Extracorporeal Membrane Oxygenation Support for Pediatric Burn Patients: Is It Worth the Risk?

OBJECTIVES: Examine the outcomes of pediatric burn patients requiring extracorporeal membrane oxygenation to determine whether extracorporeal membrane oxygenation should be considered in this special population. DESIGN: Retrospective cohort study. SETTING: All extracorporeal membrane oxygenation centers reporting to the Extracorporeal Life Support Organization. SUBJECTS: Pediatric patients (birth to younger than 18 yr) who were supported with extracorporeal membrane oxygenation with a burn diagnosis between 1990 and 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 113 patients were identified from the registry by inclusion criteria. Patients cannulated for respiratory failure had the highest survival (55.7%, n = 97) compared to those supported for cardiac failure (33.3%, n = 6) or extracorporeal cardiopulmonary resuscitation (30%, n = 10). Patients supported on venovenous extracorporeal membrane oxygenation for respiratory failure had the best overall survival at 62.2% (n = 37). Important for the burn population, rates of surgical site bleeding were similar to other surgical patients placed on extracorporeal membrane oxygenation at 22.1%. Cardiac arrest prior to cannulation was associated with increased hospital mortality (odds ratio, 3.41; 95% CI, 0.16-1.01; p = 0.048). Following cannulation, complications including the need for inotropes (odds ratio, 2.64; 95% CI, 1.24-5.65; p = 0.011), presence of gastrointestinal hemorrhage (p = 0.049), and hyperglycemia (glucose > 240 mg/dL) (odds ratio, 3.42; 95% CI, 1.13-10.38; p = 0.024) were associated with increased mortality. Of patients with documented burn percentage of total body surface area (n = 19), survival was 70% when less than 60% total body surface area was involved. CONCLUSIONS: Extracorporeal membrane oxygenation could be considered as an additional level of support for the pediatric burn population, especially in the setting of respiratory failure. Additional studies are necessary to determine the optimal timing of cannulation and other patient characteristics that may impact outcomes.

Endothelial-Dependent Vasomotor Dysfunction in Infants After Cardiopulmonary Bypass.

OBJECTIVES: Cardiopulmonary bypass-induced endothelial dysfunction has been inferred by changes in pulmonary vascular resistance, alterations in circulating biomarkers, and postoperative capillary leak. Endothelial-dependent vasomotor dysfunction of the systemic vasculature has never been quantified in this setting. The objective of the present study was to quantify acute effects of cardiopulmonary bypass on endothelial vasomotor control and attempt to correlate these effects with postoperative cytokines, tissue edema, and clinical outcomes in infants. DESIGN: Single-center prospective observational cohort pilot study. SETTING: Pediatric cardiac ICU at a tertiary children's hospital. PATIENTS: Children less than 1 year old requiring cardiopulmonary bypass for repair of a congenital heart lesion. INTERVENTION: None. MEASUREMENTS AND MAIN RESULTS: Laser Doppler perfusion monitoring was coupled with local iontophoresis of acetylcholine (endothelium-dependent vasodilator) or sodium nitroprusside (endothelium-independent vasodilator) to quantify endothelial-dependent vasomotor function in the cutaneous microcirculation. Measurements were obtained preoperatively, 2-4 hours, and 24 hours after separation from cardiopulmonary bypass. Fifteen patients completed all laser Doppler perfusion monitor (Perimed, Järfälla, Sweden) measurements. Comparing prebypass with 2-4 hours postbypass responses, there was a decrease in both peak perfusion (p = 0.0006) and area under the dose-response curve (p = 0.005) following acetylcholine, but no change in responses to sodium nitroprusside. Twenty-four hours after bypass responsiveness to acetylcholine improved, but typically remained depressed from baseline. Conserved endothelial function was associated with higher urine output during the first 48 postoperative hours (R = 0.43; p = 0.008). CONCLUSIONS: Cutaneous endothelial dysfunction is present in infants immediately following cardiopulmonary bypass and recovers significantly in some patients within 24 hours postoperatively. Confirmation of an association between persistent endothelial-dependent vasomotor dysfunction and decreased urine output could have important clinical implications. Ongoing research will explore the pattern of endothelial-dependent vasomotor dysfunction after cardiopulmonary bypass and its relationship with biochemical markers of inflammation and clinical outcomes.

