Deep Sedation in Pediatric Patients With Single Ventricle Physiology Outside of the Operating Room.

Background: Advancements in palliative surgery of patients with single ventricle physiology have led to an increase in the need for deep sedation protocols for painful procedures. However, positive pressure ventilation during anesthesia can result in unfavorable cardiopulmonary interactions. This patient population may benefit from sedation from these painful procedures. Methods: This study aims to demonstrate the safety and efficacy of deep sedation by pediatric intensivists outside the operating room for children with single ventricle physiology. This is a single-center, retrospective chart review on consecutive pediatric patients with single ventricle physiology who received deep sedation performed by pediatric intensivists between 2013 and 2020. Results: Thirty-three sedations were performed on 27 unique patients. The median age was 3.7 years (25th%-75th%: 2.1-15.6). The majority of the sedations, 88% (29/33), were done on children with Fontan physiology and 12% (4/33) were status-post superior cavopulmonary anastomosis. The primary cardiac defect was hypoplastic left heart in 63% (17/27) of all sedation procedures. There were 24 chest tube placements and 9 cardioversions. Ketamine alone [median dose 1.5 mg/kg (range 0.8-3.7)], ketamine [median dose 1 mg/kg (range 0.1-2.1)] with propofol [median dose 2.3 mg/kg (range 0.7-3.8)], and ketamine [median dose 1.5 mg/kg (range 0.4-3.0)] with morphine [median dose 0.06 mg/kg (range 0.03-0.20)] were the most common sedation regimens used. Adverse events (AEs) occurred in 4 patients (15%), three of which were transient AEs. All sedation encounters were successfully completed. Conclusion: Procedural deep sedation can be safely and effectively administered to single ventricle patients by intensivist-led sedation teams in selective case.

Pediatric Highlights From the Extracorporeal Life Support Organization Registry: 2017-2022.

The Extracorporeal Life Support Organization (ELSO) registry which collects data from hundreds of participating centers supports research in ECMO to help improve patient outcomes. The ELSO Scientific Oversight Committee, an international and diverse group of ECMOlogists ( https://www.elso.org/registry/socmembers.aspx ), selected the most impactful and innovative research articles on pediatric ECMO emerging from ELSO data. Here they present brief highlights of these publications.

Adult Highlights From the Extracorporeal Life Support Organization Registry: 2017-2022.

The Extracorporeal Life Support Organization (ELSO) registry captures clinical data and outcomes on patients receiving extracorporeal membrane oxygenation (ECMO) support across the globe at participating centers. It provides a very unique opportunity to benchmark outcomes and analyze the clinical course to help identify ways of improving patient outcomes. In this review, we summarize select adult ECMO articles published using the ELSO registry over the past 5 years. These articles highlight innovative utilization of the registry data in generating hypotheses for future clinical trials. Members of the ELSO Scientific Oversight Committee can be found here: https://www.elso.org/registry/socmembers.aspx .

Neurological and Psychological Sequelae Associated With Multisystem Inflammatory Syndrome in Children.

