Anesthetic Management and Outcomes of Patients With Epidermolysis Bullosa: Experience at a Tertiary Referral Center.

BACKGROUND: Epidermolysis bullosa (EB) is a group of rare epithelial disorders caused by abnormal or absent structural proteins at the epidermal-dermal junction. As a result, patients experience blisters and wounds from mild shearing forces. Some forms of EB are complicated by resultant scarring and contractures. The perioperative anesthetic management of patients with EB is complex and requires a systems-based approach to limit harm. We reviewed our experience with providing general anesthesia to patients at our tertiary EB referral center, including adverse events related to anesthetic care, outcomes in the immediate perioperative period, and details of anesthetic management. METHODS: We retrospectively reviewed the charts of all patients with EB anesthetized at the Children's Hospital Colorado between January 2011 and December 2016. A subset of pediatric anesthesiologists cared for all patients using a standardized clinical care pathway. Patient demographics, detailed anesthetic methods, immediate perioperative outcomes, and adverse events were characterized. RESULTS: Over a 6-year period, 37 patients underwent 202 general anesthetics. Most patients (75.7%) had dystrophic EB (DEB). Female patients comprised 48.6%. The majority (56.7%) traveled >50 miles to receive care, and many (35.1%) traveled >150 miles for their care. Common adaptations to care included avoidance of electrocardiogram leads (88.6%) and temperature probes (91.6%). Nasal fiberoptic intubation (n = 160) was performed, or natural airway/mask (n = 27) was maintained for most patients. Supraglottic devices were not used for airway management during any of the anesthetics. Anesthesia preparation time was longer (average 25.8 minutes [standard deviation {SD} = 12.7]) than our average institutional time (14 minutes). Succinylcholine was never used, and nondepolarizing muscle relaxants were used in only 1.5% of patient encounters. Blood was transfused in 16.3% of cases and iron infused in 24.8%. Average length of stay in the postanesthesia care unit was comparable to our institutional average (average 40.1 [SD = 28.6] vs 39 minutes). New skin or mucosal injury occurred in 8 encounters (4%), and desaturation occurred in 43 cases (21.3%). There were no major adverse events. CONCLUSIONS: By using a specialized team and a standardized clinical care pathway, our institution was able to minimize adverse events caused by the anesthetic and surgical care of patients with EB. We recommend natural airway or nasal fiberoptic airway management, meticulous avoidance of shear stress on the skin, and a multidisciplinary approach to care. Supportive therapy such as perioperative blood transfusions and iron infusions are feasible for the treatment of chronic anemia in this population.

Enhanced recovery after surgery and anesthetic outcomes in pediatric reconstructive urologic surgery.

PURPOSE: Enhanced recovery after surgery (ERAS) is a perioperative management strategy to hasten postoperative recovery. We examined the effects of a pilot implementation of ERAS for pediatric patients on anesthetic outcomes. METHODS: We performed a prospective case-control study utilizing an ERAS protocol in patients aged < 18 years undergoing urologic reconstruction that included a bowel anastomosis. Protocol elements included: multimodal analgesia, opioid minimization, and routine nausea/vomiting prophylaxis. ERAS patients were propensity-matched with historical controls. Outcomes of interest included maximum PACU pain score, time to first opioid, opioid-free days, and need for opioids on day of discharge. RESULTS: A total of 13 ERAS patients and 26 historical controls were included, with median ages 9.9 years (IQR 9.1-11) and 10.4 years (IQR 8.0-12.4), respectively. ERAS increased the percentage of patients who did not receive any intraoperative or postoperative opioids (0% vs 15%, p = 0.046 for both) and reduced maximum PACU pain score (3 vs 0, p < 0.001). The use of postoperative supplemental oxygen was decreased in the ERAS group (85% vs 38%, p = 0.013). CONCLUSIONS: The implementation of an ERAS protocol appears to decrease postoperative pain, opioid usage, and positively impact other anesthetic outcomes in children undergoing urologic reconstructive surgery utilizing a bowel anastomosis.

