Otalgia revealing McCune-Albright syndrome: A case report.

Autor: Sbai AA; Department of Ear Nose and Throat, Mohammed VI University Hospital, Medical School, Mohammed the First University, Oujda, Morocco., Es-Salhi F; Department of Ear Nose and Throat, Mohammed VI University Hospital, Medical School, Mohammed the First University, Oujda, Morocco., Tsen AA; Laboratory of epidemiology, Clinical Research and Public Health, Faculty of Medicine and Pharmacy of Oujda, Mohammed the First University, Oujda, Morocco.; Depatement of Cervicofacial Surgery, Mohammed VI University Hospital, Medical School, Mohammed the First University, Oujda, Morocco., Fahd Elayoubi; Department of Ear Nose and Throat, Mohammed VI University Hospital, Medical School, Mohammed the First University, Oujda, Morocco.; Laboratory of epidemiology, Clinical Research and Public Health, Faculty of Medicine and Pharmacy of Oujda, Mohammed the First University, Oujda, Morocco.
Jazyk: angličtina
Zdroj: Annals of medicine and surgery (2012) [Ann Med Surg (Lond)] 2022 Sep 20; Vol. 82, pp. 104706. Date of Electronic Publication: 2022 Sep 20 (Print Publication: 2022).
DOI: 10.1016/j.amsu.2022.104706
Abstrakt: 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.
Competing Interests: All authors disclose any conflicts of interest.
(© 2022 The Authors.)
Databáze: MEDLINE