Importance: Acute neurological involvement occurs in some patients with multisystem inflammatory syndrome in children (MIS-C), but few data report neurological and psychological sequelae, and no investigations include direct assessments of cognitive function 6 to 12 months after discharge. Objective: To characterize neurological, psychological, and quality of life sequelae after MIS-C. Design, Setting, and Participants: This cross-sectional cohort study was conducted in the US and Canada. Participants included children with MIS-C diagnosed from November 2020 through November 2021, 6 to 12 months after hospital discharge, and their sibling or community controls, when available. Data analysis was performed from August 2022 to May 2023. Exposure: Diagnosis of MIS-C. Main Outcomes and Measures: A central study site remotely administered a onetime neurological examination and in-depth neuropsychological assessment including measures of cognition, behavior, quality of life, and daily function. Generalized estimating equations, accounting for matching, assessed for group differences. Results: Sixty-four patients with MIS-C (mean [SD] age, 11.5 [3.9] years; 20 girls [31%]) and 44 control participants (mean [SD] age, 12.6 [3.7] years; 20 girls [45%]) were enrolled. The MIS-C group exhibited abnormalities on neurological examination more frequently than controls (15 of 61 children [25%] vs 3 of 43 children [7%]; odds ratio, 4.7; 95% CI, 1.3-16.7). Although the 2 groups performed similarly on most cognitive measures, the MIS-C group scored lower on the National Institutes of Health Cognition Toolbox List Sort Working Memory Test, a measure of executive functioning (mean [SD] scores, 96.1 [14.3] vs 103.1 [10.5]). Parents reported worse psychological outcomes in cases compared with controls, particularly higher scores for depression symptoms (mean [SD] scores, 52.6 [13.1] vs 47.8 [9.4]) and somatization (mean [SD] scores, 55.5 [15.5] vs 47.0 [7.6]). Self-reported (mean [SD] scores, 79.6 [13.1] vs 85.5 [12.3]) and parent-reported (mean [SD] scores, 80.3 [15.5] vs 88.6 [13.0]) quality of life scores were also lower in cases than controls. Conclusions and Relevance: In this cohort study, compared with contemporaneous sibling or community controls, patients with MIS-C had more abnormal neurologic examinations, worse working memory scores, more somatization and depression symptoms, and lower quality of life 6 to 12 months after hospital discharge. Although these findings need to be confirmed in larger studies, enhanced monitoring may be warranted for early identification and treatment of neurological and psychological symptoms.

Decision-Making and Ethical Considerations of Tracheostomy and Chronic Mechanical Ventilation in Children With Acute Neurological Injury.

The decisions around whether or not to provide tracheostomy and chronic mechanical ventilation to children with acute neurologic injury are difficult for medical providers and surrogate decision makers. Consideration of the 4 primary principles of medical ethics-autonomy, beneficence, non-maleficence, and justice-can provide a framework from which constructive discussions can form. Determination of the goals of care is a good first step in navigating these complex decisions. A shared decision model should be used, including education of decision-makers by medical providers and appropriate recommendations based on the stated goals of care. In this paper, 2 illustrative cases are discussed highlighting the utility of this decision-making framework.

Early-Onset Ventilator-Associated Pneumonia in Pediatric Severe Traumatic Brain Injury.

BACKGROUND: Early-onset ventilator-associated pneumonia (VAP) is associated with poor outcomes in patients with severe traumatic brain injury (TBI). The primary aim of this study was to describe VAP, including the microbiology of VAP and differences in frequency of VAP when various definitions are applied. The secondary aim was to determine the clinical variables associated with the development of VAP in children with severe TBI. METHODS: This is a retrospective cohort study at a quaternary referral children's hospital with a level I trauma center designation. Inclusion criteria were patients aged 0-18 years admitted to the pediatric intensive care unit between 2015 and 2020 with severe TBI requiring at least 2 days of invasive ventilation. VAP was defined by using Center of Disease Control (CDC) definition or clinical VAP, based on physician diagnosis. We compared general demographics, reviewed trauma and injury data, and outcomes to assess any differences between patients with VAP and non-VAP patients. Associations were tested with regression models. RESULTS: After applying all inclusion and exclusion criteria, 90 patients were included in the analysis. Patients with VAP were older (8.5 vs. 5.6 years, P = 0.03). Patients with VAP were less likely to have suffered from abusive head trauma (P = 0.01). Patients who received continuous neuromuscular blockade or targeted temperature management did not have different frequencies of VAP. CDC-defined VAP was diagnosed in 27% of patients. Number of patients with VAP increased to 41% for physician-diagnosed or clinical VAP. Methicillin-sensitive Staphylococcus aureus was the most common isolate grown, followed by Hemophilus influenza, with most VAP occurring on days 2-5 of intubation. VAP was not associated with mortality but was associated with worse functional status scale in patients who survived to discharge (8 vs. 7.5, P = 0.048). Over a cumulative period of days, nebulized 3% and albuterol were associated with decreased incidence of VAP. CONCLUSIONS: Ventilator-associated pneumonia occurs commonly in children with severe TBI, with rates of 27-41%, depending on CDC-defined VAP or clinical VAP. The discrepancy between clinical VAP and CDC-defined VAP further illustrates the need for a standardized definition for VAP. Although most interventions were not associated with VAP, nebulized 3% saline and albuterol were associated with reduced incidence of VAP; future investigation is needed to determine whether mucolytic agents can decrease the rate of VAP in children with severe TBI.