Whole exome sequencing identifies de novo mutations in GATA6 associated with congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a common birth defect affecting 1 in 3000 births. It is characterised by herniation of abdominal viscera through an incompletely formed diaphragm. Although chromosomal anomalies and mutations in several genes have been implicated, the cause for most patients is unknown. METHODS: We used whole exome sequencing in two families with CDH and congenital heart disease, and identified mutations in GATA6 in both. RESULTS: In the first family, we identified a de novo missense mutation (c.1366C>T, p.R456C) in a sporadic CDH patient with tetralogy of Fallot. In the second, a nonsense mutation (c.712G>T, p.G238*) was identified in two siblings with CDH and a large ventricular septal defect. The G238* mutation was inherited from their mother, who was clinically affected with congenital absence of the pericardium, patent ductus arteriosus and intestinal malrotation. Deep sequencing of blood and saliva-derived DNA from the mother suggested somatic mosaicism as an explanation for her milder phenotype, with only approximately 15% mutant alleles. To determine the frequency of GATA6 mutations in CDH, we sequenced the gene in 378 patients with CDH. We identified one additional de novo mutation (c.1071delG, p.V358Cfs34*). CONCLUSIONS: Mutations in GATA6 have been previously associated with pancreatic agenesis and congenital heart disease. We conclude that, in addition to the heart and the pancreas, GATA6 is involved in development of two additional organs, the diaphragm and the pericardium. In addition, we have shown that de novo mutations can contribute to the development of CDH, a common birth defect.

The use of an extracorporeal membrane oxygenation anticoagulation laboratory protocol is associated with decreased blood product use, decreased hemorrhagic complications, and increased circuit life.

OBJECTIVES: To determine if a comprehensive extracorporeal membrane oxygenation anticoagulation monitoring protocol results in fewer hemorrhagic complications, reduced blood product usage, and increased circuit life. DESIGN: In September 2011, we augmented our standard extracorporeal membrane oxygenation laboratory protocol to include anti-factor Xa assays, thromboelastography, and antithrombin measurements. We performed a retrospective chart review to determine outcomes for patients placed on extracorporeal membrane oxygenation prior to and after the initiation of our anticoagulation laboratory protocol. SETTING: Tertiary care, academic children's hospital. PATIENTS: All patients who were placed on extracorporeal membrane oxygenation at our institution from January 1, 2007, to September 30, 2013. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 261 extracorporeal membrane oxygenation runs before the initiation of the protocol and 105 extracorporeal membrane oxygenation runs after the initiation of the protocol. There were no major changes to our extracorporeal membrane oxygenation circuit or changes to our transfusion threshold during the study period. The indication for extracorporeal membrane oxygenation, age, and severity of illness of the patients were similar before and after protocol initiation. Median blood product usage for packed RBCs, fresh frozen plasma, platelets, and cryoprecipitate decreased significantly after protocol initiation. The occurrence of cannula site bleeding decreased from 22% to 12% (p = 0.04), and surgical site bleeding decreased from 38% to 25% (p = 0.02). Median extracorporeal membrane oxygenation circuit life increased from 3.6 to 4.3 days (p = 0.02). A trend toward increased patient survival was noted, but it did not reach statistical significance. CONCLUSIONS: We demonstrate an association between an extracorporeal membrane oxygenation anticoagulation laboratory protocol using anti-factor Xa assays, thromboelastography, and antithrombin measurements and a decrease in blood product transfusion, a decrease in hemorrhagic complications, and an increase in circuit life. To our knowledge, this is the first study to demonstrate clinical benefit associated with the use of these laboratory values for patients on extracorporeal membrane oxygenation.

Proactive enteral tube feeding in pediatric patients undergoing chemotherapy.