Consensus Guidelines for Perioperative Care in Neonatal Intestinal Surgery: Enhanced Recovery After Surgery (ERAS®) Society Recommendations.

BACKGROUND: Enhanced Recovery After Surgery (ERAS®) Society guidelines integrate evidence-based practices into multimodal care pathways that have improved outcomes in multiple adult surgical specialties. There are currently no pediatric ERAS® Society guidelines. We created an ERAS® guideline designed to enhance quality of care in neonatal intestinal resection surgery. METHODS: A multidisciplinary guideline generation group defined the scope, population, and guideline topics. Systematic reviews were supplemented by targeted searching and expert identification to identify 3514 publications that were screened to develop and support recommendations. Final recommendations were determined through consensus and were assessed for evidence quality and recommendation strength. Parental input was attained throughout the process. RESULTS: Final recommendations ranged from communication strategies to antibiotic use. Topics with poor-quality and conflicting evidence were eliminated. Several recommendations were combined. The quality of supporting evidence was variable. Seventeen final recommendations are included in the proposed guideline. DISCUSSION: We have developed a comprehensive, evidence-based ERAS guideline for neonates undergoing intestinal resection surgery. This guideline, and its creation process, provides a foundation for future ERAS guideline development and can ultimately lead to improved perioperative care across a variety of pediatric surgical specialties.

The role of preoperative instructions in parents' understanding of preoperative fasting for outpatient pediatric urology procedures.

PURPOSE: To assess the impact of modifications in preoperative instructions on parental understanding of preoperative fasting guidelines. METHODS: A prospective postoperative parental survey was conducted to assess parental understanding of preoperative fasting requirements in patients undergoing surgery before and after institution of instructions that included visual aids. Data regarding demographics, procedure type, and time to surgery from preoperative visit were also captured. Survey data were compared between pre- and post-intervention groups using Chi-squared tests for categorical variables and Wilcoxon rank sum test for continuous variables. RESULTS: 173 parents in the pre-intervention group and 162 parents in the post-intervention group were included in the analysis. Parent identification of aspiration risk as the reason for fasting almost doubled after intervention (72.2% vs. 38.2%). There was some evidence of demographic differences between groups; however, in an adjusted model, there was strong evidence (p < 0.001) that parents in the post-intervention group were more likely to identify aspiration as the reason for preoperative fasting (OR 4.73; 95% CI 2.93-7.63). CONCLUSIONS: Addition of visual aids in preoperative instructions was associated with improvement in parents' understanding of the rationale behind preoperative fasting instructions. Further studies are needed to determine whether improved understanding is associated with improved adherence.

Enhanced recovery after surgery in children: Promising, evidence-based multidisciplinary care.

Enhanced recovery after surgery (ERAS) is a multimodal approach to the care of the surgical patient focused on reducing the stress response and associated physiologic changes that accompany surgery. Over the past 20 years, ERAS programs have been found to result in reduced LOS and complications in adult patients. Despite abundant adult literature describing implementation and outcomes of enhanced recovery programs, pediatric data in this area is sparse. This educational review describes the history and elements of ERAS protocols, reviews the available evidence in adult and pediatric populations, compares and contrasts ERAS with the PSH, and offers strategies for implementation and ideas for future directions of ERAS in children.

Feasibility of Enhanced Recovery After Surgery (ERAS) implementation in Pediatric Urology: Pilot-phase outcomes of a prospective, multi-center study.

