Caffey Disease
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Caffey Disease Articles:

• Source: Pediatric Database (PEDBASE)
  http://www.icondata.com/health/pedbase/files/CAFFEYDI.HTM
  
  Incidence: 48/100,000, age of onset: usually around 9 wks and rarely after 5 months of age
  
  Bones Affected: 80% - mandible, 50% - clavicle, others - ulna, tibia, scapulae, ribs, occasionally - humerus, fibula, femur, more than one bone is usually affected and is not symmetrical, swelling may resolve in one site and arise at another, defects have been noted as early as 24 weeks in utero
  
  CLINICAL FEATURES:
  1. Constitutional: hyperirritability, fever
  2. Musculoskeletal Manifestations: swelling of soft tissues over the affected area, very rarely may get long-term complications, residual deformities of affected site, fusion of adjacent bones, anterior bowing of tibia or femur, lower limb length discrepancy, persistent facial asymmetry
  
  Serum: mildly elevated alkaline phosphatase and ESR +/- anemia, thrombo-cytopenia, and elevated IgA and IgM,
  
  Skeletal X-rays: Cortical Hyperostoses subperiosteal new bone formation with progressive cortical thickening and widening of the long and flat bones initially the external surface of the new bone is coarse and the underlying cortex is visible but as the disease progresses, the density of the new bone increases and becomes homogeneous with the underlying cortex soft tissue swelling noted over the affected site affected areas confined to the diaphysis while the metaphysis and epiphysis are spared
  
  
• Source: OMIM Database - Johns Hopkins University
  http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?114000
  
  This condition has somewhat unusual features for a hereditary disorder. It rarely if ever appears after 5 months of age; it is sometimes present at birth and has been identified by x-ray in the fetus in utero. The acute manifestations are inflammatory in nature, with fever and hot, tender swelling of involved bones (e.g.,mandible, ribs). Despite striking radiologic changes in the acute stages, previously affected bones are often completely normal on restudy.
  
  
• Source: Virtual Children's Hospital: Children's Hospital of Iowa
  http://www.vh.org/Providers/TeachingFiles/PAP/MSDiseases/CaffeyDis.html
  
  Clinical Presentation: Limited to first few months of life. Can be seen in newborns, can be familial. Presents with irritability, soft tissue swelling, underlying cortical thickening. Also see fever, pallor, pseudoparalysis.
  
  Etiology/Pathophysiology:Is a hyperostotic inflammatory reaction with the etiology unknown.
  
  Imaging Findings: Most frequently in mandible, ribs, clavicles, long bones, scapula, short tubular bones, pelvis. See a dense periosteal reaction. DDX: Child abuse, Congenital syphilis, Rickets, Scurvy. Can tell them apart because in Caffey's the periosteal reaction is only diaphyseal in location and it spares the metaphyses and epiphyses.
  
  
• Source: Medical College of Wisconsin
  http://chorus.rad.mcw.edu/doc/00054.html
  
  Caffey disease - "infantile cortical hyperostosis", etiology unknown, onset before 5 months of age, hyperostosis + periosteal reaction, mandible (80-95%), clavicles, ribs, long bones (DIAPHYSES often asymmetric) DDx: if metaphyses involved, consider battery (child abuse)