Osteogenesis imperfecta (OI) which is also known as Vroliks disease is a connective tissue disorder. OI tarda is an autosomal recessive disorder associated with abnormalities of type 1 collagen. The other form of the disease known as OI congenita is a dominant form in which the child usually dies in utero. This disorder associated with many potential problems; fragile bones and teeth, abnormal airway, megalocephaly, macroglossia, short neck, thoracic deformities, susceptibility to malignant hyperthermia, hyperthyroidism, congenital cardiac diseases, emphysema and bleeding disorders due to platelet dysfunction are the most prominent problems concerning anesthesia management. Also, many potential problems are concerning operative management. It might be greatly increases the risk of a refracture due to the pathological induction of stress risers. Therefore, fracture treatment by unreamed elastic intramedullary nailing was combined with simultaneous correction osteotomy, resulting in anatomic alignment and uncomplicated fracture healing. The single stage surgical stabilization performed allowed rapid mobilization along with a decreased likelihood of refracture.
Last date updated on July, 2014