Neuromonitoring During ECMO Support in Children.

Extracorporeal membrane oxygenation is a potentially lifesaving intervention for children with severe cardiac or respiratory failure. It is used with increasing frequency and in increasingly more complex and severe diseases. Neurological injuries are important causes of morbidity and mortality in children treated with extracorporeal membrane oxygenation and include ischemic stroke, intracranial hemorrhage, hypoxic-ischemic injury, and seizures. In this review, we discuss the epidemiology and pathophysiology of neurological injury in patients supported with extracorporeal membrane oxygenation, and we review the current state of knowledge for available modalities of monitoring neurological function in these children. These include structural imaging with computed tomography and ultrasound, cerebral blood flow monitoring with near-infrared spectroscopy and transcranial Doppler ultrasound, and physiological monitoring with electroencephalography and plasma biomarkers. We highlight areas of need and emerging advances that will improve our understanding of neurological injury related to extracorporeal membrane oxygenation and help to reduce the burden of neurological sequelae in these children.

Multi-Lobar Atelectasis in Children Receiving Venoarterial Extracorporeal Membrane Oxygenation for Cardiac Indications.

BACKGROUND: Respiratory complications are common in patients who require venoarterial (VA) extracorporeal membrane oxygenation (ECMO) for cardiac indications. We aimed to examine the frequency and characteristics of patients who develop multi-lobar atelectasis early in the course of VA ECMO and to identify factors associated with its occurrence. METHODS: We performed a single-center retrospective review of consecutive pediatric subjects on VA ECMO in the cardiovascular ICU from 2014 to 2019. Chest radiographs before VA ECMO initiation and daily for up to 5 d of VA ECMO support were reviewed. Multi-lobar atelectasis was defined as the collapse of ≥2 lobes of the same lung. Patients with multi-lobar atelectasis before or immediately after VA ECMO cannulation were excluded. Bivariate comparisons and multivariable logistic regression analyses were performed to identify factors independently associated with lung collapse. Results of the multivariable analysis are provided as odds ratio (OR) with 95% CI. RESULTS: We reviewed 119 VA ECMO runs in 101 unique subjects. Multi-lobar atelectasis occurred in 36 runs (30%), with an isolated collapse of the left lung occurring most frequently (no. runs = 20). VA ECMO runs complicated by multi-lobar atelectasis were significantly longer and associated with lower hospital survival (51% vs 77%, survival in subjects without multi-lobar atelectasis; P = .01). Multivariable logistic regression analysis identified a subsequent ECMO run during the same admission to be independently associated with multi-lobar atelectasis (OR 5.4, 95% CI 1.2-21.5). Subanalysis of the subjects with isolated left lung collapse revealed male sex (OR 8.9, 95% CI 1.6-48.2) and subsequent ECMO run during the same admission (OR 4.0, 95% CI 1.2-13.6) to be independently associated with this complication, and mechanical ventilation at least 12 h before ECMO may be protective (OR 0.22, 95% CI 0.07-0.76). CONCLUSIONS: Multi-lobar atelectasis commonly occurred in children who were receiving VA ECMO for cardiac failure and was associated with worse outcomes. Male patients, a subsequent VA ECMO run during the same hospitalization, and patients in whom mechanical ventilation was initiated shortly before ECMO cannulation may be at increased risk for this complication.

Flexible Bronchoscopy in Pediatric Venovenous Extracorporeal Membrane Oxygenation.

