The McCune Albright syndrome (MAS) is a rare, multi-system disease composed of the triad of polyostotic fibrous dysplasia of bone (PFDB), café-au-lait skin hyperpigmentation, and endocrine disorders. The diagnosis involves clinical, biochemical and imaging findings, with dentistry playing an important role in MAS, since many patients present DFPO in the craniofacial bones, including the maxilla and mandible, and in view of their dental needs, the correct management of these patients is not only an essential but important area to be investigated. This report presents a case of a patient with McCune Albright Syndrome, the behavior of the disease over a period of 10 years and how imaging exams such as scintigraphy and tomography were important for planning the dental treatment of this patient, since they are fundamental allies for identification and evaluation of the progression and/or stability of the disease. Craniofacial fibrous dysplasia, cone-beam computed tomography, scintigraphy, imaging diagnosis.
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http://dx.doi.org/10.4317/jced.60161 | DOI Listing |
Cureus
December 2024
Department of Neurology, 417 Army Share Fund Hospital, Athens, GRC.
Posterior reversible encephalopathy syndrome (PRES) is a clinical manifestation of various underlying causes, characterized by the combination of clinical and imaging findings associated with the posterior cerebral areas and relating to arterial hypertension and endothelial dysfunction. No association was made so far between PRES and McCune-Albright syndrome (MAS), a rare genetic disorder resulting in fibrous dysplasia. A 33-year-old female with MAS was presented to the emergency department of the 417 Army Share Fund Hospital in Athens (Greece) after seizure activity with two episodes of ocular upward deviation and transient facial palsy, each lasting a few minutes, followed by a postictal phase.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
General Surgery, MS Ramaiah Medical College, Bangalore, Karnataka, India.
A woman in her 50s presented with a 1-year history of left hip pain that increased over the past 2 weeks. The pain began following a low-energy fall. During the 1-year period, she had multiple lesions in the thigh and gluteal region, which were surgically excised.
View Article and Find Full Text PDFF S Rep
December 2024
Department of Obstetrics and Gynaecology, University of Ottawa, Ottawa, Ontario, Canada.
Objective: To report a patient with McCune-Albright syndrome (MAS) with bilateral ovarian involvement who had achieved a pregnancy through in vitro fertilization (IVF).
Design: Case report.
Setting: Academic fertility center.
Calcif Tissue Int
January 2025
Rheumatology department, Hôpital E. Herriot, Lyon, France.
This study explores FD/MAS patient's perceptions about their disease and its impact on their quality of life. We have evaluated quality of life (QoL) in French Fibrous Dysplasia/MacCune-Albright Syndrome (FD/MAS) patients using a qualitative approach with focus groups to explore perceptions, symptoms and limitations associated with FD/MAS and a quantitative method with the Short Form-36 (SF36) to quantify QoL. Focus groups revealed the heterogeneity of FD forms and allowed for understanding the reasons of reduced QoL.
View Article and Find Full Text PDFJ Oral Maxillofac Surg
December 2024
Assistant Professor, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA; Assistant Professor, Department of Psychiatry, McLean Hospital, Harvard Medical School, Belmont, MA. Electronic address:
Background: Fibrous dysplasia/McCune-Albright syndrome (FD/MAS) is a genetic disorder, marked by bone lesions, often affecting the craniofacial skeleton. Pain is a prevalent yet heterogeneous symptom reported by patients with craniofacial FD. Effective treatments are currently lacking, posing a significant clinical challenge to patient care.
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