INTRODUCTION/BACKGROUND: Enhanced Recovery After Surgery (ERAS) is a fundamental shift in perioperative care that has consistently demonstrated an improved outcome for a wide variety of surgeries in adults but has only limited evidence in the pediatric population. OBJECTIVE: We aimed to assess the success with and barriers to implementation of ERAS in a prospective, multi-center study on patients undergoing complex lower urinary tract reconstruction. STUDY DESIGN: Centers were directed to implement an ERAS protocol using a multidisciplinary team and quality improvement methodologies. Providers completed pre- and post-pilot surveys. An audit committee met after enrolling the first 5 patients at each center. Pilot-phase outcomes included enrollment of ≥2 patients in the first 6 months of enrollment, completion of 90 days of follow-up, identification of barriers to implementation, and protocol adherence. RESULTS: A total of 40 patients were enrolled across 8 centers. The median age at surgery was 10.3 years (IQR 6.4-12.5). Sixty five percent had a diagnosis of myelomeningocele, and 33 % had a ventriculoperitoneal shunt. A bladder augmentation was performed in 70 %, Mitrofanoff appendicovesicostomy in 52 %, Monti ileovesicostomy in 15 %, and antegrade continence enema channel in 38 %. The most commonly perceived barriers to implementation on the pre-pilot survey were "difficulty initiating and maintaining compliance with care pathway" in 51 % followed by a "lack of time, money, or clinical resources" in 36 %. The pre-pilot study experience, implementation, and pilot-phase outcomes are provided in the Table. All primary and secondary outcomes were achieved. DISCUSSION: The findings of the present study were similar to several small comparative studies with regard to the importance of a multidisciplinary team, strong leadership, and continuous audit for successful implementation of ERAS. Similar barriers were also encountered to other studies, which primarily related to a lack of administrative support, leadership, and buy-in from other services. The limitations of the present study included a relatively small heterogeneous cohort and absence of a comparative group, which will be addressed in the larger exploratory phase of the trial. The findings may also not be generaziable due to the need for sustainable processes that were unique to each center as well as an absence of adequate volume or resources at smaller centers. CONCLUSIONS: ERAS was successfully implemented for complex lower urinary tract reconstruction across 8 centers through a multidisciplinary team, structured approach based on the local context, and focus on a continuous audit.

Key Strategies for Optimizing Pediatric Perioperative Nutrition-Insight from a Multidisciplinary Expert Panel.

Adequate nutrition is an essential factor in healing and immune support in pediatric patients undergoing surgery, but its importance in this setting is not consistently recognized. Standardized institutional nutrition protocols are rarely available, and some clinicians may be unaware of the importance of assessing and optimizing nutritional status. Moreover, some clinicians may be unaware of updated recommendations that call for limited perioperative fasting. Enhanced recovery protocols have been used in adult patients undergoing surgery to ensure consistent attention to nutrition and other support strategies in adult patients before and after surgery, and these are now under evaluation for use in pediatric patients as well. To support better adoption of ideal nutrition delivery, a multidisciplinary panel of experts in the fields of pediatric anesthesiology, surgery, gastroenterology, cardiology, nutrition, and research have gathered and reviewed current evidence and best practices to support nutrition goals in this setting.

Evaluating the role for regional analgesia in children with spina bifida: a retrospective observational study comparing the efficacy of regional versus systemic analgesia protocols following major urological surgery.

INTRODUCTION: Regional techniques are a key component of multimodal analgesia and help decrease opioid use perioperatively, but some techniques may not be suitable for all patients, such as those with spina bifida. We hypothesized peripheral regional catheters would reduce postoperative opioid use compared with no regional analgesia without increasing pain scores in pediatric patients with spina bifida undergoing major urological surgery. METHODS: A retrospective review of a multicenter database established for the study of enhanced recovery after surgery was performed of patients from 2009 to 2021 who underwent bladder augmentation or creation of catheterizable channels. Patients without spina bifida and those receiving epidural analgesia were excluded. Opioids were converted into morphine equivalents and normalized to patient weight. RESULTS: 158 patients with pediatric spina bifida from 7 centers were included, including 87 with and 71 without regional catheters. There were no differences in baseline patient factors. Anesthesia setup increased from median 40 min (IQR 34-51) for no regional to 64 min (IQR 40-97) for regional catheters (p<0.01). The regional catheter group had lower median intraoperative opioid usage (0.24 vs 0.80 mg/kg morphine equivalents, p<0.01) as well as lower in-hospital postoperative opioid usage (0.05 vs 0.23 mg/kg/day morphine equivalents, p<0.01). Pain scores were not higher in the regional catheters group. DISCUSSION: Continuous regional analgesia following major urological surgery in children with spina bifida was associated with a 70% intraoperative and 78% postoperative reduction in opioids without higher pain scores. This approach should be considered for similar surgical interventions in this population. TRIAL REGISTRATION NUMBER: NCT03245242.