BACKGROUND: Pediatric patients with ARDS will on occasion need venovenous extracorporeal membrane oxygenation (VV-ECMO) for organ support. As these patients recover, they may benefit from lung recruitment maneuvers including flexible bronchoscopy (FB). The objective of this study was to assess the clinical course of patients who underwent FB while on VV-ECMO for ARDS. METHODS: This was a secondary analysis of a retrospective multi-center cohort at 10 United States pediatric academic quaternary care centers. Data were collected on 204 subjects age 14 d-18 y on VV-ECMO. RESULTS: 271 FBs were performed on 129 (63%) subjects. Pre-FB tidal volume was 1.8 mL/kg compared to 2.22 mL/kg following FB (P = .007). Dynamic compliance also improved from pre-FB to post-FB (2.23 vs 3.04 mL/cm H2O, P = .005). There was a low incidence of complications following FB (3.1%). Subjects in the FB group had fewer ECMO-free days (EFDs) (17.9 vs 22.1 d, P < .001), fewer ventilator-free days (VFDs) (40.0 vs 46.5 d, P = .001), and longer ICU length of stay (LOS) (18 vs 32 d, P < .001). Subjects in the early versus late FB group had more EFDs (19.4 vs 15.2 d, P = .003), more VFDs (43.0 vs 34.0 d, P = .004), and shorter ICU LOS (27.5 vs 35.5 d, P = .045). Mortality in the subjects who had at least one FB was 27.1% compared to 40% in the subjects who did not have a FB while on VV-ECMO (P = .057). CONCLUSIONS: FB can be performed on patients while anticoagulated on VV-ECMO with a low incidence of complications. FB may be beneficial especially when performed early in the course of VV-ECMO.

Noninfectious Endophthalmitis Following Intraoperative Indocyanine Green.

BACKGROUND AND OBJECTIVE: The purpose of this article is to describe a cluster of four cases of severe postoperative inflammation in eyes that received intraoperative indocyanine green (ICG) from the same lot. PATIENTS AND METHODS: This was a retrospective chart review of patients from a single-center, retina-only group practice. The ICG lot associated with the inflammatory events was identified and analyzed with high-performance liquid chromatography with UV spectroscopy. RESULTS: Four patients presented on postoperative day 1 with severe inflammation. The first patient was treated with aqueous biopsy and injection of intravitreal antibiotics, followed by topical steroid and antibiotic drops. The subsequent three patients were treated with topical steroid and antibiotic drops. All patients had resolution of inflammation by postoperative day 14 (range 10 to 14 days). High-performance liquid chromatography with UV spectroscopy failed to identify a contaminant. CONCLUSIONS: The use of intravitreal ICG dye as a surgical adjuvant may uncommonly be associated with severe postoperative inflammation. This inflammation may resolve within weeks after topical corticosteroid and antibiotic treatment. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:148-151.].

Tracheostomy Practices and Outcomes in Children During Respiratory Extracorporeal Membrane Oxygenation.

OBJECTIVES: Children receiving prolonged extracorporeal membrane oxygenation (ECMO) support may benefit from tracheostomy during ECMO by facilitating rehabilitation; however, the procedure carries risks, especially hemorrhagic complications. Knowledge of tracheostomy practices and outcomes of ECMO-supported children who undergo tracheostomy on ECMO may inform decision-making. DESIGN: Retrospective cohort study. SETTING: ECMO centers contributing to the Extracorporeal Life Support Organization registry. PATIENTS: Children from birth to 18 years who received ECMO support for greater than or equal to 7 days for respiratory failure from January 1, 2015, to December 31, 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Three thousand six hundred eighty-five children received at least 7 days of ECMO support for respiratory failure. The median duration of ECMO support was 13.0 days (interquartile range [IQR], 9.3-19.9 d), and inhospital mortality was 38.7% (1,426/3,685). A tracheostomy was placed during ECMO support in 94/3,685 (2.6%). Of those who received a tracheostomy on ECMO, the procedure was performed at a median 13.2 days (IQR, 6.3-25.9 d) after initiation of ECMO. Surgical site bleeding was documented in 26% of children who received a tracheostomy (12% after tracheostomy placement). Among children who received a tracheostomy, the median duration of ECMO support was 24.2 days (IQR, 13.0-58.7 d); inhospital mortality was 30/94 (32%). Those that received a tracheostomy before 14 days on ECMO were older (median age, 15.8 yr [IQR, 4.7-15.5] vs 11.7 yr [IQR, 11.5-17.3 yr]; p =0.002) and more likely to have been supported on venovenous-ECMO (84% vs 52%; p = 0.001). Twenty-two percent (11/50) of those who received a tracheostomy before 14 days died in the hospital, compared with 19/44 (43%) of those who received a tracheostomy at 14 days or later (p = 0.03). CONCLUSIONS: Tracheostomies during ECMO were uncommon in children. One in four patients who received a tracheostomy on ECMO had surgical site bleeding. Children who had tracheostomies placed after 14 days were younger and had worse outcomes, potentially representing tracheostomy as a "secondary" strategy for prolonged ECMO support.

