The Spanish Association of Pediatrics has as one of its main objectives the dissemination of rigorous and updated scientific information on the different areas of pediatrics. Annals of Pediatrics is the Body of Scientific Expression of the Association and is the vehicle through which members communicate. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years. CiteScore measures average citations received per document published.

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For a general discussion of the underlying pathology, refer to the parent article fibrous dysplasia. Young adult patients are most frequently affected. Refer to fibrous dysplasia article for a discussion of epidemiology. Occasionally it is seen in the setting of McCune-Albright syndrome 2. Refer to fibrous dysplasia article for a discussion of pathology. Extracranial involvement is rare 6. Similar to fibrous dysplasia elsewhere, affected bones demonstrate a variety of radiographic features ranging from lucency to sclerosis.

Affected bones are usually expanded with an intact cortex and lose the normal corticomedullary differentiation, being replaced classically by a homogeneous ground glass appearance, although mixed lucencies and sclerosis are also common Sometimes the mixed regions of sclerosis and lucency are reminiscent of Paget disease , and are thus referred to as 'pagetoid' 3. When the maxilla or mandible are involved, resorption of the roots of teeth is uncommon 2.

Treatment is reserved for cases where function is being threatened particularly the airway or vision and is surgical, although much controversy exists over the best approach e. As the region affected is often large and involves complex facial anatomy, complete resection is usually not possible. Likewise, reconstruction can be challenging 5. Historically, radiotherapy was used to attempt to control growth. This not only was of limited success but encouraged sarcomatous degeneration, and is thus now contraindicated 5.

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On this page:. Article: Terminology Epidemiology Clinical presentation Pathology Radiographic features Treatment and prognosis Differential diagnosis References Images: Cases and figures Imaging differential diagnosis. Quiz questions. Fibrous dysplasia involving the base of the skull. Promoted articles advertising.

Edit article Share article View revision history Report problem with Article. URL of Article. Article information. Synonyms or Alternate Spellings: Cranio-facial fibrous dysplasia. Support Radiopaedia and see fewer ads. Cases and figures. Case 1 Case 1. Case 2 Case 2. Case 3 Case 3. Case 5 Case 5. Case 6 Case 6. Case7 Case7. Case 8 Case 8. Case 9 Case 9. Case 10 Case Case mandibular Case mandibular. Case 12 Case Case 13 Case Imaging differential diagnosis.

Paget's disease Paget's disease. Meningioma with florid hyperostosis Meningioma with florid hyperostosis. Cemento-ossifying fibroma Cemento-ossifying fibroma.

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CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact. SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field.


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