Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
| Autor: | Sira Stanslaus Owibingire, Elison N. M. Simon, Jeremiah Robert Moshy, Karpal Singh Sohal, Theodora Miti Kabali |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
musculoskeletal diseases
Male Pediatrics medicine.medical_specialty Delayed Diagnosis Craniofacial fibrous dysplasia Puberty Precocious Case Report Fibrous Dysplasia Polyostotic Tanzania McCune–Albright syndrome Facial Bones 03 medical and health sciences 0302 clinical medicine Medicine Precocious puberty Humans 030212 general & internal medicine Craniofacial Polyostotic fibrous dysplasia Child General Dentistry business.industry Gradual onset Fibrous dysplasia McCune-Albright syndrome Skull 030206 dentistry medicine.disease lcsh:RK1-715 body regions Radiography Treatment Outcome lcsh:Dentistry Oral and maxillofacial surgery Severe morbidity Female Morbidity business Tomography X-Ray Computed |
| Zdroj: | BMC Oral Health BMC Oral Health, Vol 19, Iss 1, Pp 1-7 (2019) |
| ISSN: | 1472-6831 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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