Osteogenesis Imperfecta

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(Brittle Bone Disease)

  • defective osteoid formation

  • due to congenital inability to form adequate intercellular substances

    • osteoid

    • collagen

    • dentine

  • failure of maturation of collagen in all tissues

Clinical Features

  • broad skull

  • blue sclera

  • premature

  • deafness

  • scoliosis

  • ligament laxity

  • coxa vara

    • decrease of the femoral neck shaft angle to less than 120-135°

  • knock knees

Investigations

  • translucent bones

  • multiple fractures

  • wormian bones

    • irregular patches of ossification

  • trefoil pelvis

 

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