Joint Replacement Surgery in Patients with Skeletal Dysplasias

Skeletal dysplasias are commonly associated with an increased risk for joint degeneration and osteoarthritis, particularly affecting the large, weight-bearing joints of the lower extremities. Depending on the specific condition, potential causes to varying extent include both, the underlying metabolic disorder which may directly interfere with joint health as well as unfavorable biomechanical loading due to deformities and particular individual aspects of mobilization and mobility. Consecutively, patients with skeletal dysplasias and their physicians are regularly facing debilitating osteoarthritis and the medical need to overcome associated pain and mobility issues by joint replacement surgery, inevitably raising questions if and how this can be safely and sustainably accomplished. Key challenges in this context beyond regular endoprosthetics consist of particular anatomical and biomechanical setting, frequently with short stature, requiring appropriately sized and sometimes individually shaped implants. In addition, the task of optimal implant fixation and retention in a setting of compromised bone quality and bone biology have to be addressed along with questions around potential supportive treatment strategies to improve bone stability and healing capacity perioperatively. This includes considerations around sequelae and implications resulting from previous medical and surgical interventions. Planning requires being mindful about foreseeable challenges regarding postoperative rehabilitation as well as forthcoming surgeries. Conclusively, these considerations and concepts will be illustrated and exemplified, referring to comparatively common skeletal dysplasias like osteogenesis imperfecta (OI), Hypophosphatasia (HPP) and X-linked hypophosphatemia (XLH).