BACKGROUND: To determine feasibility and safety of proactive enteral tube feeding (ETF) in pediatric oncology patients. METHODS: Pediatric patients with newly diagnosed brain tumors, myeloid leukemia or high-risk solid tumors were eligible. Subjects agreeing to start ETF before cycle 2 chemotherapy were considered proactive participants (PPs). Those who declined could enroll as chart collection receiving nutritional standard of care. Nutritional status was assessed using standard anthropometric measurements. Episodes of infection and toxicity related to ETF were documented from diagnosis to end of therapy. A descriptive comparison between PPs and controls was conducted. RESULTS: One hundred four eligible patients were identified; 69 enrolled (20 PPs and 49 controls). At diagnosis, 17% of all subjects were underweight and 26% overweight. Barriers to enrollment included physician, subject and/or family refusal, and inability to initiate ETF prior to cycle 2 of chemotherapy. Toxicity of ETF was minimal, but higher percentage of subjects in the proactive group had episodes of infection than controls. Thirty-nine percent of controls eventually started ETF and were twice as likely to receive parenteral nutrition. PPs experienced less weight loss at ETF initiation than controls receiving ETF and were the only group to demonstrate improved nutritional status at end of study. CONCLUSIONS: Proactive ETF is feasible in children with cancer and results in improved nutritional status at end of therapy. Episodes of infection in this study are concerning; therefore, a larger randomized trial is required to further delineate infectious risks and toxicities that may be mitigated by improved nutritional status.

Outcomes of congenital diaphragmatic hernia in the modern era of management.

OBJECTIVE: To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ(2), and regression analysis. RESULTS: Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes. CONCLUSIONS: Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation.

Extracorporeal membrane oxygenation as a platform for recovery: a case report of a child with pulmonary hemorrhage, refractory hypoxemic respiratory failure, and new onset goodpasture syndrome.

We report a case of a 9-year-old female with acute pulmonary hemorrhage and refractory hypoxemic respiratory failure secondary to Goodpasture syndrome (GS). After failing treatment with high frequency oscillatory ventilation and inhaled nitric oxide, she was successfully managed with venovenous extracorporeal membrane oxygenation (VV ECMO). The patient's weight at the time of cannulation was 31 kg. A 19 French 18 cm (arterial) Biomedicus cannula was inserted in the right internal jugular vein and used as the drain. A 17 French 50 cm (venous) Biomedicus cannula was inserted in the right femoral vein and used as the return. Then the patient was anticoagulated with 100 units/kg of intravenous heparin and the circuit was primed with one unit of packed red blood cells. VV ECMO was performed with an SIII Sorin roller head pump with integrated servo regulator and a Quadrox D Bioline coated oxygenator. Despite systemic anticoagulation with heparin, the patient's pulmonary hemorrhage resolved. Extracorporeal membrane oxygenation served as a platform through which we were able to provide renal replacement therapy and plasmapheresis. The patient was successfully discharged home with normal pulmonary function.

Is preterm delivery indicated in fetuses with gastroschisis and antenatally detected bowel dilation?

INTRODUCTION: Due to the controversy surrounding diagnostic ultrasound evaluations and elective preterm delivery of fetuses with gastroschisis, we sought to calculate the predictive value of bowel dilation in fetuses with gastroschisis and evaluate the effect of preterm delivery on neonatal outcomes. MATERIALS AND METHODS: Ultrasounds and medical records of 103 mother-infant pairs with fetal gastroschisis were reviewed. Eighty-nine pairs met the criteria. Intestinal complications, gestational age at delivery, birth weight, and number of abdominal surgeries were documented. RESULTS: Forty-eight fetuses (54%) had bowel dilation and 41 (46%) did not. The positive predictive value of bowel dilation for complicated gastroschisis was 21%. There were 50 (56%) preterm and 39 (44%) term deliveries. The mean birth weight was 2,114 g (SD = 507) and 2,659 g (SD = 687), p = 0.001. For infants delivered preterm, the mean number of postnatal abdominal surgeries was 2.1 (SD = 1.1) as compared to 1.3 (SD = 0.5) surgical procedures for those infants delivered at term gestation. This was not statistically significant. With respect to hospital stay for each group, the mean length of neonatal intensive care unit admission was 48 days (SD = 33) in the preterm group and 35 days (SD = 50) in the term group, which was not statistically significant. DISCUSSION: Ultrasound-detected bowel dilation was not predictive of important intestinal complications. Our data did not substantiate any benefit for elective preterm delivery of neonates with gastroschisis.