Simulation-based assessment of pediatric anesthesia skills.

BACKGROUND: Assessment of pediatric anesthesia trainees is complicated by the random nature of adverse patient events and the vagaries of clinical exposure. However, assessment is critical to improve patient safety. In previous studies, a multiple scenario assessment provided reliable and valid measures of the abilities of anesthesia residents. The purpose of this study was to develop a set of relevant simulated pediatric perioperative scenarios and to determine their effectiveness in the assessment of anesthesia residents and pediatric anesthesia fellows. METHODS: Ten simulation scenarios were designed to reflect situations encountered in perioperative pediatric anesthesia care. Anesthesiology residents and fellows consented to participate and were debriefed after each scenario. Two pediatric anesthesiologists scored each scenario by key action checklist. The psychometric properties (reliability, validity) of the scores were studied. RESULTS: Thirty-five anesthesiology residents and pediatric anesthesia fellows participated. The participants with greater experience administering pediatric anesthetics generally outperformed those with less experience. Score variance attributable to raters was low, yielding a high interrater reliability. CONCLUSIONS: A multiple-scenario, simulation-based assessment of pediatric perioperative care was designed and administered to residents and fellows. The scores obtained from the assessment indicated the content was relevant and that raters could reliably score the scenarios. Participants with more training achieved higher scores, but there was a wide range of ability among subjects. This method has the potential to contribute to pediatric anesthesia performance assessment, but additional measures of validity including correlations with more direct measures of clinical performance are needed to establish the utility of this approach.

A Graceful EXIT impeded by obstetrical complications.

We report an ex utero intrapartum therapy-to-airway procedure in which obstetric factors dramatically influenced the sequence of events necessary to complete the procedure.

Opioid prescribing patterns following implementation of Enhanced Recovery After Surgery (ERAS) protocol in pediatric patients undergoing lower tract urologic reconstruction.

BACKGROUND: With increasing awareness of the opioid epidemic, there is a push for providers to minimize opioid prescriptions. Enhanced Recovery After Surgery (ERAS) is a comprehensive multidisciplinary perioperative protocol that includes minimization of opioid analgesia in favor of non-opioid alternatives and regional analgesia. While ERAS protocols have consistently been shown to decrease inpatient opioid utilization, the impact on opioid prescribing practices and use after discharge in pediatric surgical patients is unclear. OBJECTIVE: This study aims to assess the impact of an ERAS protocol on outpatient opioid prescription patterns after pediatric lower urinary tract reconstructive surgery. We hypothesize that implementation of an ERAS protocol leads to fewer outpatient opioid prescriptions as measured by number and total quantity of oral morphine milligram equivalents by body weight per patient. METHODS: All patients who underwent bladder augmentation, creation of a continent catheterizable channel, bladder neck reconstruction or closure, or revision of prior reconstructive procedures at our tertiary care facility between 2011 and 2017 were reviewed. Patients were divided into pre-ERAS and ERAS cohorts based on whether surgery occurred before or after ERAS implementation. The Colorado Prescription Drug Monitoring Program was used to track filling of postoperative opioid prescriptions for patients covered by the database. RESULTS: A total of 167 urologic reconstructive surgeries were analyzed, including 83 before ERAS and 84 after ERAS implementation. Patients in the ERAS cohort received and filled more outpatient opioid prescriptions at time of discharge (82.6% historical vs 93.9% ERAS, p = 0.015; 76.1% vs 57.9%, p = 0.012). There were no differences in prescription total morphine milligram equivalents normalized to body mass, total days supplied, or 90-day opioid prescription refill rates. DISCUSSION: We found an unexpected increase in postoperative outpatient opioid prescriptions following implementation of an ERAS protocol for lower urinary tract reconstructive surgery. Possible reasons include worry about pain crisis at home in the setting of decreased hospital length of stay in the ERAS cohort or generalized upward drift in opioid prescribing patterns over time. ERAS protocols in other subspecialties reveal mixed findings but consistently suggest standardization of outpatient opioid prescribing patterns leads to a decrease in opioid prescriptions. CONCLUSIONS: Patients received more, not fewer, outpatient opioid prescriptions following major urologic reconstructive surgery after implementation of an ERAS protocol. Purposeful efforts should be made to standardize opioid prescriptions at discharge based on meaningful clinical criteria.

