Cargando…

Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports

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 throu...

Descripción completa

Detalles Bibliográficos
Autores principales: Kabali, Theodora Miti, Moshy, Jeremiah Robert, Owibingire, Sira Stanslaus, Sohal, Karpal Singh, Simon, Elison N. M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6686505/
https://www.ncbi.nlm.nih.gov/pubmed/31395055
http://dx.doi.org/10.1186/s12903-019-0872-8
Descripción
Sumario: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.