Perioperative care of the patient with osteogenesis imperfecta.

Osteogenesis imperfecta is an inherited disorder of the connective tissue whose primary manifestation is an increased susceptibility to fractures. Severely affected patients often suffer multiple fractures after minimal or no trauma. In addition to its primary effect on the skeletal system, the alterations in connective tissue may affect several extraskeletal structures, such as the cardiovascular system, sclera, middle and inner ear, tendons/ligaments, central nervous system, and teeth. Patients with osteogenesis imperfecta also have a greater incidence of airway anomalies, thoracic anatomy abnormalities, coagulation dysfunction, hyperthyroidism, and an increased tendency to develop perioperative hyperthermia. Given the multisystem involvement of osteogenesis imperfecta, several issues exist that may impact the perioperative management of these patients. Of particular concern are the associated cardiovascular anomalies, increased incidence of perioperative bleeding, easily fractured bones and teeth, airway anomalies, the tendency to develop intraoperative hyperthermia, and hyperthyroidism.