Safety and efficacy of a propofol and ketamine based procedural sedation protocol in children with cerebral palsy undergoing botulinum toxin A injections.

Background Pediatric patients with cerebral palsy (CP) often undergo intramuscular botulinum toxin (BoNT‐A) injections. These injections can be painful and may require procedural sedation. An ideal sedation protocol has yet to be elucidated. Objective To investigate the safety and efficacy of a propofol and ketamine based sedation protocol in pediatric patients with cerebral palsy requiring BoNT‐A injections. Design This is a retrospective chart review of children with CP undergoing propofol and ketamine based sedation for injections with botulinum toxin A. Setting The sedations took place in a procedural sedation suite at a tertiary children’s hospital from Feb 2013 through Sept 2017. Patients 164 patients with diagnoses of cerebral palsy were included in this study. Methods An initial bolus of 0.5 mg/kg ketamine followed by a 2 mg/kg bolus of propofol was administered with supplemental boluses of propofol as needed to achieve deep sedation during the intramuscular BoNT‐A injections. Main Outcome Measurements Propofol dosages, adverse events, serious adverse events, and sedation time parameters were reviewed. Results 345 sedations were successfully performed on 164 patients. The median total dose of propofol was 4.7 mg/kg (IQR 3.5, 6.3). Adverse events were encountered in 10.1% of procedures including hypoxemia responsive to supplemental oxygen (9.6%) and transient apnea (1.4%). The mean procedure time, recovery time and total sedation time were 10, 11 and 33 minutes, respectively. With regard to patient variables, including age, weight, dose of propofol, sedation time, and Gross Motor Function Classification System classification, there was no association with increased incidence of adverse events. Conclusion Our sedation protocol of propofol and ketamine is safe and effective in children with cerebral palsy undergoing procedural sedation for intramuscular injections with BoNT‐A. The adverse events encountered appeared to be related to airway and respiratory complications secondary to musculoskeletal deformities, emphasizing the importance of airway monitoring and management in these patients

Fat Embolism Syndrome in Duchenne Muscular Dystrophy Patients: Early Recognition and Aggressive Therapy

We describe two pediatric patients with Duchenne muscular dystrophy that presented with acute neurologic deterioration and hypoxic respiratory failure requiring mechanical ventilation. These cases fulfill the clinical criteria for Fat Embolism Syndrome. Early recognition and aggressive supportive therapy with mechanical ventilation, right ventricular afterload reduction, and blood transfusion led to survival without any residual effects from the event. Fat Embolism Syndrome needs to be considered early in the course of patients with Duchenne muscular dystrophy who present with respiratory and neurological symptoms

Food Protein-Induced Enterocolitis Syndrome Causing Hypovolemic Shock and Methemoglobinemia

A 5-week-old previously healthy male presented with vomiting and diarrhea leading to hypovolemic shock and profound metabolic acidosis. He was subsequently found to have severe methemoglobinemia. The acidosis and shock improved with fluid resuscitation and methemoglobinemia was successfully treated with methylene blue. An extensive workup, including evaluations for infectious and metabolic etiologies, was unremarkable. However, a detailed dietary history revealed a recent change in diet, supporting a diagnosis of food protein-induced enterocolitis syndrome (FPIES). We present this case to highlight the importance of considering FPIES in an infant with vomiting and diarrhea, in the setting of a recent dietary change, leading to profound dehydration, metabolic acidosis, and methemoglobinemia. Diagnosis of FPIES, although difficult to make and one of exclusion, can be potentially life-saving

Brain Death Secondary to Rocky Mountain Spotted Fever Encephalitis

A two-year-old female presented with acutely altered mental status following eight days of fever and rash. She had been camping at an Indiana campground 11 days prior to the onset of illness and was evaluated twice for her fever and rash prior to admission. Laboratory evaluation on admission revealed thrombocytopenia, hyponatremia, and elevated transaminases. The patient developed diffuse cerebral edema, and despite intensive care, the edema led to brain death from Rocky Mountain spotted fever (RMSF). We present this case to highlight the importance of considering RMSF and other tick-borne illnesses in a child with prolonged fever and rash in a nonendemic area and also the difficulty of diagnosis in early stages of disease. A detailed travel history, evaluation of key laboratory findings (white blood count, platelet count, and transaminases), and close follow-up if rash and fevers persist may help to improve detection of RMSF. If a tick-borne illness such as RMSF is suspected, empiric doxycycline therapy should be started immediately, as lab confirmation may take several days and mortality increases greatly after five days of symptoms