Congenital Diaphragmatic Hernia Repair at the Bedside or Operating Theater.

BACKGROUND: For critically ill congenital diaphragmatic hernia (CDH) patients on high frequency oscillatory ventilation (HFOV), extracorporeal membrane oxygenation (ECMO), and/or inhaled nitric oxide (iNO), operative repair in the neonatal intensive care unit (NICU) has been proposed to avoid complications during transport to an operating room (OR). This study compared neonates with CDH who received herniorrhaphy in the NICU or OR, with a subgroup analysis considering only patients supported with ECMO. METHODS: Patients admitted to the NICU in the first 2 weeks of life at a free-standing children's hospital between July 2004 and September 2021 were examined. Patients were categorized according to location of CDH repair, and impact on operative complications and survival was compared. RESULTS: 185 patients were admitted to the NICU with posterolateral CDH and received operative repair. 48 cases were operated on at the bedside in the NICU and 137 in the OR. Patients repaired in the NICU had higher use of HFOV, pulmonary vasodilators, and ECMO (all P < .001). Children repaired in the NICU experienced significantly higher in-hospital death and overall mortality (P < .001). However, in multivariate analysis, repair location was not a significant predictor of survival to discharge in patients receiving ECMO. No significant difference in surgical site infection was detected for operative location (P = .773). DISCUSSION: Congenital diaphragmatic hernia repair in the NICU occurred more frequently among higher risk patients who experienced worse survival. The rate of surgical site infection appeared similar overall and across subgroups suggesting adequate sterility and technique for bedside procedures, when necessary, despite restricted access to advanced operative equipment.

Rapid resolution of hyperammonemia in neonates using extracorporeal membrane oxygenation as a platform to drive hemodialysis.

OBJECTIVE: We aimed to clarify the impact of extracorporeal membrane oxygenation (ECMO) as a platform to drive hemodialysis (HD) for ammonia clearance on outcomes of neonates with severe hyperammonemia. STUDY DESIGN: All neonates treated for hyperammonemia at a single children's hospital between 1992 and 2016 were identified. Patient characteristics and outcomes were compared between those receiving medical management or ECMO/HD. RESULT: Twenty-five neonates were treated for hyperammonemia, of which 13 (52%) received ECMO/HD. Peak ammonia levels among neonates treated with ECMO/HD were significantly higher than those medically managed (1041 [IQR 902-1581] µmol/L versus 212 [IQR 110-410] µmol/L; p = 0.009). Serum ammonia levels in the ECMO/HD cohort declined to the median of medically managed within 4.5 (IQR 2.9-7.0) hours and normalized within 7.3 (IQR 3.6-13.5) hours. All neonates survived ECMO/HD, and nine (69.2%) survived to discharge. CONCLUSION: ECMO/HD is an effective adjunct to rapidly clear severe hyperammonemia in newborns, reducing potential neurodevelopmental morbidity.

Extracorporeal Cardiopulmonary Resuscitation Among Patients with Structurally Normal Hearts.

Extracorporeal cardiopulmonary resuscitation (eCPR) has been well described as a rescue therapy in refractory cardiac arrest among patients with congenital heart disease. The purpose of this retrospective analysis of data from the Extracorporeal Life Support Organization was to evaluate outcomes of eCPR in patients with structurally normal hearts and to identify risk factors that may contribute to mortality. During the study period, 1,431 patients met inclusion criteria. Median age was 16 years. Overall survival to hospital discharge was 32%. Conditional logistic regression demonstrated an independent survival benefit among smaller patients, patients with a lower partial pressure of carbon dioxide (PaCO2) on cannulation, and those with a shorter duration from intubation to eCPR cannulation. A diagnosis of sepsis was independently associated with a nearly threefold increase in odds of mortality, whereas the diagnosis of myocarditis portended a more favorable outcome. Neurologic complications, pulmonary hemorrhage, disseminated intravascular coagulation, CPR, pH less than 7.20, and hyperbilirubinemia after eCPR cannulation were independently associated with an increase in odds of mortality. When utilizing eCPR in patients with structurally normal hearts, a diagnosis of sepsis is independently associated with mortality, whereas a diagnosis of myocarditis is protective. Neurologic complications and pulmonary hemorrhage while on extracorporeal membrane oxygenation (ECMO) are independently associated with mortality.

