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Congenital Bone Disorders

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Intramedullary Nailing

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Abstract

The term congenital bone disorder describes alterations in typical combination that present themselves at one or multiple parts of the skeletal system. Osteogenesis imperfecta (OI) is a genetic heterogeneous group of bone disorders. In case of diaphyseal fractures, telescopic rods are fixed in the proximal and distal epiphyses of the long bones. The design allows that the rods grow with the bone. These rods dramatically reduced the number of operations needed for one child. But complications like the cutting out of the nail or bowing of the bone around the nail can occur in any method of treatment due to the nature of the disease. Fibrous dysplasia is a non-hereditary, benign tumorous bone disorder, in which normal bone is displaced by fibrous bone. Surgical approaches include removing the affected intraosseous lesion and filling this cavity with autologous or allogenic bone material. Depending on the extent of the fibrous dysplasia, one also has to consider stabilizing the bone by intramedullary rods. Hypophosphatasie is a rare autosomal recessive disease, which presents mainly in a hypomineralization of the bone and the teeth. Intramedullary rodding can be performed once there are symptomatic fractures. The entry portal of the femur is situated at the tip of the greater trochanter. For the tibia, the entry portal is situated at the tibia tuberosity. The entry point should be exactly in the center of the medullary canal in the anteroposterior view. For the humerus, the ideal entry point is situated medial to the greater tuberosity, which is lateral to the axis of the medullary canal in the anteroposterior view and in line with the axis in the lateral view. Rod migration and telescoping are the two most frequent complications.

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Authors and Affiliations

  1. Orthopedic and Trauma Surgery, University Clinic Frankfurt Friedrichsheim, Marienburgstrasse 2, Frankfurt, 60528, Germany

    Dara Froemel MD & Andrea Meurer MD, PhD

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  1. Dara Froemel MD
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  2. Andrea Meurer MD, PhD
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Correspondence to Dara Froemel MD .

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Editors and Affiliations

  1. Center for Orthopedics &Traumatology, University Medical Center Johannes Gutenberg University, Mainz, Germany

    Pol M. Rommens

  2. Academic Teaching Hospital Fulda, Fulda, Germany

    Martin H. Hessmann

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Froemel, D., Meurer, A. (2015). Congenital Bone Disorders. In: Rommens, P., Hessmann, M. (eds) Intramedullary Nailing. Springer, London. https://doi.org/10.1007/978-1-4471-6612-2_33

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  • DOI: https://doi.org/10.1007/978-1-4471-6612-2_33

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