Perioperative Management of Pediatric Patients with Osteogenesis Imperfecta Undergoing Orthopedic Procedures

Osteogenesis imperfecta (OI) is a predominantly autosomal dominant inherited condition involving connective tissue associated with not only bone fragility, but multi-system involvement with perioperative implications. The perioperative management of patients with OI undergoing orthopedic procedures is high risk for anatomical and physiological reasons. We performed a retrospective analysis of 13 years of data covering the perioperative management of 146 pediatric patients with OI for 280 operative procedures involving up to five bones each by two fellowship-trained pediatric orthopedic surgeons and a team of pediatric anesthesiologists. The purpose of the review was to describe updated perioperative anesthetic management based on over a decade of experience in a pediatric hospital specializing in the management of OI patients.