Archival Issues
Volume 19, 2017
Volume 18, 2016
Volume 17, 2015
Volume 16, 2014
Volume 15, 2013
Volume 14, 2012
Volume 13, 2011
Volume 12, 2010
Volume 11, 2009
Volume 10, 2008
Volume 9, 2007
Volume 8, 2006
Volume 7, 2005
Volume 6, 2004
Volume 5, 2003
Volume 4, 2002
Volume 3, 2001
Volume 2, 2000
Volume 1, 1999
Journal Abstract
Familial occurrence of Caffey-Silverman syndrome
Aleksander Szwed , Maciej Kołban Kołban, Hanna Romanowska , Elżbieta Baryła-Pankiewicz
Ortop Traumatol Rehabil 2012; 14(1):75-83
ICID: 976906
Article type: Case report
IC™ Value: 1.80
Abstract provided by Publisher
Caffey-Silverman syndrome, or infantile hyperostosis, is a rare condition of unclear etiology and pathogenesis affecting the skeletal system and the surrounding soft tissues. It is characterized by indurated swelling of soft tissues and cortical bone hyperostosis. The changes are usually multiple and affect such parts as the mandible, scapulae, ribs, clavicles, and forearm and shank bones. When long bones are affected, the lesions are typically limited to shafts, with the sparing of meta- and epiphyses. The prognosis is usually good and, in most patients, the changes resolve spontaneously after several months to over a year, leaving no permanent sequelae. Caffey-Silverman syndrome needs to be distinguished from osteitis, for which it is most often mistaken.
We present a case of an early form of Caffey-Silverman syndrome. The course of disease in this form is usually severe, with multifocal lesions, and the typical self-limiting regression is not complete. In our patient, a systemic musculoskeletal condition was already suspected following the detection of skeletal defects in a prenatal US examination.
Physical and radiological work-up in the first weeks of life revealed the typical signs of congenital Caffey-Silverman syndrome. Several years of follow-up showed gradual regression of the bone deformities with persistent lower-extremity bowing. Due to a positive family history for Caffey-Silverman syndrome, the patient’s pedigree was prepared. On the basis of the history data, existing radiographs and in- and outpatient medical records, Caffey-Silverman syndrome was confirmed in 10 family members. It was established that the cases of Caffey-Silverman syndrome in the patient’s family were characterized by diverse phenotypic expression and different times of onset.

ICID 976906

DOI 10.5604/15093492.976906
PMID 22408114 - click here to show this article in PubMed

Related articles
  • in IndexCopernicus™
         Caffey-Silverman [0 related records]
         hiperostoza niemowlęca [0 related records]
         korowy przerost kości [0 related records]
         infantile hyperostosis [0 related records]
         cortical hyperostosis [0 related records]

  • Related articlesin PubMed database
  • in PubMed database [ related records]


    Copyright © Ortopedia Traumatologia Rehabilitacja  2017
    Page created by Index Copernicus Ltd. All Rights reserved.