Katherine Cashen

Extracorporeal Membrane Oxygenation in Pediatric Trisomy 21: 30 Years of Experience from the Extracorporeal Life Support Organization Registry

OBJECTIVES To describe the use of extracorporeal membrane oxygenation (ECMO) in patients with trisomy 21 (T21), to identify risk factors for hospital mortality, and to compare outcomes with those of patients without T21. STUDY DESIGN Children under age 18 years registered in the Extracorporeal Life Support Organization Registry were included. Comparisons between patients with T21 and patients without T21 were performed using the χ(2) or Wilcoxon rank-sum test and multivariable logistic regression. RESULTS The study cohort included 623 patients with T21 and 46 239 patients without T21. The prevalence of T21 was 13.5/1000 patients receiving ECMO. ECMO utilization in patients with T21 increased over time, with 60% of cases occurring in the last decade. There was no significant difference in survival between patients without T21 and those with T21 (63% vs 57%; P = .23). In patients with T21, independent risk factors for mortality before cannulation were a cardiac indication for ECMO support and milrinone use (P ≤ .001 for both). Multivariable risk factors for mortality on ECMO included hemorrhagic, neurologic, renal, and pulmonary complications (P < .04 for all). CONCLUSION The use of ECMO in patients with T21 has increased over time. Patients with a cardiac indication for ECMO have higher mortality compared with those supported for respiratory indications. Despite differences in indications for ECMO, patients with T21 have similar hospital survival as those without T21; thus, by itself, a diagnosis of T21 should not be considered a risk factor for in-hospital mortality when contemplating ECMO cannulation.

Functional Status of Neonatal and Pediatric Patients After Extracorporeal Membrane Oxygenation

Objectives: To describe functional status at hospital discharge for neonatal and pediatric patients treated with extracorporeal membrane oxygenation, and identify factors associated with functional status and mortality. Design: Secondary analysis of observational data collected by the Collaborative Pediatric Critical Care Research Network between December 2012 and September 2014. Setting: Eight hospitals affiliated with the Collaborative Pediatric Critical Care Research Network. Patients: Patients were less than 19 years old and treated with extracorporeal membrane oxygenation. Interventions: Functional status was evaluated among survivors using the Functional Status Scale. Total Functional Status Scale scores range from 6 to 30 and are categorized as 6–7 (good), 8–9 (mildly abnormal), 10–15 (moderately abnormal), 16–21 (severely abnormal), and greater than 21 (very severely abnormal). Measurements and Main Results: Of 514 patients, 267 (52%) were neonates (⩽ 30 d old). Indication for extracorporeal membrane oxygenation was respiratory for 237 (46%), cardiac for 207 (40%), and extracorporeal cardiopulmonary resuscitation for 70 (14%). Among 282 survivors, 89 (32%) had good, 112 (40%) mildly abnormal, 67 (24%) moderately abnormal, and 14 (5%) severely or very severely abnormal function at hospital discharge. Among neonates, development of renal failure and longer hospitalization were independently associated with worse Functional Status Scale. Chronic conditions, prematurity, venoarterial extracorporeal membrane oxygenation, increased red cell transfusion in the first 24 hours of extracorporeal membrane oxygenation, and longer extracorporeal membrane oxygenation duration were independently associated with mortality. Among pediatric patients, chronic neurologic conditions, tracheostomy or home ventilator, extracorporeal cardiopulmonary resuscitation, hepatic dysfunction, and longer ICU stay were independently associated with worse Functional Status Scale. Chronic cardiac conditions, hepatic dysfunction, and neurologic or thrombotic complications were independently associated with mortality. Achieving blood lactate concentration less than or equal to 2 mmol/L during extracorporeal membrane oxygenation was independently associated with survival in both neonatal and pediatric patients. Conclusions: In this study, about half of extracorporeal membrane oxygenation patients survived with good, mildly abnormal, or moderately abnormal function at hospital discharge. Patient and extracorporeal membrane oxygenation-related factors are associated with functional status and mortality.

Extracorporeal membrane oxygenation outcomes in children with hemophagocytic lymphohistiocytosis