Comparison between chloral hydrate and propofol-ketamine as sedation regimens for pediatric auditory brainstem response testing

Introduction The use of diagnostic auditory brainstem response testing under sedation is currently the “gold standard” in infants and young children who are not developmentally capable of completing the test. Objective The aim of the study is to compare a propofol-ketamine regimen to an oral chloral hydrate regimen for sedating children undergoing auditory brainstem response testing. Methods Patients between 4 months and 6 years who required sedation for auditory brainstem response testing were included in this retrospective study. Drugs doses, adverse effects, sedation times, and the effectiveness of the sedative regimens were reviewed. Results 73 patients underwent oral chloral hydrate sedation, while 117 received propofol-ketamine sedation. 12% of the patients in the chloral hydrate group failed to achieve desired sedation level. The average procedure, recovery and total nursing times were significantly lower in the propofol-ketamine group. Propofol-ketamine group experienced higher incidence of transient hypoxemia. Conclusion Both sedation regimens can be successfully used for sedating children undergoing auditory brainstem response testing. While deep sedation using propofol-ketamine regimen offers more efficiency than moderate sedation using chloral hydrate, it does carry a higher incidence of transient hypoxemia, which warrants the use of a highly skilled team trained in pediatric cardio-respiratory monitoring and airway management

A Collaborative In Situ Simulation-based Pediatric Readiness Improvement Program for Community Emergency Departments

Background More than 30 million children are cared for across 5,000 U.S. emergency departments (EDs) each year. Most of these EDs are not facilities designed and operated solely for children. A Web-based survey provided a national and state-by-state assessment of pediatric readiness and noted a national average score was 69 on a 100-point scale. This survey noted wide variations in ED readiness with scores ranging from 61 in low-pediatric-volume EDs to 90 in the high-pediatric-volume EDs. Additionally, the mean score at the state level ranged from 57 (Wyoming) to 83 (Florida) and for individual EDs ranged from 22 to 100. The majority of prior efforts made to improve pediatric readiness have involved providing Web-based resources and online toolkits. This article reports on the first year of a program that aimed to improve pediatric readiness across community hospitals in our state through in situ simulation-based assessment facilitated by our academic medical center. The primary aim was to improve the pediatric readiness scores in the 10 participating hospitals. The secondary aim was to explore the correlation of simulation-based performance of hospital teams with pediatric readiness scores. Methods This interventional study measured the Pediatric Readiness Survey (PRS) prior to and after implementation of an improvement program. This program consisted of three components: 1) in situ simulations, 2) report-outs, and 3) access to online pediatric readiness resources and content experts. The simulations were conducted in situ (in the ED resuscitation bay) by multiprofessional teams of doctors, nurses, respiratory therapists, and technicians. Simulations and debriefings were facilitated by an expert team from a pediatric academic medical center. Three scenarios were conducted for all teams and include: a 6-month-old with respiratory failure, an 8-year-old with diabetic ketoacidosis (DKA), and a 6-month-old with supraventricular tachycardia (SVT). A performance score was calculated for each scenario. The improvement of PRS was compared before and after the simulation program. The correlation of the simulation performance of each hospital and the PRS was calculated. Results Forty-one multiprofessional teams from 10 EDs in Indiana participated in the study, five were of medium pediatric volume and five were medium- to high-volume EDs. The PRS significantly improved from the first to the second on-site verification assessment (58.4 ± 4.8 to 74.7 ± 2.9, p = 0.009). Total adherence scores to scenario guidelines were 54.7, 56.4, and 62.4% in the respiratory failure, DKA, and SVT scenarios, respectively. We found no correlation between simulation performance and PRS scores. Medium ED pediatric volume significantly predicted higher PRS scores compared to medium-high pediatric ED volume (β = 8.7; confidence interval = 0.72–16.8, p = 0.034). Conclusions Our collaborative improvement program that involved simulation was associated with improvement in pediatric readiness scores in 10 EDs participating statewide. Future work will focus on further expanding of the network and establishing a national model for pediatric readiness improvement

Practice Variation in the Immediate Postoperative Care of Pediatric Kidney Transplantation: A National Survey

