Neuraxial and regional anesthesia in surgical patients with osteogenesis imperfecta: a narrative review of literature.

BACKGROUND AND OBJECTIVES: Regional 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. METHODS: All 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. RESULTS: Of 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. CONCLUSIONS: There 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.

The Road to Transfusion-free Craniosynostosis Repair in Children Less Than 24 Months Old: A Quality Improvement Initiative.

INTRODUCTION: Pediatric craniofacial reconstruction has historically resulted in extensive blood loss necessitating transfusion. This single-center quality improvement initiative evaluates the impact of perioperative practice changes on the allogeneic transfusion rate for children 24 months and younger of age undergoing craniofacial reconstruction. METHODS: At project initiation, an appointed core group of anesthesiologists provided all intraoperative anesthetic care for patients undergoing craniofacial reconstruction. Standardized anesthetic guidelines established consistency between providers. Using the Plan-do-check-act methodology, practice changes had been implemented and studied over a 5-year period. Improvement initiatives included developing a temperature-management protocol, using a postoperative transfusion protocol, administering intraoperative tranexamic acid, and a preincisional injection of 0.25% lidocaine with epinephrine. For each year of the project, we acquired data for intraoperative and postoperative allogeneic transfusion rates. RESULTS: A cohort of 119 pediatric patients, ages 4-24 months, underwent anterior or posterior vault reconstruction for craniosynostosis at a tertiary children's hospital between March 2013 and November 2018. Intraoperative and postoperative transfusion of allogeneic blood products in this cohort decreased from 100% preintervention to 22.7% postintervention. CONCLUSIONS: Interdepartmental collaboration and practice modifications using sequential Plan-do-check-act cycles resulted in a bundle of care that leads to a sustainable decrease in the rate of intraoperative and postoperative allogeneic blood transfusions in patients less than 24 months of age undergoing craniosynostosis repair. This bundle decreases the risk of transfusion-related morbidity for these patients. Other institutions looking to achieve similar outcomes can implement this project.

Associations between Charlson Comorbidity Index and surgical risk severity and the surgical outcomes in advanced-age patients.

The Charlson Comorbidity Index (CCI) has not been assessed for elderly (95 years of age or older) surgical patients. We examined the association between the CCI and life-threatening complications and 30-day mortality rate. Medical records of patients 95 years old or older from 2004 through 2008 were reviewed for major postoperative morbidity or death. Logistic regression analyses of age, sex, the CCI, American College of Cardiology/American Heart Association Surgical Risk Stratification, and surgical urgency were performed to identify associations with poor surgical outcome. One hundred eighty-seven patients were identified (mean [standard deviation] age, 96.6 [1.9] years; median [interquartile range] CCI, 4 [2 to 6]). Ninety patients (48.1%) underwent moderate-risk and 20 (10.7%) underwent high-risk surgical procedures. Twenty patients (10.7%) died within 30 postoperative days and 20 others had major morbidity. Only moderate-risk (P = 0.045) and high-risk surgical procedures (P = 0.001) were associated with poor outcome. Patients of advanced age have high rates of morbidity and death after surgical procedures. These events are associated with surgical risk stratification and are independent of patient comorbidities. Risks, benefits, and alternatives must be considered carefully and discussed with patients and their families before deciding to proceed with high-risk surgery.

Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery.

OBJECTIVE: DiGeorge syndrome is a genetic disorder with multisystem involvement resulting in craniofacial and cardiac anomalies and parathyroid and immune system dysfunction. This study describes perioperative management of a large cohort of patients with DiGeorge syndrome undergoing cardiac surgery. DESIGN: Retrospective cohort study. SETTING: Major academic tertiary institution. PARTICIPANTS: The medical records of patients diagnosed with DiGeorge syndrome and undergoing cardiac surgery at this institution, from January 1, 1976, to July 31, 2012, were reviewed for phenotypic characteristics and intraoperative and postoperative complications, with specific attention to hemodynamic instability, perioperative perturbations of plasma calcium homeostasis, and airway difficulty. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Sixty-two patients underwent 136 cardiac surgical procedures; 47 patients (76%) had multiple operations. Sternotomies for reoperations often were complex (8 complicated by vascular injury or difficulty achieving hemostasis and 5 requiring bypass before sternotomy). Two patients had persistent hypocalcemia intraoperatively, requiring infusion of calcium chloride, and hypocalcemia developed postoperatively in 8 patients. Prolonged mechanical ventilation (>24 hours) was required after 48 procedures (35%), and 25 (18%) required prolonged inotropic support (>72 hours). Infectious complications occurred after 31 procedures (23%). There was no in-hospital or 30-day mortality. CONCLUSIONS: Patients with DiGeorge syndrome often have complex cardiac anomalies that require surgical repair. The postoperative course is notable for the frequent need for prolonged respiratory and hemodynamic support. Patients can develop hypocalcemia and may require calcium supplementation. Immunodeficiencies may be associated with the increased rate of postoperative infections and may dictate the need for specific transfusion management practices.