Osteogenesis imperfecta is a genetic disease resulting in abnormal collagen formation, with multiple clinical manifestations. Advancements in medical and surgical treatments have prolonged the life expectancy of these patients in recent decades. As a result, orthopedic surgeons are likely to be faced with the challenge of performing arthroplasty in these patients on a more frequent basis. Here, we describe a patient with osteogenesis imperfecta and subsequent severe osteoarthritis prompting primary total knee arthroplasty. This rare case presents an opportunity to explore special considerations unique to this patient population, including comorbid bone defects, the need for using extramedullary guides, careful alignment of prostheses to accommodate abnormalities in limb axes, and equipment utilization.
Keywords: Osteogenesis imperfecta; Total knee arthroplasty.