Background and objectivesRegional and neuraxial anesthesia techniques have become instrumental in the perioperative period yet have not been well described in patients with osteogenesis imperfecta (OI), a congenital connective tissue disorder characterized by skeletal dysplasia and fragility. Patients with skeletal dysplasia present unique perioperative challenges that warrant consideration of these techniques despite their relative contraindication in this population due to reports of increased bleeding with surgery, skeletal fragility concerns with positioning, and risk of spinal cord injury with continuous neuraxial catheters. The aim of this narrative review was to evaluate literature describing the use of regional and neuraxial techniques in patients with OI and any associated clinical outcomes.MethodsAll available literature from inception to July 2020 was retrieved, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, from MEDLINE, Embase, Google Scholar and The Cochrane Library. Three authors reviewed all references for eligibility, abstracted data, and appraised quality.ResultsOf 412 articles initially identified, 42 met our inclusion criteria, yielding 161 cases with regional and/or neuraxial techniques described. In 117 (72.6%) of the 161 cases, neuraxial technique was performed, including 76 (64.9%) epidural, 7 (5.9%) caudal, 5 (4.2%) combined spinal epidural, and 29 (24.7%) spinal procedures. In 44 (27.4%) of the 161 cases, the use of regional anesthesia was described. Our review was confounded by incomplete data reporting and small sample sizes, as most were case reports. There were no randomized controlled trials, and the two single-center retrospective data reviews lacked sufficient data to perform meta-analysis. While complications or negative outcomes related to these techniques were not reported in any of the cases, less than half specifically discuss outcomes beyond placement and immediate postoperative course.ConclusionsThere is insufficient evidence to validate or refute the potential risks associated with the use of regional and neuraxial techniques in patients with OI. This review did not uncover any reports of negative sequelae related to the use of these modalities to support relative contraindication in this population; however, further research is needed to adequately assess clinically relevant outcomes such as complications and opioid-sparing effect.