Seizures in Children with Cardiac Disease on Extracorporeal Membrane Oxygenation.

BACKGROUND: Children supported with extracorporeal membrane oxygenation (ECMO) have been shown to be at risk for developing seizures. However, previous studies have consisted of heterogeneous patient populations. We aimed to describe the rate of seizures in pediatric patients receiving ECMO for cardiac indications and to identify risk factors for the occurrence of this complication. METHODS: This is a retrospective cohort study of consecutive pediatric patients on ECMO for congenital or acquired cardiac disease between 2014 and 2018 at a tertiary care pediatric hospital. RESULTS: We reviewed 110 children, of whom 104 (95%) received continuous electroencephalogram for at least 48 h after ECMO initiation. Seizures were observed in 20 (18%) children. Seizures were subclinical only in 13 (65%) patients, and 8 (40%) developed status epilepticus. The median time from ECMO initiation to first seizure was 34 h (25%, 75%: 19, 44). Children with seizures were more likely to have suffered pre-ECMO cardiac arrest (odds ratio 5.7, 95% confidence interval 2.0-16.1, p < 0.001), require extracorporeal cardiopulmonary resuscitation (odds ratio 5.2, 95% confidence interval 1.9-14.7, p < 0.001), and have been cannulated via the cervical vessels (p = 0.029). Children with seizures also had lower pH nadir prior to ECMO (p = 0.015) and had higher peak lactate prior to ECMO (p = 0.002). Patients with seizures had significantly a longer median intensive care unit length of stay, (43 versus 32 days, p = 0.02), had a significantly worse pediatric cerebral performance score (2 versus 1, p = 0.03), and tended to have worse survival to hospital discharge (50% versus 71%, p = 0.069). CONCLUSIONS: Seizures in pediatric patients on ECMO for cardiac indications are common, occurring in nearly one in five patients. Seizures are frequently subclinical only and often progress to status epilepticus. Continuous electroencephalogram is therefore warranted for this patient population, especially in the setting of cardiac arrest, extracorporeal cardiopulmonary resuscitation, or severe metabolic acidosis.

Rest ventilator management in children on veno-venous extracorporeal membrane oxygenation.

BACKGROUND: We aimed to use the Extracorporeal Life Support Organization registry to describe the current practice of rest mechanical ventilation setting in children receiving veno-venous extracorporeal membrane oxygenation (V-V ECMO) and to determine if relationships exist between ventilator settings and mortality. METHODS: Data for patients 14 days to 18 years old who received V-V ECMO from 2012-2016 were reviewed. Mechanical ventilation data available includes mode and settings at 24 h after ECMO cannulation. Multivariable logistic regression analysis was performed to determine if rest settings were associated with mortality. RESULTS: We reviewed 1161 subjects, of which 1022 (88%) received conventional mechanical ventilation on ECMO. Rest settings, expressed as medians (25th%, 75th%), are as follows: rate 12 breaths/minute (10, 17); peak inspiratory pressure (PIP) 22 cmH2O (20,27); positive end expiratory pressure (PEEP) 10 cmH2O (8, 10); and fraction of inspired oxygen (FiO2) 0.4 (0.37, 0.60). Survival to discharge was 68%. Higher ventilator FiO2 (odds ratio:1.13 per 0.1 increase, 95% confidence interval:1.04, 1.23), independent of arterial oxygen saturation, was associated with mortality. CONCLUSIONS: Current rest ventilator management for children receiving V-V ECMO primarily relies on conventional mechanical ventilation with moderate amounts of PIP, PEEP, and FiO2. Further study on the relationship between FiO2 and mortality should be pursued.