Review of 1,000 consecutive extracorporeal membrane oxygenation runs as a quality initiative.

BACKGROUND: Extracorporeal membrane oxygenation is a resource-intensive mode of life-support potentially applicable when conventional therapies fail. Given the initial success of extracorporeal membrane oxygenation to support neonates and infants in the 1980s, indications have expanded to include adolescents, adults, and selected moribund patients during cardiopulmonary resuscitation. This single-institution analysis was conducted to evaluate programmatic growth, outcomes, and risk for death despite extracorporeal membrane oxygenation across all ages and diseases. METHODS: Beginning in 1989, we registered prospectively all extracorporeal membrane oxygenation patient data with the Extracorporeal Life Support Organization. We queried this registry for our institution-specific data to compare the parameter of "discharge alive" between age groups (neonatal, pediatric, adult), disease groups (respiratory, cardiac, cardiopulmonary resuscitation), and modes of extracorporeal membrane oxygenation (veno-venous; veno-arterial). Extracorporeal membrane oxygenation-specific complications (mechanical, hemorrhagic, neurologic, renal, cardiovascular, pulmonary, infectious, metabolic) were analyzed similarly. Descriptive statistics, Kaplan-Meier, and linear regression analyses were conducted. RESULTS: After 1,052 extracorporeal membrane oxygenation runs, indications have expanded to include adults, to supplement cardiopulmonary resuscitation, to support hemodialysis in neonates and plasmapheresis in children, and to bridge all age patients to heart and lung transplant. Overall survival to discharge was 52% and was better for respiratory diseases (P < .001). Probability of individual survival decreased to <50% if pre-extracorporeal membrane oxygenation mechanical ventilation exceeded respectively 123 hours for cardiac, 166 hours for cardiopulmonary resuscitation, and 183 hours for respiratory diseases (P = .013). Complications occurred most commonly among cardiac and cardiopulmonary resuscitation runs (P < .001), the veno-arterial mode (P < .001), and in adults (P = .044). CONCLUSION: Our extracorporeal membrane oxygenation program, an Extracorporeal Life Support Organization-designated Center of Excellence, has experienced substantial growth in volume and indications, including increasing age and disease severity. Considering the entire cohort, pre-extracorporeal membrane oxygenation ventilation exceeding 7 days was associated with an increased probability of death.

Late presentation of alveolar capillary dysplasia in an infant.

OBJECTIVE: To report a case of late presentation of alveolar capillary dysplasia and misalignment of pulmonary veins. DESIGN: Descriptive case report. SETTING: Pediatric intensive care unit of a tertiary care children's hospital. PATIENT: Seven-week-old female infant with severe hypoxemic respiratory failure. CONCLUSION: Alveolar capillary dysplasia with misalignment of pulmonary veins is a cause of primary pulmonary hypertension in newborns that can rarely present past the newborn period. Early lung biopsy can be helpful in establishing the diagnosis and avoiding ineffective, futile, and expensive therapeutic interventions.

Leukopheresis therapy for severe infantile pertussis with myocardial and pulmonary failure.

OBJECTIVE: To describe an infant with severe pertussis successfully treated with leukopheresis. DESIGN: Case report. SETTING: Pediatric intensive care unit of a children's hospital. PATIENT: Five-wk-old female with pertussis complicated by cardiorespiratory failure. INTERVENTIONS: Single leukopheresis treatment. MEASUREMENTS AND MAIN RESULTS: Normalization of the white blood cell count and marked cardiorespiratory improvement after treatment; patient survived. CONCLUSION: Given the temporal association between treatment and improvement, we hypothesize that the markedly elevated white blood cell count has a major role in the cardiopulmonary compromise.

Smart Multi-Frequency Bioelectrical Impedance Spectrometer for BIA and BIVA Applications.