Introduction: Pediatric patients with hemophagocytic lymphohistiocytosis (HLH) may develop refractory respiratory or cardiac failure that warrants consideration for extracorporeal membrane oxygenation (ECMO) support. The purposes of this study were to describe the use and outcomes of ECMO in pediatric HLH patients, to identify risk factors for hospital mortality and to compare their ECMO use and outcomes to the ECMO population as a whole. Methods: Pediatric patients (⩽ 18 years) with a diagnosis of HLH in the Extracorporeal Life Support Organization (ELSO) Registry were included. Results: Between 1983 and 2014, data for 30 children with HLH were available in the ELSO registry and all were included in this study. All cases occurred in the last decade. Of the 30 HLH patients, 24 (80%) had a respiratory indication for ECMO and six (20%) had a cardiac indication (of which 4 were E-CPR and 2 cardiac failure). Of the 24 respiratory ECMO patients, 63% were placed on VA ECMO. Compared with all pediatric patients in the ELSO registry during the study period (n=17,007), HLH patients had worse hospital survival (non-HLH 59% vs HLH 30%, p=0.001). In pediatric HLH patients, no pre-ECMO risk factors for mortality were identified. The development of a hemorrhagic complication on ECMO was associated with decreased mortality (p=0.01). Comparing HLH patients with respiratory failure to patients with other immune compromised conditions, the overall survival rate is similar (HLH 38% vs. non-HLH immune compromised 31%, p=0.64). Conclusions: HLH is an uncommon indication for ECMO and these patients have increased mortality compared to the overall pediatric ECMO population. These data should be factored into decision-making when considering ECMO for pediatric HLH patients.

Recurrent Fever and Rash

Periodic fever is uncommon in children. The differential diagnosis is large, even though associated symptoms such as rash may help narrow the differential diagnosis. Atypical presentations require thoughtful evaluation. This article describes a case of a 4-year-old boy who presented to the emergency department with recurrent fever, vomiting, abdominal pain, myalgias, and rash. His hospital course is described along with a review on the background, evaluation, management, and complications of tumor necrosis receptor-1 alpha periodic syndrome.

Is therapeutic hypothermia during neonatal extracorporeal membrane oxygenation associated with intracranial hemorrhage

Introduction: The use of therapeutic hypothermia during neonatal extracorporeal membrane oxygenation (ECMO) as a neurologic protective strategy has gained interest among clinicians despite limited data. Our objective is to describe the relationship between the use of therapeutic hypothermia during neonatal ECMO and complications, mortality and functional status among survivors. Methods: Secondary analysis of data collected by the Collaborative Pediatric Critical Care Research Network between December 2012 and September 2014. Data were collected prospectively from 267 neonates (<30 days) undergoing ECMO at eight clinical sites. Twenty neonates received therapeutic hypothermia. Results: Neonates receiving therapeutic hypothermia were more likely to have intracranial hemorrhage during the first seven days of ECMO than were non-hypothermic neonates (40.0% vs 15.8%, p=0.012). No differences were observed between groups for hospital mortality or functional status at hospital discharge among survivors. Variables independently associated with intracranial hemorrhage in the first seven days of ECMO included therapeutic hypothermia, gestational age at birth, age at initiation of ECMO, fibrinogen concentration and mode of ECMO. Conclusion: Therapeutic hypothermia during neonatal ECMO appears to be associated with intracranial hemorrhage.

Acquired infection during neonatal and pediatric extracorporeal membrane oxygenation

Introduction: Our objectives are to (1) describe the pathogens, site, timing and risk factors for acquired infection during neonatal and pediatric ECMO and (2) explore the association between acquired infection and mortality. Methods: Secondary analysis of prospective data collected by the Collaborative Pediatric Critical Care Research Network between December 2012 and September 2014. Clinical factors associated with acquired infection were assessed with multivariable Cox regression. Factors associated with mortality were assessed with logistic regression. Results: Of 481 patients, 247 (51.3%) were neonates and 400 (83.2%) received venoarterial ECMO. Eighty (16.6%) patients acquired one or more infections during ECMO; 60 (12.5%) patients had bacterial, 21 (4.4%) had fungal and 11 (2.3%) had viral infections. The site of infection included respiratory for 53 (11.0%) patients, bloodstream for 21 (4.4%), urine for 20 (4.2%) and other for 7 (1.5%). Candida species were most common. Median time to infection was 5.2 days (IQR 2.3, 9.6). On multivariable analysis, a greater number of procedures for ECMO cannula placement was independently associated with increased risk of acquired infection during ECMO (Hazard Ratio 2.13 (95% CI 1.22, 3.72), p<0.01) and receiving ECMO in a neonatal ICU compared to a pediatric or cardiac ICU was associated with decreased risk (Hazard Ratio pediatric ICU 4.25 (95% CI 2.20, 8.20), cardiac ICU 2.91 (95% CI 1.48, 5.71), neonatal ICU as reference, p<0.001). Acquired infection was not independently associated with mortality. Conclusion: ECMO procedures and location may contribute to acquired infection risk; however, acquired infection did not predict mortality in this study.

