Osteogenesis Imperfecta
摘要
Osteogenesis imperfecta is a rare heterogeneous skeletal dysplasia characterized by low bone density that can manifest as bone fragility with multiple repetitive fractures leading to functional impairment and short stature. Patients with osteogenesis imperfect may require multiple interventions, creating unique challenges for anesthesiologists. Challenges facing the anesthesiology team when caring for a patient with osteogenesis imperfecta are many: (1) extreme fragility of the bones, (2) inability to employ standard anesthesiology monitoring tools such as sphygmomanometer to check blood pressure and/or monitoring for neuromuscular blockade using peripheral nerve stimulation, (3) greater risk when performing regional anesthesia due to inability to stimulate target nerve and plexus to prevent neural injury, (4) difficulty obtaining intravenous access, and (5) greater than average blood loss.