Total femur arthroplasty for revision hip failure in osteogenesis imperfecta: limits of biology

Abstract

Osteogenesis imperfecta (OI) is a rare congenital disease characterized by alterations in bone quality, with susceptibility to fractures, instability, deformities, and osteoarthrosis. Prosthetic surgery in these patients is associated with an abnormally high rate of implant failures. On the other hand, abnormal bone fragility adds to the complexity of revision surgery in such individuals—thus representing a genuine challenge for the orthopaedic surgeon. We present a case of femoral reconstruction in a patient with OI and prosthetic loosening after reconstruction secondary to femoral septic pseudoarthrosis. Intramedullary total femoral reconstruction was carried out after exceeding the biological reconstruction limits. This is the first reported instance of the use of an intramedullary total femur arthroplasty as salvage technique in an OI patient. This technique should be considered when we have exceeded biological limits for femoral fixation.

Total knee replacement in Osteogenesis Imperfecta: a case report and review of the literature

“…[ 7 ] 1 69, F Osteoarthritis Posterior-stabilised Sanz-Ruiz et al . [ 1 ] 1 65, F Hip femoral stem perforated through trochlea Total femur replacement, hinged prosthesis and stemmed tibia …”

“…It presents in 1 in 20,000 births and is caused by a genetic alteration resulting in abnormal collagen structure, ossification and mineralisation. This condition leads to various orthopaedic manifestations and inherent coagulation defects due to abnormal collagen within the endothelial capillary bed [ 1 , 2 ].…”

“…OI patients, thanks to modern medical advances, have a virtually normal life span [ 4 ]. The joint laxity and fractures with secondary joint degeneration leads to hip and knee osteoarthrosis requiring joint arthroplasty [ 1 ].…”

Challenges of total knee arthroplasty in osteogenesis imperfecta: case report and literature review

“… 8 , 9 , 14 – 18 One additional case report described a failed hip femoral revision component perforating the femoral trochlea due to poor bone quality that was treated by total femur replacement. 19 …”

Pauwels Osteotomy for Femoral Neck Nonunion in Two Adult Siblings with Osteogenesis Imperfecta

“…Surgical alternatives are few for these problems. A customized hip replacement is fraught with other problems such as high costs, infection, loosening and limited bone stock 10) . A girdlestone would have been an alternative but both patients were both quite mobile and not willing to give up their hip.…”