Osteogenesis Imperfecta

The surgical treatment of lower extremities deformities in osteogenesis imperfecta (OI) remains a challenge despite recent improvements in the medical treatment, the development of new surgical tools, and a team approach including postoperative rehabilitation. Preoperative assessment is crucial to appreciate the soft-tissue tension and evaluate bone deformities and the size of the medullary canal. Osteotomies and intramedullary (IM) rodding is the solution of choice to support fragile bones, while plates create a stress-raiser effect and should not be used. The choice of IM rods remains controversial. While regular IM devices (e.g., Rush rods, Kirschner wires, and elastic nails) are easy to use and readily available, they are rapidly outgrown and lead to multiple interventions during skeletal growth. The telescopic (or growing) rods (such as Dubow-Bailey, Sheffield, and Fassier-Duval) are not always available, more expensive, and more difficult to use. However, the reoperation rate with these devices is generally lower than with more conventional devices. Parents and caregivers must remember though that despite these advances in the management, several surgical interventions will likely be necessary during growth.