Open abdomen during extracorporeal membrane oxygenation is a safe and effective treatment for abdominal compartment syndrome.

BACKGROUND/PURPOSE: Decompressive laparotomy and open abdomen for abdominal compartment syndrome have been historically avoided during Extracorporeal Membrane Oxygenation (ECMO) due to seemingly elevated risks of bleeding and infection. Our goal was to evaluate a cohort of pediatric respiratory ECMO patients who underwent decompressive laparotomy with open abdomen at a single institution and to compare these patients to ECMO patients without open abdomen. METHODS: We reviewed all pediatric respiratory ECMO (30 days-18 years) patients treated with decompressive laparotomy with open abdomen at Riley Hospital for Children (1/2000-12/2019) and compared these patients to concurrent respiratory ECMO patients with closed abdomen. We excluded patients with surgical cardiac disease. We assessed demographics, ECMO data, and outcomes and defined significance as p = 0.05. RESULTS: 6 of 81 ECMO patients were treated with decompressive laparotomy and open abdomen. Open and closed abdomen groups had similar age (p = 0.223) and weight (0.286) at cannulation, but the open abdomen group had a higher reliance on vasoactive medications (Vasoactive Inotropic Score, p = 0.040). Open abdomen group survival was similar to closed abdomen patients (66.7%, vs 62.7%, p = 1). Open abdomen patients had lower incidence of ECMO complications (33.3% vs 83.6%, p = 0.014), but the groups had similar bleeding complications (p = 0.412) and PRBC transfusion volume (p = 0.941). CONCLUSION/IMPACT: Pediatric ECMO patients with open abdomen after decompressive laparotomy had similar survival, blood products administered, and complications as those with a closed abdomen. An open abdomen is not a contra-indication to ECMO support in pediatric respiratory patients and should be considered in select patients.

Tracheostomy and long-term mechanical ventilation in children after veno-venous extracorporeal membrane oxygenation.

OBJECTIVE: Our objective is to characterize the incidence of tracheostomy placement and of new requirement for long-term mechanical ventilation after extracorporeal membrane oxygenation (ECMO) among children with acute respiratory failure. We examine whether an association exists between demographics, pre-ECMO and ECMO clinical factors, and the placement of a tracheostomy or need for long-term mechanical ventilation. METHODS: A retrospective multicenter cohort study was conducted at 10 quaternary care pediatric academic centers, including children supported with veno-venous (V-V) ECMO from 2011 to 2016. RESULTS: Among 202 patients, 136 (67%) survived to ICU discharge. All tracheostomies were placed after ECMO decannulation, in 22 patients, with 19 of those surviving to ICU discharge (14% of survivors). Twelve patients (9% of survivors) were discharged on long-term mechanical ventilation. Tracheostomy placement and discharge on home ventilation were not associated with pre-ECMO severity of illness or pre-existing chronic illness. Patients who received a tracheostomy were older and weighed more than patients who did not receive a tracheostomy, although this association did not exist among patients discharged on home ventilation. ECMO duration was longer in those who received a tracheostomy compared with those who did not, as well as for those discharged on home ventilation, compared to those who were not. CONCLUSION: The 14% rate for tracheostomy placement and 9% rate for discharge on long-term mechanical ventilation after V-V ECMO are important patient-centered findings. This study informs anticipatory guidance provided to families of patients requiring prolonged respiratory ECMO support, and lays the foundation for future research.

Veno-Venous Extracorporeal Membrane Oxygenation for Children With Cancer or Hematopoietic Cell Transplant: A Ten Center Cohort.

