheodora Miti Kabali0Jeremiah Robert Moshy1Sira Stanslaus Owibingire2Karpal Singh Sohal3Elison N. M. Simon4Dental department, Litete District HospitalDepartment of oral and maxillofacial surgery, Muhimbili University of Health and Allied SciencesDepartment of oral and maxillofacial surgery, Muhimbili University of Health and Allied SciencesDepartment of oral and maxillofacial surgery, Muhimbili University of Health and Allied SciencesDepartment of oral and maxillofacial surgery, Muhimbili University of Health and Allied SciencesAbstract Background McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. Case presentation This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. Conclusion Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment.http://link.springer.com/article/10.1186/s12903-019-0872-8Craniofacial fibrous dysplasiaMcCune-Albright syndromeMorbidityCase report |
| spellingShingle | Theodora Miti Kabali Jeremiah Robert Moshy Sira Stanslaus Owibingire Karpal Singh Sohal Elison N. M. Simon Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports BMC Oral Health Craniofacial fibrous dysplasia McCune-Albright syndrome Morbidity Case report |
| title | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
| title_full | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
| title_fullStr | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
| title_full_unstemmed | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
| title_short | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
| title_sort | craniofacial fibrous dysplasia associated with mccune albright syndrome challenges in diagnosis and treatment case reports |
| topic | Craniofacial fibrous dysplasia McCune-Albright syndrome Morbidity Case report |
| url | http://link.springer.com/article/10.1186/s12903-019-0872-8 |
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