Abstract
McCune-Albright syndrome consists of polyostotic fibrous dysplasia, precocious puberty and cafe-au-lait skin lesions. Bone lesions are characterized by the presence of fibrous connective tissue with a characteristic whorled pattern and containing trabeculae of immature non-lamellar (woven) bone. They may be solitary (monostotic) or multiple (polyostotic). Commonly involved bonnes include the femur, tibia, ribs and facial skeleton. In the fibula pseudo-cystic areas and 'ground glass'-like areas, in the femur 'shepherd crook' deformation due to weight on a less resistant bone and secondary to many cortical microfractures, are typical. MRI gives the exact delimitation of the lesions and is especially indicated in the follow-up of monostotic forms and in the outcome of surgical corrections. Bone scintigraphy with technetium 99 is essential in the follow-up of the disease. Ultrasonography is very useful in ovarian cyst follow-up and in the detection of thyroid and adrenal nodules and testicular microlithiasis.
Original language | English |
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Pages (from-to) | 561-570 |
Number of pages | 10 |
Journal | Journal of Pediatric Endocrinology and Metabolism |
Volume | 19 |
Issue number | SUPPL. 2 |
Publication status | Published - 2006 |
Keywords
- Bone fibrous dysplasia
- Imaging
- McCune-Albright syndrome
ASJC Scopus subject areas
- Endocrinology
- Pediatrics, Perinatology, and Child Health