Implementation and sustainability of an enhanced recovery pathway in pediatric bladder reconstruction: Flexibility, commitment, teamwork.

INTRODUCTION: Although enhanced recovery pathways (ERP) provide a safe and effective way to improve the recovery of children undergoing bladder reconstruction, ERPs have not been widely adopted in pediatric urology. We describe a quality improvement initiative and outcomes after implementing a 24-element ERP at a single, freestanding children's hospital. STUDY DESIGN: Multiple stakeholder meetings were planned and executed, initially with pediatric practitioners with ERP experience to understand potential implementation barriers then with anesthesiologists, nurses, case managers, and other ancillary staff to draft our institution-specific ERP. A standardized order set was generated to improve ERP adherence. ERP adherence audits and cyclic performance evaluations held every 6-9 months facilitated continuous pathway refinement. Patient outcomes were compared with a pre-ERP historic cohort. RESULTS: Time from initial ERP planning to first implementation was 7 months. ERP was implemented in twenty consecutive patients undergoing bladder reconstruction (median age 11.3 years, range 4.1-21.1) who were compared to twenty consecutive pre-ERP patients (median age 11.4 years, range 7.7-25.1). Median post-operative length of stay (LOS) significantly decreased from 9 days (range 2-31) pre-ERP to 4 days (range 3-29) post-ERP (p < 0.05). A median of 16 (range 12-19) of 24 institutional pathway elements were implemented for each patient. Balancing measures showed no significant increases in highest Clavien complication grade, readmission rate, or unplanned return to the operating room within 30 post-operative days. DISCUSSION: Implementation of ERP is feasible but requires commitment from multi-disciplinary stakeholders. While we were unable to consistently achieve 80% of the elements, we successfully implemented the pathway and improved our patients' recovery processes (indirectly reflected by a decreased post-operative LOS) with adherence to a median of 67% of elements. Our implementation and effectiveness results are specific to our center and may not be generalizable. However, our experience may offer some insight for others interested in ERP implementation and encourage initiation of their own institutional pathways. CONCLUSION: Successful ERP implementation at our hospital for children undergoing bladder reconstruction was facilitated by open communication, early stakeholder involvement, and monitoring ERP adherence. ERP implementation significantly decreased LOS without increasing post-operative complications and readmissions (Summary figure).

Design and development of the Pediatric Urology Recovery After Surgery Endeavor (PURSUE) multicentre pilot and exploratory study.

INTRODUCTION: Lower urinary tract reconstruction in paediatric urology represents a physiologically stressful event that is associated with high complication rates, including readmissions and emergency room visits. Enhanced recovery after surgery (ERAS) protocol is a set of multidisciplinary, perioperative strategies designed to expedite surgical recovery without adversely impacting readmission or reoperation rates. Early paediatric urology data demonstrated ERAS reduced complications in this population. METHODS AND ANALYSIS: In 2016, a working group of paediatric urologists and anaesthesiologists convened to develop an ERAS protocol suitable for patients undergoing lower urinary tract reconstruction and define study process measures, patient-reported outcomes and clinically relevant outcomes in paediatric and adolescent/young adult patients. A multicentre, prospective, propensity-matched, case-control study design was chosen. Each centre will enrol five pilot patients to verify implementation. Subsequent enrolled patients will be propensity matched to historical controls. Eligible patients must be aged 4-25 years and undergoing planned operations (bladder augmentation, continent ileovesicostomy or appendicovesicostomy, or urinary diversion). 64 ERAS patients and 128 controls will be needed to detect a decrease in mean length of stay by 2 days. Pilot phase outcomes include attainment of ≥70% mean protocol adherence per patient and reasons for protocol deviations. Exploratory phase primary outcome is ERAS protocol adherence, with secondary outcomes including length of stay, readmissions, reoperations, emergency room visits, 90-day complications, pain scores, opioid usage and differences in Quality of Recovery 9 scores. ETHICS AND DISSEMINATION: This study has been registered with authors' respective institution review boards and will be published in peer-reviewed journals. It will provide robust insight into the feasibility of ERAS in paediatric urology, determine patient outcomes and allow for iteration of ERAS implementations as new best practices and evidence for paediatric surgical care arise. We anticipate this study will take 4 years to fully accrue with completed follow-up. TRIAL REGISTRATION NUMBER: NCT03245242; Pre-results.