We performed a multicenter retrospective cohort study of children with 14 days to 18 years of age in the United States from 2011 to 2016 with cancer or hematopoietic cell transplant (HCT) who were supported with veno-venous extracorporeal membrane oxygenation (V-V ECMO). We compared the outcomes of children with oncological diagnoses or HCT supported with V-V ECMO to other children who have received V-V ECMO support. In this cohort of 204 patients supported with V-V ECMO, 30 (15%) had a diagnosis of cancer or a history of HCT. There were 21 patients who had oncological diagnoses without HCT and 9 children were post-HCT. The oncology/HCT group had a higher overall ICU mortality (67% vs. 28%, P < 0.001), mortality on ECMO (43% vs. 21%, P < 0.01), and ICU mortality among ECMO survivors (35% vs. 8%, P < 0.01). The oncology/HCT group had a higher rate of conversion to veno-arterial (V-A) ECMO (23% vs. 9%, P = 0.02) (RR, 2.5; 95% CI, 1.1-5.6). Children with cancer or HCT were older (6.6 vs. 2.9 years, P = 0.02) and had higher creatinine levels (0.65 vs. 0.4 mg/dL, P = 0.04) but were similar to the rest of the cohort for other pre-ECMO variables. For post-HCT patients, survival was significantly worse for those whose indication for HCT was cancer or immunodeficiency (0/6) as compared to other nonmalignant indications (3/3) (P = 0.01).

Guillain-Barré Syndrome in a Child With COVID-19 Infection.

Guillain-Barré syndrome (GBS) is characterized by a monophasic, ascending, and symmetrical paralysis with areflexia that progresses over days to weeks. It is typically a postinfectious autoimmune process that leads to destruction of myelin. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), originated in Wuhan, China, in late 2019 and rapidly spread around the world, causing a pandemic of novel coronavirus disease 2019 (COVID-19). There have been scattered reports of adults with possible GBS and concurrent evidence of COVID-19, but no previous reports in children. The patient is an 8-year-old boy who presented to the emergency department with progressive, ascending weakness with areflexia. He was intubated for airway protection because of poor secretion control. MRI of the spine revealed abnormal enhancement of posterior nerve roots. A lumbar puncture revealed albuminocytologic dissociation with 1 nucleated cell per mm3 and a protein level of 620 mg/dL. Electrodiagnostic findings were compatible with sensorimotor demyelinating polyneuropathy. The lumbar puncture, MRI, and electrodiagnostics were all consistent with GBS. Results of SARS-CoV-2 nucleic acid amplification and SARS-CoV-2 immunoglobulin G antibody tests were positive. Treatment was initiated with intravenous immunoglobulin; he received a total of 2 g/kg. His neurologic examination revealed improvement in the subsequent days. He was extubated after 4 days of intubation. This case is the first reported case of a child with GBS in the setting of an acute COVID-19 infection. This case reveals the wide scope of presentations of COVID-19 and postinfectious processes. Clinicians should constantly have a high level of suspicion for COVID-19.

Extubation Readiness Practices and Barriers to Extubation in Pediatric Subjects.

BACKGROUND: Invasive mechanical ventilation is a lifesaving intervention that is associated with short- and long-term morbidities. Extubation readiness protocols aim to decrease extubation failure rates and simultaneously shorten the duration of invasive ventilation. This study sought to analyze extubation readiness practices at one institution and identify barriers to extubation in pediatric patients who have passed an extubation readiness test (ERT). METHODS: We performed a retrospective chart review of all pediatric subjects admitted between April 2017 and March 2018, and who were on mechanical ventilation. Exclusion criteria were cardiac ICU admission, tracheostomy, chronic ventilator support, limited resuscitation status, and death before extubation attempt. Data with regard to the method of ERT and reasons for delaying extubation were collected. RESULTS: There were 427 subjects included in the analysis with 69% having had an ERT before extubation. Of those, 39% were extubated per our daily spontaneous breathing trial (SBT) protocol, and the daily SBT failed in 30% but they had passed a subsequent pressure support and CPAP trial on the same day. The most common reasons for failing the daily SBT were a lack of spontaneous breathing (30% [75/252]), being intubated < 24 h (24% [60/252]), breathing frequency outside the target range (22% [55/252]), and not meeting tidal volume goal (14% [34/252]). The most common documented reasons for delaying extubation despite passing daily SBT were planned procedure (29% [26/90]), neurologic status (23% [21/90]), and no leak around the endotracheal tube (18% [16/90]). The median time between passing ERT and extubation was 7 h (interquartile range, 5-10). CONCLUSIONS: In our institution, there was variation in extubation readiness practices that could lead to a significant delay in liberation from invasive ventilation. Adjustment of our daily SBT to tolerate a higher work of breathing, such as higher breathing frequencies and lower tidal volumes, and incorporating sedation scoring into the protocol could be made without significantly affecting extubation failure rates.

