IMAGING  

• Osseous fragility,blue sclera,otosclerosis,Dentinogenesis imperfecta
  
  
• Postnatal x-ray: Obtain skull, chest, long bones, and pelvis x-ray films.
  
  
• Type I
  
  
  • Tam O'Shanter skull - Flattening in vertical axis and widening in transverse axis
    
    
  • Thin bones
    
    
  • Multiple wormian bones
    
    
  • Fractures with deformities
    
    
  • Osteopenia
    
    
  • Platyspondylia
    
    
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• Type I
  
  
• A: Dentinogenesis imperfecta is absent.
  
  
• B: Dentinogenesis imperfecta is present.
  
  

• Type II
  
  
  • Beaded ribs
    
    
  • Broad bones
    
    
  • Fractures with deformities
    
    
  • Osteopenia
    
    
  • Platyspondylia
    
    
  • Blue sclera may be present
    
    
  • In utero fractures are present in 100% cases .
    
    

• Type III
  
  
  • Cystic metaphyses (popcorn appearance)
    
    
  • Normal or broad bones early on; thin bones later
    
    
  • Fractures with deformities
    
    
  • Osteopenia
    
    
  • Sclera of variable hue
    
    
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• Type IV
  
  
  • Thin bones
    
    
  • Fractures with deformities
    
    
  • Osteopenia
    
    
• Prenatal ultrasound
  
  
• Prenatal ultrasound can be used to detect limb length abnormalities at 15-16 weeks’ gestation.
  
  
• Limb abnormality can be detected by 15-18 weeks’ gestation. Mild forms may have normal sonogram findings.
  
  
• Features
  
  
  • Supervisualization of intracranial contents caused by decreased mineralization of calvarium (also calvarial compressibility)
    
    
  • Long bones bowing, decrease in length (especially the femur)
    
    
  • Multiple rib fractures
    
    
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