Hyperoxia and Hypocapnia During Pediatric Extracorporeal Membrane Oxygenation: Associations With Complications, Mortality, and Functional Status Among Survivors*

Objectives: To determine the frequency of hyperoxia and hypocapnia during pediatric extracorporeal membrane oxygenation and their relationships to complications, mortality, and functional status among survivors. Design: Secondary analysis of data collected prospectively by the Collaborative Pediatric Critical Care Research Network. Setting: Eight Collaborative Pediatric Critical Care Research Network–affiliated hospitals. Patients: Age less than 19 years and treated with extracorporeal membrane oxygenation. Interventions: Hyperoxia was defined as highest PaO2 greater than 200 Torr (27 kPa) and hypocapnia as lowest PaCO2 less than 30 Torr (3.9 kPa) during the first 48 hours of extracorporeal membrane oxygenation. Functional status at hospital discharge was evaluated among survivors using the Functional Status Scale. Measurements and Main Results: Of 484 patients, 420 (86.7%) had venoarterial extracorporeal membrane oxygenation and 64 (13.2%) venovenous; 69 (14.2%) had extracorporeal membrane oxygenation initiated during cardiopulmonary resuscitation. Hyperoxia occurred in 331 (68.4%) and hypocapnia in 98 (20.2%). Hyperoxic patients had higher mortality than patients without hyperoxia (167 [50.5%] vs 48 [31.4%]; p < 0.001), but no difference in functional status among survivors. Hypocapnic patients were more likely to have a neurologic event (49 [50.0%] vs 143 (37.0%]; p = 0.021) or hepatic dysfunction (49 [50.0%] vs 121 [31.3%]; p < 0.001) than patients without hypocapnia, but no difference in mortality or functional status among survivors. On multivariable analysis, factors independently associated with increased mortality included highest PaO2 and highest blood lactate concentration in the first 48 hours of extracorporeal membrane oxygenation, congenital diaphragmatic hernia, and being a preterm neonate. Factors independently associated with lower mortality included meconium aspiration syndrome. Conclusions: Hyperoxia is common during pediatric extracorporeal membrane oxygenation and associated with mortality. Hypocapnia appears to occur less often and although associated with complications, an association with mortality was not observed.

Emergency Presentation of Heart Disease

Pediatric patients with congenital and acquired heart disease may present to the emergency department (ED) with acute life-threatening decompensation. These children often present with high acuity and in shock, with cyanosis, or with respiratory distress. In this chapter we review common ED presentations of pediatric heart disease and their chronology, pathophysiology, management strategies, and common diagnostic clues/pitfalls that clinicians may face when treating these complex patients.

Pediatric Mechanical Circulatory Support

Extracorporeal life support (ECLS) via extracorporeal membrane oxygenation (ECMO) and ventricular assist devices (VADs) has evolved as a therapeutic treatment option for the care of children with severe ventricular dysfunction, refractory to medical and/or surgical therapy. The American Heart Association currently includes ECLS in both the pediatric and the adult algorithms for refractory shock. In this chapter, we review indications for ECLS initiation, history, basic principles and management strategies, complications, and outcomes.

Pleural Effusions and Pneumothoraces

1. Katherine Cashen, DO* 2. Tara L. Petersen, MD† 1. *Department of Pediatrics, Childrens Hospital of Michigan/Wayne State University School of Medicine, Detroit, MI 2. †Division of Critical Care, Department of Pediatrics, The Children’s Hospital of Wisconsin/The Medical College of Wisconsin, Milwaukee, WI * Abbreviations: BTS: : British Thoracic Society CHF: : congestive heart failure CT: : computed tomography VATS: : video-assisted thoracoscopic surgery 1. Clinicians should be aware of the causes and clinical presentation of pleural effusions and pneumothoraces. 2. Clinicians should understand the current role of diagnostic tests, imaging modalities, and timing of minimally invasive treatments. After completing this article, readers should be able to: 1. Describe the pathogenesis of pleural fluid accumulation. 2. Identify the most likely causes of pleural effusion and pneumothorax. 3. Understand the basic clinical presentation, diagnostic tests, and management of pleural effusions and pneumothoraces. 4. Differentiate between transudative and exudative pleural effusions. 5. Understand the natural history of spontaneous pneumothorax. The pleural space is created by the parietal and visceral pleura that line the chest wall and the lung surface, respectively. Normally, only a small amount (0.3 mL/kg) of hypotonic fluid is present within the pleural space due to homeostatic balances in physiologic fluid production and absorption. Various infectious and noninfectious processes can lead to pathologic filling of the pleural space with fluid (effusion) or air (pneumothorax). Such pathologic changes create a true space that can interfere with normal lung mechanics and, in severe cases, cardiac function. Although much less common in pediatric than adult populations, pleural effusions and pneumothoraces in both groups can lead to substantial complications, resulting in significant morbidity and mortality if unrecognized or untreated. Overall, the cause of pleural effusions and pneumothoraces differs in children compared to adults. In adults, congestive heart failure (CHF) and malignancy account for a substantial number of pleural effusions, but these are uncommon causes in children. Infectious pleural effusions in the setting of pneumonia (parapneumonic effusions) remain the most common cause of effusions in both children and adults. Unlike the adult population, children experience spontaneous …