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Otalgia revealing McCune-Albright syndrome: A case report

INTRODUCTION AND IMPORTANCE: McCune-Albright syndrome (MAS) is typically described by the asociation of cutaneous (coffee-at-milk spots), endocrine (endocrine hyperfunction most often precocious puberty), and fibrous dysplasia (FD). In 90% of cases, AD manifests itself as a disorder of the craniofac...

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Autores principales: Sbai, Achraf Amine, Es-Salhi, FatimZahra, Tsen, Adil Abdenbi, Fahd Elayoubi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9577849/
https://www.ncbi.nlm.nih.gov/pubmed/36268423
http://dx.doi.org/10.1016/j.amsu.2022.104706
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author Sbai, Achraf Amine
Es-Salhi, FatimZahra
Tsen, Adil Abdenbi
Fahd Elayoubi
author_facet Sbai, Achraf Amine
Es-Salhi, FatimZahra
Tsen, Adil Abdenbi
Fahd Elayoubi
author_sort Sbai, Achraf Amine
collection PubMed
description INTRODUCTION AND IMPORTANCE: McCune-Albright syndrome (MAS) is typically described by the asociation of cutaneous (coffee-at-milk spots), endocrine (endocrine hyperfunction most often precocious puberty), and fibrous dysplasia (FD). In 90% of cases, AD manifests itself as a disorder of the craniofacial skeleton, affecting the maxilla, mandible, and even the temporal bone. CASE PRESENTATION: We report the case of a 14-year-old girl who presented with complaints of left otalgia with the notion of recurrent otitis evolving for one year, she presents as antecedent an early puberty. At the examination, we found café-au-lait macules, and a slight left exophthalmos without visual acuity decrease. Otoscopic examination showed a narrowing of the external auditory canal (EAC). An audiogram showed conductive hearing loss in the left ear; the air-bone gap was 35 dB. A computed tomography (CT) scan was performed, showing a large "ground glass" appearance of the left temporal region. Given the presence of the cafe-au-lait spot, fibrous dysplasia of the temporal bone, and a history of early puberty, the diagnosis of McCune-Albright syndrome was made. CLINICAL DISCUSSION: Management of SMA is based on the needs of the individual patient and should be performed by a multidisciplinary team. Management of endocrinopathies is usually medical, with precocious puberty in girls most often treated with aromatase inhibitors. The objectives of the management of craniofacial DF are to correct the functional and aesthetic damage. CONCLUSION: McCune-Albright syndrome (MAS) is a rare disease. The involvement of the craniofacial region by FD during SAM is a complicated entity, in its effects and in its management
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spelling pubmed-95778492022-10-19 Otalgia revealing McCune-Albright syndrome: A case report Sbai, Achraf Amine Es-Salhi, FatimZahra Tsen, Adil Abdenbi Fahd Elayoubi Ann Med Surg (Lond) Case Report INTRODUCTION AND IMPORTANCE: McCune-Albright syndrome (MAS) is typically described by the asociation of cutaneous (coffee-at-milk spots), endocrine (endocrine hyperfunction most often precocious puberty), and fibrous dysplasia (FD). In 90% of cases, AD manifests itself as a disorder of the craniofacial skeleton, affecting the maxilla, mandible, and even the temporal bone. CASE PRESENTATION: We report the case of a 14-year-old girl who presented with complaints of left otalgia with the notion of recurrent otitis evolving for one year, she presents as antecedent an early puberty. At the examination, we found café-au-lait macules, and a slight left exophthalmos without visual acuity decrease. Otoscopic examination showed a narrowing of the external auditory canal (EAC). An audiogram showed conductive hearing loss in the left ear; the air-bone gap was 35 dB. A computed tomography (CT) scan was performed, showing a large "ground glass" appearance of the left temporal region. Given the presence of the cafe-au-lait spot, fibrous dysplasia of the temporal bone, and a history of early puberty, the diagnosis of McCune-Albright syndrome was made. CLINICAL DISCUSSION: Management of SMA is based on the needs of the individual patient and should be performed by a multidisciplinary team. Management of endocrinopathies is usually medical, with precocious puberty in girls most often treated with aromatase inhibitors. The objectives of the management of craniofacial DF are to correct the functional and aesthetic damage. CONCLUSION: McCune-Albright syndrome (MAS) is a rare disease. The involvement of the craniofacial region by FD during SAM is a complicated entity, in its effects and in its management Elsevier 2022-09-20 /pmc/articles/PMC9577849/ /pubmed/36268423 http://dx.doi.org/10.1016/j.amsu.2022.104706 Text en © 2022 The Authors https://creativecommons.org/licenses/by/4.0/This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Sbai, Achraf Amine
Es-Salhi, FatimZahra
Tsen, Adil Abdenbi
Fahd Elayoubi
Otalgia revealing McCune-Albright syndrome: A case report
title Otalgia revealing McCune-Albright syndrome: A case report
title_full Otalgia revealing McCune-Albright syndrome: A case report
title_fullStr Otalgia revealing McCune-Albright syndrome: A case report
title_full_unstemmed Otalgia revealing McCune-Albright syndrome: A case report
title_short Otalgia revealing McCune-Albright syndrome: A case report
title_sort otalgia revealing mccune-albright syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9577849/
https://www.ncbi.nlm.nih.gov/pubmed/36268423
http://dx.doi.org/10.1016/j.amsu.2022.104706
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