A retrospective observational cohort study of the anesthetic management and outcomes of pediatric patients with Alexander disease undergoing lumbar puncture or magnetic resonance imaging.

BACKGROUND: Alexander disease is a rare, progressive leukodystrophy, which predisposes patients to complications under general anesthesia due to clinical manifestations including developmental delay, seizures, dysphagia, vomiting, and sleep apnea. However, study of anesthetic outcomes is limited. AIMS: Our aim was to describe patient characteristics, anesthetic techniques, and anesthesia-related complications for Alexander disease patients undergoing magnetic resonance imaging and/or lumbar puncture at a quaternary-care children's hospital. METHODS: We performed a retrospective review of anesthetic outcomes in patients with Alexander disease enrolled in a prospective observational study. Included patients had diagnosed Alexander disease and underwent magnetic resonance imaging and/or lumbar puncture at our institution. We excluded anesthetics for other procedures or at outside institutions. Collected data included patient characteristics, anesthetic techniques, medications, and complications under anesthesia and in the subsequent 24 h. We performed descriptive statistics as appropriate. RESULTS: Forty patients undergoing 64 procedures met inclusion criteria. Fifty-six procedures (87.5%) required general anesthesia or monitored anesthesia care (MAC) and eight (12.5%) did not. The general anesthesia/MAC group tended to be younger than nonanesthetized patients (median age 6 years [IQR 3.8; 9] vs. 14.5 years [IQR 12.8; 17.5]). In both groups, dysphagia (78.6% vs. 87.5%, respectively), seizures (62.5% vs. 25%), and recurrent vomiting (17.9% vs. 25%) were frequently reported preprocedure symptoms. Inhalational induction was common (N = 48; 85.7%), and two (3.6%) underwent rapid sequence induction. Serious complications were rare, with no aspiration or seizures. Hypotension resolving with ephedrine occurred in eight cases (14.3%). One patient each (1.8%) experienced postprocedure emergence agitation or vomiting. Fifty-three (94.6%) were ambulatory procedures. No inpatients required escalation in acuity of care. CONCLUSIONS: In this single-center study, patients with Alexander disease did not experience frequent or irreversible complications while undergoing general anesthesia/MAC. Co-morbid symptoms were not increased postanesthesia. Some patients may not require anesthesia to complete short procedures.

Creation and Implementation of a Hospitalist-Run Propofol Sedation Program.

BACKGROUND: Pediatric hospitalists increasingly provide sedation outside the operating room. Given the large body of safety data available, propofol was identified as a beneficial addition to our hospitalist-run sedation service's medication repertoire. Currently, the training required for hospitalists to provide sedation is defined and determined locally by individual institutions. METHODS: We convened a task force to develop and implement training for hospitalists in the use of propofol for deep sedation. After implementing training, we analyzed the outcome of patients receiving propofol for deep sedation for MRI, including the adverse event rate and successful completion rate. An adverse event was defined as a significant desaturation, persistent upper airway obstruction, laryngospasm, administration of neuromuscular blockade, conversion to anesthesia, call for additional backup, or if the procedure was not able to be completed. Successful completion was defined as any patient being able to complete the imaging study or procedure with sedation performed by a hospitalist physician. RESULTS: Between September 2015 and September 2018, sedation services sedated 12 979 patients, 3929 of whom were deeply sedated with propofol. During this period, the trained hospitalists had an adverse event rate of 3.6% and a 98.9% rate of successful completion of all studies or procedures when using propofol for deep sedation. CONCLUSION: With a comprehensive training program for hospitalists in the administration of propofol, we provided effective sedation for a selective population of patients. We now have a standard approach that uses credentialed hospitalists to train new faculty for propofol administration.

How do I Incorporate a two-sample blood type verification in a pediatric hospital?