Upper body peripherally inserted central catheter in pediatric single ventricle patients.

BACKGROUND: There is risk of stenosis and thrombosis of the superior vena cava after upper extremity central catheter replacement. This complication is more serious among patients with single ventricle physiology, as it might preclude them from undergoing further life-sustaining palliative surgery. AIM: To describe complications associated with the use of upper extremity percutaneous intravenous central catheters (PICCs) in children with single ventricle physiology. METHODS: A single institution retrospective review of univentricular patients who underwent superior cavopulmonary anastomoses as their stage 2 palliation procedure from January 2014 until December 2018 and had upper body PICCs placed at any point prior to this procedure. Clinical data including ultrasonography, cardiac catheterization, echocardiogram reports and patient notes were used to determine the presence of thrombus or stenosis of the upper extremity and cervical vessels. Data regarding the presence and duration of upper extremity PICCs and upper extremity central venous catheter (CVC), and use of anticoagulation were recorded. RESULTS: Seventy-six patients underwent superior cavopulmonary anastomoses, of which 56 (73%) had an upper extremity PICC at some point prior to this procedure. Median duration of PICC usage was 24 d (25%, 75%: 12, 39). Seventeen patients (30%) with PICCs also had internal jugular or subclavian central venous catheters (CVCs) in place at some point prior to their superior cavopulmonary anastomoses, median duration 10 d (25%, 75%: 8, 14). Thrombus was detected in association with 2 of the 56 PICCs (4%) and 3 of the 17 CVCs (18%). All five patients were placed on therapeutic dose of low molecular weight heparin at the time of thrombus detection and subsequent cardiac catheterization demonstrated resolution in three of the five patients. No patients developed clinically significant venous stenosis. CONCLUSION: Use of upper extremity PICCs in patients with single ventricle physiology prior to super cavopulmonary anastomosis is associated with a low rate of catheter-associated thrombosis.

Progression of Respiratory Support Following Pediatric Extubation.

OBJECTIVES: High-flow nasal cannula and noninvasive positive pressure ventilation have become ubiquitous in contemporary PICUs. Practice patterns associated with the use of these modalities have not been well described. In this study, we aimed to describe the use of high-flow nasal cannula and noninvasive positive pressure ventilation in children after extubation and analyze the progression of usage in association with patient factors. Our secondary aim was to describe interventions used for postextubation stridor. DESIGN: Single-center retrospective cohort study. SETTING: A 36-bed quaternary medical-surgical PICU. PATIENTS: Mechanically ventilated pediatric patients admitted between April 2017 and March 2018. Exclusions were patients in the cardiac ICU, patients requiring a tracheostomy or chronic ventilatory support, and patients with limited resuscitation status. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Data regarding respiratory modality use was collected for the first 72 hours after extubation. There were 427 patients included in the analysis; 51 patients (11.9%) were extubated to room air, 221 (51.8%) to nasal cannula, 132 (30.9%) to high-flow nasal cannula, and 23 (5.4%) to noninvasive positive pressure ventilation. By 72 hours, 314 patients (73.5%) were on room air, 52 (12.2%) on nasal cannula, 29 (6.8%) on high-flow nasal cannula, eight (1.9%) on noninvasive positive pressure ventilation, and 24 (5.6%) were reintubated. High-flow nasal cannula was the most used respiratory modality for postextubation stridor. Multivariate analysis demonstrated that longer duration of invasive mechanical ventilation increased the odds of initial high-flow nasal cannula and noninvasive positive pressure ventilation use, and a diagnosis of cerebral palsy increased the odds of escalating from high-flow nasal cannula to noninvasive positive pressure ventilation in the first 24 hours post extubation. CONCLUSIONS: High-flow nasal cannula is commonly used immediately after pediatric extubation and the development of postextubation stridor; however, its usage sharply declines over the following 72 hours. Larger multicenter trials are needed to identify high-risk patients for extubation failure that might benefit the most from prophylactic use of high-flow nasal cannula and noninvasive positive pressure ventilation after extubation.