Anesthesia in the Pediatric Patient.

Improvements in anesthetic drugs and monitoring techniques over the past several decades have significantly reduced the anesthetic risks for pediatric patients. Neonates and infants are at increased risk for cardiovascular and pulmonary complications, and recent reports have led to concern that these young patients may be at risk for long-term detrimental neurodevelopmental effects as well. Although studies are currently under way to answer the question of anesthetic neurotoxicity in children, surgeons and anesthesiologists must work with parents to determine the best course of action for these vulnerable patients.

Anesthetic Considerations for a Child With Rare B3GALT6 Mutations: A Case Report.

A rare autosomal recessive disorder caused by mutations in the B3GALT6 gene on chromosome 1p36 results in deficiency of ß-1,3-galactosyltransferase 6, an enzyme critical for glycosaminoglycan biosynthesis. Defects in this gene result in a phenotype that has features of both skeletal dysplasia and a connective tissue disorder. The anesthetic considerations for children with this disorder have not previously been described. We report a collaborative, multidisciplinary approach to the perioperative care of a child with B3GALT6 mutations with severe phenotypic expression.

Creation of an Enhanced Recovery After Surgery (ERAS) Guideline for neonatal intestinal surgery patients: a knowledge synthesis and consensus generation approach and protocol study.

INTRODUCTION: Enhanced Recovery After Surgery (ERAS) guidelines integrate evidence-based practices into multimodal care pathways designed to optimise patient recovery following surgery. The objective of this project is to create an ERAS protocol for neonatal abdominal surgery. The protocol will identify and attempt to bridge the gaps between current practices and best evidence. Our study is the first paediatric ERAS protocol endorsed by the International ERAS Society. METHODS: A research team consisting of international clinical and family stakeholders as well as methodological experts have iteratively defined the scope of the protocol in addition to individual topic areas. A modified Delphi method was used to reach consensus. The second phase will include a series of knowledge syntheses involving a rapid review coupled with expert opinion. Potential protocol elements supported by synthesised evidence will be identified. The Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) system will be used to determine strength of recommendations and the quality of evidence. The third phase will involve creation of the protocol using a modified RAND/UCLA Appropriateness Method. Group consensus will be used to rate each element in relation to the quality of evidence supporting the recommendation and the appropriateness for guideline inclusion. This protocol will form the basis of a future implementation study. ETHICS AND DISSEMINATION: This study has been registered with the ERAS Society. Human ethics approval (REB 18-0579) is in place to engage patient families within protocol development. This research is to be published in peer-reviewed journals and will form the care standard for neonatal intestinal surgery.

Utilization of a Text and Translation Application for Communication With a Foreign Deaf Family: A Call for Validation of This Technology-A Case Report.

Effective communication with patients is essential to quality care. Obviously, language barriers significantly impact this and can increase the risk of poor patient outcomes. Smartphones and mobile health technology are valuable resources that are beginning to break down language barriers in health care. We present a case of a challenging language barrier where successful perioperative communication was achieved using mobile technology. Although quite beneficial, use of technology that is not validated exposes providers to unnecessary medicolegal risk. We hope to highlight the need for validation of such technology to ensure that these tools are an effective way to accurately communicate with patients in the perioperative setting.