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Fibrous dysplasia: A tale of two syndromes

التفاصيل البيبلوغرافية
العنوان: Fibrous dysplasia: A tale of two syndromes
المؤلفون: Jacques Fourie, Farhana Suleman, Zarina Lockhat, Kumeshnie Kollapen
المصدر: South African Journal of Radiology, Vol 28, Iss 1, Pp e1-e5 (2024)
بيانات النشر: AOSIS, 2024.
سنة النشر: 2024
المجموعة: LCC:Medical physics. Medical radiology. Nuclear medicine
مصطلحات موضوعية: fibrous dysplasia, mazabraud syndrome, mccune–albright syndrome, musculoskeletal, skeletal dysplasia, Medical physics. Medical radiology. Nuclear medicine, R895-920
الوصف: Fibrous dysplasia (FD) is a rare, non-inherited, congenital bone disorder which may be monostotic or polyostotic. The polyostotic form may rarely present in syndromic forms when associated with extra-skeletal manifestations. Mazabraud syndrome is a rare syndrome consisting of polyostotic FD presenting with intramuscular myxomas. McCune–Albright syndrome is recognised by polyostotic FD, precocious puberty and ‘café au lait’ spots. This report describes an adult patient with Mazabraud syndrome and a child with McCune–Albright syndrome. Contribution: Radiographic findings are typical with bowing deformities, sclerotic, lucent or mixed lesions and bony expansion, often with endosteal scalloping. MRI is often non-contributory and may actually mimic a more aggressive process. Early detection and correct diagnosis allow for early preventative treatment and rehabilitation to prevent devastating neurological sequelae and disability.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1027-202X
2078-6778
العلاقة: https://sajr.org.za/index.php/sajr/article/view/2877Test; https://doaj.org/toc/1027-202XTest; https://doaj.org/toc/2078-6778Test
DOI: 10.4102/sajr.v28i1.2877
الوصول الحر: https://doaj.org/article/e044fb925caa419896089954a56a6ad0Test
رقم الانضمام: edsdoj.044fb925caa419896089954a56a6ad0
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:1027202X
20786778
DOI:10.4102/sajr.v28i1.2877