Osteogenesis imperfecta (OI) is a genetically determined systemic pathology that involves a disturbance in the synthesis of type one collagen and is mainly characterized by bone brittleness and other abnormalities. The musculoskeletal system is the most affected by bone fracture after mild mechanical stress occurs. Pathologic bowing appears without trauma, and hyperlaxity is jeopardizing joint stability. Having such an abrupt debut, some patients report in-utero fractures, and treatment is very challenging since surgery has to be postponed until the children grow and become good candidates for intramedullary nailing. In this paper, we discuss the experience of "Grigore Alexandrescu" Hospital for Children in treating these patients and the results we obtained regarding the revision surgeries that we had done. Revision surgery is mandatory for OI patients, and there are multiple factors in deciding to use an implant. We looked back at the data collected for those cases where revision was not related to infection, trauma, or disengagement of the male-female system due to the overlengthening of the implant. The conclusions made are comparable to other centers worldwide with respect to lower limb surgery, and we changed the management protocol according to the problems encountered.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7803301PMC
http://dx.doi.org/10.25122/jml-2020-0161DOI Listing

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