Anesthetic implications for the patient with osteogenesis imperfecta

Osteogenesis imperfecta is an inherited disorder of the connective tissue stemming from gross abnormalities in collagen formation and structure. Affected patients fall into 4 classifications each displaying the similar properties of easily fractured bones, hypermobile joints, blue or gray sclera, skeletal deformities, and fragile skin. More severe forms of the disease may manifest platelet dysfunction, cardiac anomalies, hypermetabolic syndromes, respiratory compromise, and/or basilar invagination. Treatment of osteogenesis imperfecta is mainly supportive, consisting of prompt surgical treatment of fractures to prevent deformity and maintaining mobility to lessen the chance of pulmonary or cardiovascular complications. These treatment modalities make anesthesia of paramount importance. Current literature exposes many potential anesthetic complications associated with osteogenesis imperfecta. The research shows that implications range from simply positioning the patient on the operating room table to management of rare occurrences such as malignant hyperthermia and basilar invagination. Commonly encountered complications include a difficult airway, intraoperative bleeding due to platelet dysfunction, respiratory compromise due to skeletal deformity, and congenital cardiac anomalies. Proper preparation and preoperative assessment is important, as is the choice of anesthetic technique. Correct identification of risk factors and optimization of health before surgery should lead to an uneventful anesthetic course.