Introduction Advances in organ allocation, surgical technique, immunosuppression, and long-term follow-up have led to a significant improvement in kidney transplant outcomes. Although there are clear recommendations for several aspects of kidney transplant management, there are no pediatric-specific guidelines for immediate postoperative care. The aim of this survey is to examine practice variations in the immediate postoperative care of pediatric kidney transplant patients. Methods We surveyed medical directors of Pediatric Acute Lung Injury and Sepsis Investigators (PALISI)-affiliated pediatric intensive care units regarding center-specific immediate postoperative management of pediatric kidney transplantation. Results The majority of PALISI centers admit patients to the pediatric intensive care unit postoperatively, and 97% of the centers involve a pediatric nephrologist in immediate postoperative care. Most patients undergo invasive hemodynamic monitoring; 97% of centers monitor invasive arterial blood pressure and 88% monitor central venous pressure. Most centers monitor serum electrolytes every 4 to 6 hours. Wide variation exists regarding blood pressure goal, fluid replacement type, frequency of obtaining kidney ultrasound, and use of prophylactic anticoagulation. Conclusion There is consistent practice across PALISI centers in regards to many aspects of immediate postoperative management of pediatric kidney transplantation. However, variation still exists in some management aspects that warrant further discussions to reach a national consensus

Intensivist-based deep sedation using propofol for pediatric outpatient flexible bronchoscopy

AIM To evaluate the safety and efficacy of sedating pediatric patients for outpatient flexible bronchoscopy. METHODS A retrospective chart review was conducted for all children, age 17 years or under who underwent flexible bronchoscopy under deep sedation in an outpatient hospital-based setting. Two sedation regimens were used; propofol only or ketamine prior to propofol. Patients were divided into three age groups; infants (less than 12 mo), toddlers (1-3 years) and children (4-17 years). Demographics, indication for bronchoscopy, sedative dosing, sedation and recovery time and adverse events were reviewed. RESULTS Of the total 458 bronchoscopies performed, propofol only regimen was used in 337 (74%) while propofol and ketamine was used in 121 (26%). About 99% of the procedures were successfully completed. Children in the propofol + ketamine group tend to be younger and have lower weight compared to the propofol only group. Adverse events including transient hypoxemia and hypotension occurred in 8% and 24% respectively. Median procedure time was 10 min while the median discharge time was 35 min. There were no differences in the indication of the procedure, propofol dose, procedure or recovery time in either sedative regimen. When compared to other age groups, infants had a higher incidence of hypoxemia. CONCLUSION Children can be effectively sedated for outpatient flexible bronchoscopy with high rate of success. This procedure should be performed under vigilance of highly trained providers

Survival outcomes of in-hospital cardiac arrest in pediatric patients in the USA

We report on in-hospital cardiac arrest outcomes in the USA. The data were obtained from the National (Nationwide) Inpatient Sample datasets for the years 2000–2017, which includes data from participating hospitals in 47 US states and the District of Columbia. We included pediatric patients (< 18 years of age) with cardiac arrest, and we excluded patients with no cardiopulmonary resuscitation during the hospitalization. Primary outcome of the study was in-hospital mortality after cardiac arrest. A multivariable logistic regression was performed to identify factors associated with survival. A total of 20,654 patients were identified, and 8226 (39.82%) patients survived to discharge. The median length of stay and cost of hospitalization were significantly higher in the survivors vs. non-survivors (LOS 18 days vs. 1 day, and cost $187,434 vs.$45,811, respectively, p < 0.001). In a multivariable model, patients admitted to teaching hospitals, elective admissions, and those admitted on weekdays had higher survival (aOR=1.19, CI: 1.06–1.33; aOR=2.65, CI: 2.37–2.97; and aOR=1.17, CI: 1.07–1.27, respectively). There was no difference in mortality between patients with extracorporeal cardiopulmonary resuscitation (E-CPR) and those with conventional cardiopulmonary resuscitation. E-CPR patients were likely to have congenital heart surgery (51.0% vs. 20.8%). Conclusion: We highlighted the survival predictors in these events, which can guide future studies aimed at improving outcomes in pediatric cardiac arrest

Acute Cytotoxic Cerebellar Edema Subsequent to Fentanyl Patch Intoxication in an Infant

The opioid epidemic continues to have devastating consequences for children and families across the United States with rising prevalence of opioid use and abuse. Given the ease of access to these medications, accidental ingestion and overdose by children are becoming increasingly more common. The recognition of opioid-induced neurotoxicity and the associated life-threatening complication of acute cerebellar cytotoxic edema are crucial, as are the high morbidity and mortality without timely intervention. We discuss an infant with acute cytotoxic cerebellar edema following mucosal exposure to a transdermal fentanyl patch