Bioelectrical impedance analysis (BIA) is a noninvasive and commonly used method for the assessment of body composition including body water. We designed a small, portable and wireless multi-frequency impedance spectrometer based on the 12 bit impedance network analyzer AD5933 and a precision wide-band constant current source for tetrapolar whole body impedance measurements. The impedance spectrometer communicates via Bluetooth with mobile devices (smart phone or tablet computer) that provide user interface for patient management and data visualization. The export of patient measurement results into a clinical research database facilitates the aggregation of bioelectrical impedance analysis and biolectrical impedance vector analysis (BIVA) data across multiple subjects and/or studies. The performance of the spectrometer was evaluated using a passive tissue equivalent circuit model as well as a comparison of body composition changes assessed with bioelectrical impedance and dual-energy X-ray absorptiometry (DXA) in healthy volunteers. Our results show an absolute error of 1% for resistance and 5% for reactance measurements in the frequency range of 3 kHz to 150 kHz. A linear regression of BIA and DXA fat mass estimations showed a strong correlation (r(2)=0.985) between measures with a maximum absolute error of 6.5%. The simplicity of BIA measurements, a cost effective design and the simple visual representation of impedance data enables patients to compare and determine body composition during the time course of a specific treatment plan in a clinical or home environment.

Effect of a Clinical Practice Guideline for Pediatric Complicated Appendicitis.

IMPORTANCE: Complicated appendicitis is a common condition in children that causes substantial morbidity. Significant variation in practice exists within and between centers. We observed highly variable practices within our hospital and hypothesized that a clinical practice guideline (CPG) would standardize care and be associated with improved patient outcomes. OBJECTIVE: To determine whether a CPG for complicated appendicitis could be associated with improved clinical outcomes. DESIGN, SETTING, AND PARTICIPANTS: A comprehensive CPG was developed for all children with complicated appendicitis at Monroe Carell Jr Children's Hospital at Vanderbilt, a freestanding children's hospital in Nashville, Tennessee, and was implemented in July 2013. All patients with complicated appendicitis who were treated with early appendectomy during the study period were included in the study. Patients were divided into 2 cohorts, based on whether they were treated before or after CPG implementation. Clinical characteristics and outcomes were recorded for 30 months prior to and 16 months following CPG implementation. EXPOSURE: Clinical practice guideline developed for all children with complicated appendicitis at Monroe Carell Jr Children's Hospital at Vanderbilt. MAIN OUTCOMES AND MEASURES: The primary outcome measure was the occurrence of any adverse event such as readmission or surgical site infection. In addition, resource use, practice variation, and CPG adherence were assessed. RESULTS: Of the 313 patients included in the study, 183 were boys (58.5%) and 234 were white (74.8%). Complete CPG adherence occurred in 78.7% of cases (n = 96). The pre-CPG group included 191 patients with a mean (SD) age of 8.8 (4.0) years, and the post-CPG group included 122 patients with a mean (SD) age of 8.7 (4.1) years. Compared with the pre-CPG group, patients in the post-CPG group were less likely to receive a peripherally inserted central catheter (2.5%, n = 3 vs 30.4%, n = 58; P < .001) or require a postoperative computed tomographic scan (13.1%, n = 16 vs 29.3%, n = 56; P = .001), and length of hospital stay was significantly reduced (4.6 days post-CPG vs 5.1 days pre-CPG, P < .05). Patients in the post-CPG group were less likely to have a surgical site infection (relative risk [RR], 0.41; 95% CI, 0.27-0.74) or require a second operation (RR, 0.35; 95% CI, 0.12-1.00). In the pre-CPG group, 30.9% of patients (n = 59) experienced any adverse event, while 22.1% of post-CPG patients (n = 27) experienced any adverse event (RR, 0.72; 95% CI, 0.48-1.06). CONCLUSIONS AND RELEVANCE: Significant practice variation exists among surgeons in the management of pediatric complicated appendicitis. In our institution, a CPG that standardized practice patterns was associated with reduced resource use and improved patient outcomes. Most surgeons had very high compliance with the CPG.

Neonatal carotid repair at ECMO decannulation: patency rates and early neurologic outcomes.

