Image diagnosis in McCune-Albright syndrome

Claudio Defilippi, Domenico Chiappetta, Daniela Marzari, Alessandro Mussa, Roberto Lala

Research output: Contribution to journalArticlepeer-review

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 languageEnglish
Pages (from-to)561-570
Number of pages10
JournalJournal of Pediatric Endocrinology and Metabolism
Volume19
Issue numberSUPPL. 2
Publication statusPublished - 2006

Keywords

  • Bone fibrous dysplasia
  • Imaging
  • McCune-Albright syndrome

ASJC Scopus subject areas

  • Endocrinology
  • Pediatrics, Perinatology, and Child Health

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