Abstract

Osteogenesis imperfecta (OI) is a rare connective tissue disease with varying severity. Patients with OI are highly susceptible to skeletal fractures. Optimal perioperative management of these patients is not well defined. We investigated the risks associated with intraoperative use of noninvasive blood pressure (NIBP) cuffs, tourniquets, and intra-arterial catheters, and patient positioning in children with OI. We retrospectively reviewed records of patients younger than 21 years with OI who underwent surgery with general anesthesia from 2010 to 2016 at our tertiary care center. The primary outcome of interest was iatrogenic fracture caused by NIBP cuff use, tourniquet use, or patient positioning. The secondary outcome of interest was complications associated with intra-arterial catheter use. Thirty-seven patients (15 girls) with a mean age of 10±4.8 years underwent 96 orthopaedic procedures (lower extremity, upper extremity, and spine) and 2 nonorthopaedic procedures (myringotomy, dental rehabilitation). Blood pressure was monitored with NIBP cuffs in 81 surgeries and intra-arterial catheters in 17 surgeries. Tourniquets (all applied to the lower extremity at a pneumatic pressure of 250 mm Hg) were used to minimize bleeding in 30 surgeries. There were no iatrogenic fractures associated with NIBP cuff use. One patient had a left humerus fracture that occurred during preoperative patient positioning. There were no fractures associated with tourniquet use and no complications related to intra-arterial catheters. In pediatric patients with OI, intraoperative use of NIBP cuffs and tourniquets was not associated with iatrogenic fracture. There were no complications related to intra-arterial catheter use. Care should be used during the perioperative period to prevent fractures during body positioning. Level IV.

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