PURPOSE: Neonates placed on veno-arterial extracorporeal membrane oxygenation (VA-ECMO) undergo either carotid repair or ligation at decannulation. Study aims were to evaluate carotid patency rates after repair and to compare early neurologic outcomes between repaired and ligated patients. METHODS: A retrospective study of all neonates without congenital heart disease (CHD) who had VA-ECMO between 1989 and 2012 was completed using our institutional ECMO Registry. Carotid patency after repair, neuroimaging studies, and auditory brainstem response (ABR) testing at time of discharge were examined. RESULTS: 140 neonates were placed on VA-ECMO during the study period. Among survivors, 84% of carotids repaired and imaged remained patent at last study. No significant differences were observed between infants in the repaired and ligated groups regarding diagnosis, ECMO duration, or length of stay. A large proportion (43%) developed a severe brain lesion after VA-ECMO, but few failed their ABR testing. Differences in early neurologic outcomes between the two groups of survivors were not significant. CONCLUSIONS: At this single institution, carotid patency is excellent following repair at ECMO decannulation. No increased incidence of severe brain lesions or greater neurosensory impairment in the repair group was observed. Further studies are needed to investigate the effects of ligation on longer-term neurocognitive outcomes.

Congenital diaphragmatic hernia repair on extracorporeal life support: a decade of lessons learned.

Congenital diaphragmatic hernia (CDH) is a vexing anomaly that manifests with variable pulmonary compromise in neonates. More than one-third of neonates with CDH require extracorporeal membrane oxygenation (ECMO) for refractory pulmonary hypertension (PHN). To assess the outcome of neonates having CDH repair on ECMO, we reviewed our experience for babies treated between 1992 and 2003. Of 97 neonates with CDH, 40 required ECMO, and 30 were repaired on bypass. Eighteen were supported by veno-venous bypass (VV) and 12 by veno-arterial bypass (VA). While on ECMO, transfusion requirements increased twofold postoperatively (15 to 33 cc x kg(-1) day(-1), P = 0.03) and then significantly decreased after decannulation (1.5 cc x kg(-1) x day(-1), P < 0.01). Non-intracranial hemorrhage occurred in 7 (23%) infants and intracranial hemorrhage in 3 (10%). Twelve (40%) infants died; one (3%) on ECMO secondary to refractory PHN. The mean length of stay for the 18 (60%) survivors was 48 days. Comparisons between survivors and nonsurvivors showed a significantly increased mortality for infants placed on VA bypass (P < 0.01). However, no other variable was predictive of survival. We conclude that CDH repair on ECMO is technically feasible, shows similar survival to the Extracorporeal Life Support Organization (ELSO) registry, and is associated with few bleeding complications.

Resuscitation of fat embolism syndrome with extracorporeal membrane oxygenation.

Embolization of marrow fat appears to be an inevitable consequence of long bone fractures. Pulmonary fat embolism (FE) with cardiovascular collapse is associated with a high mortality rate because of acute right ventricular failure and hypoxia. Immediate and appropriate resuscitation is required to prevent sudden death. Although extracorporeal membrane oxygenation (ECMO) has been used for a multitude of applications involving respiratory and circulatory collapse, its full potential as a standard conventional therapy has yet to be exploited. Herein, we describe the successful use of veno-venous (V-V) ECMO in a trauma patient who initially presented with fractures of the right ulna and femur. After surgery, the patient rapidly decompensated despite massive ventilatory support and was placed on ECMO. ECMO support lasted approximately 120 hours followed by an uneventful recovery and discharge 10 days later.

A case series of the successful use of ECMO, continuous renal replacement therapy, and plasma exchange for thrombocytopenia-associated multiple organ failure.

We present three cases of pediatric patients with thrombocytopenia-associated multiple organ failure and the evidence for providing extracorporeal organ support. All three patients had severe cardiac dysfunction, respiratory failure, and acute kidney injury treated with venoarterial extracorporeal membrane oxygenation, continuous renal replacement therapy, and plasma exchange. Despite the presence of multiple organ failure and high risk of mortality, all three patients survived with minimal long-term sequelae.