BACKGROUND: The AABB (American Association of Blood Banks) and the College of American Pathologists (CAP) regulations call on blood banks to address the risk of misidentification of a patient's blood type, which can result in transfusion of a mismatched product. Transfusion of mismatched blood product is potentially fatal due to acute hemolytic transfusion reaction and is considered a preventable event. CAP regulations outline options to reduce risk of mistransfusion by either documenting the ABO group of the intended recipient on a second sample collected at a separate phlebotomy, or utilizing a mechanical barrier system or electronic identification verification system that ensures the patient from whom the pretransfusion specimen was collected is the same patient who is about to be transfused. STUDY DESIGN AND METHODS: An electronic or barrier system was not available for implementation at our institution, therefore we developed a protocol for a two-sample verification system. The first determination is performed on a current sample and the second by one of the following methods: (a) comparison with previous laboratory records, (b) testing a second sample collected at a time different from the first sample (i.e., laboratory specimen available with a different timestamp, or a new blood sample). RESULTS: We improved our transfusion process and implemented a policy to require a second sample to confirm a patient's blood type. We also implemented workflows to obtain blood type confirmation from history of a second blood type result from previous laboratory records, including a policy to accept previous blood type records from an outside laboratory. CONCLUSIONS: We describe a practice change for two-sample verification for type and screen in a large-scale pediatric hospital. We outline specific workflows for pre-operative and emergency transfusion scenarios, and pediatric-specific challenges.

A multidisciplinary approach to dosing nusinersen for spinal muscular atrophy.

BACKGROUND: In December 2016, nusinersen gained FDA approval as the first pharmacologic treatment for spinal muscular atrophy (SMA), a disorder of motor neurons and the leading genetic cause of infant mortality. Nusinersen's intrathecal delivery requirement, strict dosage protocol, and accelerated FDA approval presented a challenge to health care centers hoping to implement treatment of patients with SMA. Scheduling logistics, combined with the specific ventilatory, anesthetic, and spinal access needs of this patient population, requires extensive coordination of care. This complexity, in addition to the high cost of treatment, may lead to overburdening of an institution's dosing resources, causing delays in treatment initiation and limiting patients' access to therapy and may result in barriers to coverage. METHODS: We initiated a comprehensive stepwise protocol to maximize patient inclusion, as well as safety and efficiency outcome measures. This retrospective cohort study reviews the dosing process. RESULTS: As a result of immense collaborative efforts involving care coordination of patients and families, in addition to health providers in the divisions of neurology, anesthesiology, pulmonology, orthopedics, interventional radiology, physical therapy, and neurosurgery, we have successfully dosed 62 SMA patients. Throughout this process, we have improved anesthetic techniques, as well as minimized procedural complications and missed scheduled doses. CONCLUSION: We present here recommendations for safe and effective implementation of nusinersen utilizing a multidisciplinary approach, based on our 1 and a half year experience at a tertiary care children's hospital.

STBUR: Sleep trouble breathing and unrefreshed questionnaire: Evaluation of screening tool for postanesthesia care and disposition.

BACKGROUND: The Snoring, Trouble Breathing, and Un-Refreshed (STBUR) questionnaire is a five-question screening tool for pediatric sleep-disordered breathing and risk for perioperative respiratory adverse events in children. The utility of this questionnaire as a preoperative risk-stratification tool has not been investigated. In view of limited availability of screening tools for preoperative pediatric sleep-disordered breathing, we evaluated the questionnaire's performance for postanesthesia adverse events that can impact postanesthesia care and disposition. METHODS: The retrospective study protocol was approved by the institutional research board. The data were analyzed using two different definitions for a positive screening based on a five-point scale: low threshold (scores 1 to 5) and high threshold (score of 5). The primary outcome was based on the following criteria: (a) supplemental oxygen therapy following postanesthesia care unit (PACU) stay until hospital discharge, (b) greater than two hours during phase 1 recovery, (c) anesthesia emergency activation in the PACU, and (d) unplanned hospital admission. RESULTS: About 6025 patients completed the questionnaire during the preoperative evaluation. And 1522 patients had a low threshold score and 270 had a high-threshold score. We found statistically significant associations in three outcomes based on the low threshold score: supplemental oxygen therapy (negative-predictive value [NPV] 0.97, 95% CI 0.97-98), PACU recovery time (NPV 0.99, 95% CI 0.99-0.99) and escalation of care (NPV 0.98, 95% CI 0.97-0.98). Positive-predictive values were statistically significant for all outcomes except anesthesia emergency in the PACU. CONCLUSION: The Snoring, Trouble Breathing, and Un-Refreshed questionnaire identified patients at higher risk for prolonged phase 1 recovery, oxygen therapy requirement, and escalation of care. The questionnaire's high-negative predictive value and specificity may make it useful as a screening tool to identify patients at low risk for prolonged stay in PACU.

