Bone Abstracts

Osteogenesis imperfecta is a disorder characterized by bone fragility. Current management includes the usage of bisphosphonates to improve bone stock and manage pain. Outcomes in adults have mainly been reported in terms of presentation compared to genotype, most probably due to the heterogeneity of the disease. The head and neck resection of the femur, or Girdlestone procedure, was a common procedure to treat infections of the hip but it has fallen out of favor due to the advent of hip replacements, and its use has been limited to a last resort. Functional outcomes and patient satisfaction have been reported in small samples of unilateral Girdlestone arthroplasty; however, the bilateral application of the procedure has remained anecdotal thus far, and is of novel use in a patient with osteogenesis imperfecta. Through the efforts of physiatrists, geneticists and orthopedic surgeons, with the use of rehabilitation management, pharmaceutical interventions in the form of bisphosphonates, and well planned surgical approaches, the patient was able to achieve pain-free sitting, independent transitions, and short-distance ambulation. These functional improvements have allowed the patient to care for herself more effectively and return to her work as a municipal accountant. The outcome measures taken quantitatively measure the patient’s improvement and provide guidance as to which areas need more focus, particularly in terms of interventions for fracture prevention and improvement of balance. The outcome measures also place the patient in the context of other cases worldwide, highlighting their differences in terms of physical attributes, and similarities in terms of their functional attainment.

Disclosure: The authors declared no competing interests.