A retrospective comparison of thoracic epidural infusion and multimodal analgesia protocol for pain management following the minimally invasive repair of pectus excavatum.

BACKGROUND: Pain management following minimally invasive repair of pectus excavatum is variable. We recently adopted a comprehensive multimodal analgesic protocol that standardizes perioperative analgesic management. We hypothesized that patients managed with this protocol would use more opioids postoperatively, have similar pain control, and shorter length of stay compared to patients managed with thoracic epidural infusion. AIMS: We retrospectively compared opioid consumption, pain scores, and length of stay between a cohort of patients managed with our multimodal analgesic protocol and a cohort managed with a thoracic epidural infusion. METHODS: This retrospective cohort comparison includes patients, 8 to 21 years of age, managed with either thoracic epidural infusion (n = 21) or multimodal analgesic protocol (n = 29) following minimally invasive repair of pectus excavatum from January 1, 2011 through September 15, 2015. The primary outcome, total daily opioid consumption in morphine equivalents, is presented as an average by postoperative day. Secondary outcomes included median daily pain score and length of stay. RESULTS: Patients were similar in age, weight, sex, and physical status. Patients managed with thoracic epidural infusion received less opioid (morphine equivalents-mg/kg) intraoperatively compared to multimodal analgesic protocol (difference of mean [95% confidence interval] 0.22 [0.16-0.28] P ≤ .01) but required more total opioid through postoperative day 3 (difference of mean [95% confidence interval] 1.2 [0.26-2.14] P = .01). We did not observe a difference in pain scores. Median length of stay was 1 day less in patients managed with multimodal analgesic protocol (difference of median [95% confidence interval] 1 [0.3-1.7] P = .003). CONCLUSION: Implementation of a standardized comprehensive multimodal analgesic protocol following minimally invasive repair of pectus excavatum resulted in equivalent analgesia with a modest reduction in length of stay when compared to thoracic epidural. We did not observe an opioid sparing effect in our thoracic epidural which may reflect technique variability.

Do pediatric chief residents recognize details of prepubertal female genital anatomy: a national survey.

OBJECTIVE: To evaluate how well a group of recently trained pediatric chief residents could label anatomic structures on two different photographs of female prepubertal genitalia. Additionally, the study sought to explore aspects of pediatric training in sexual abuse and clinical practice issues surrounding the routine genital examination. METHOD: A 38-item questionnaire was mailed to pediatric chief residents at all of the officially listed pediatric residency-training programs in the continental US. Comparisons were made between this study and the responses to two previous surveys, which asked a more heterogeneous group of physicians to label one of the photographs used in the study. The second photograph was added because of its improved clarity of each anatomic structure when compared to the first photograph used in the previous studies. The study also asked about clinical practice issues surrounding the prepubertal genital examination. RESULTS: An overall response rate of 73% was achieved and analysis was done on 139 respondents. One-half of chief residents thought that their training during residency on sexual abuse was inadequate for practice. Sixty-four percent of chief residents correctly labeled the hymen on the photograph used in the previous studies, which was not significantly different from the 62% and 59% of physicians who correctly labeled the hymen in the previous surveys. In the second photograph, which more clearly displayed the various anatomic structures, 71% correctly labeled the hymen. CONCLUSION: Pediatric chief residents reported variable amounts of training on issues pertaining to child sexual abuse during residency, think that this time was inadequate, and, while doing slightly better than a more diverse group of previously studied physicians, did not achieve 100% accuracy in identifying basic genital structures correctly on two different photographs.