"Extracorporeal membrane oxygenation outcomes in multisystem inflammatory syndrome of childhood - An extracorporeal life support organization registry study".

Multisystem inflammatory disease in childhood (MIS-C) is a novel pediatric syndrome after a COVID-19 infection that causes systemic injury, with potential life-threatening hemodynamic compromise requiring Extracorporeal Membrane Oxygenation (ECMO) support. We performed an observational retrospective cohort study in children aged 0-18 years with MIS-C and non-MIS-C myocarditis on ECMO between January 2020 and December 2021, using the ELSO Registry database. We aimed to compare the outcomes of both populations and to identify factors for decreased survival in MIS-C patients on ECMO. The Extracorporeal Life Support Organization (ELSO) Registry reported 310 pediatric ECMO patients with MIS-C (56.1%) and non-MIS-C myocarditis (43.9%). No difference was found in survival to hospital discharge between groups (67.2% for MIS-C vs 69.1% for non-MIS-C myocarditis, p 0.725). Multivariable analysis demonstrated that ECPR and co-infection were significantly associated with decreased survival to hospital discharge in MIS-C patients (OR 0.138, p 0.01 and OR 0.44, p 0.02, respectively). Outcomes of children with MIS-C on ECMO support are similar to those of non-MIS-C myocarditis despite higher infectious, multiorgan dysfunction and respiratory complications accompanying COVID-19 infections. The use of ECMO for MIS-C patients seems to be feasible and safe. Prospective studies on the use of ECMO support in MIS-C patients may improve outcomes in this pediatric population.

Right Atrial Lines as Primary Access for Postoperative Pediatric Cardiac Patients.

To characterize the use of right atrial lines (RALs) as primary access in the postoperative care of neonatal and pediatric patients after cardiothoracic surgery and to identify risk factors associated with RAL complications. Observational retrospective cohort study in pediatric cardiac patients who underwent RAL placement in a tertiary children's hospital from January 2011 through June 2018. A total of 692 children with congenital heart disease underwent 815 RAL placements during the same or subsequent cardiothoracic surgeries during the study period. Median age and weight were 22 days (IQR 7-134) and 3.6 kg (IQR 3.1-5.3), respectively. Neonates accounted for 53.5% of patients and those with single-ventricle physiology were 35.4%. Palliation surgery (shunts, cavo-pulmonary connections, hybrid procedures, and pulmonary artery bandings) accounted for 38%. Survival to hospital discharge was 95.5%. Median RAL duration was 11 days (IQR 7-19) with a median RAL removal to hospital discharge time of 0 days (IQR 0-3). Thrombosis and migration were the most prevalent complications (1.7% each), followed by malfunction (1.4%) and infection (0.7%). Adverse events associated with complications were seen in 12 (1.4%) of these RAL placements: decrease in hemoglobin (n = 1), tamponade requiring pericardiocentesis (n = 3), pleural effusion requiring chest tube (n = 2), and need for antimicrobials (n = 6). Multivariable logistic regression showed that RAL duration (OR 1.01, p = 0.006) and palliation surgery (OR 2.38, p = 0.015) were significant and independent factors for complications. The use of RALs as primary access in postoperative pediatric cardiac patients seems to be feasible and safe. Our overall incidence of complications from prolonged use of RALs remained similar or lower to that reported with short-term use of these lines. While RAL duration and palliation surgeries seemed to be associated with complications, severity of illness could be a confounding factor. A prospective assessment of RAL complications may improve outcomes in this medically complex population.

Complications associated with peripherally inserted central catheters in paediatric cardiac patients.

OBJECTIVES: To characterise the use of peripherally inserted central catheters in paediatric cardiac patients and to identify risk factors associated with their complications. MATERIALS AND METHODS: Observational retrospective cohort study in paediatric cardiac patients who underwent peripherally inserted central catheter placement in a tertiary children's hospital from January 2000 to June 2018. RESULTS: 1822 cardiac patients underwent 2952 peripherally inserted central catheter placements in the study period. Median age was 29 days, with survival to hospital discharge of 96.4%. Successful placement achieved 94.5% of attempts, with a median line duration of 12 days. Factors associated with successful placement were the use of general anaesthesia (odds ratio 7.52, p < 0.001) and year of placement (odds ratio 1.08, p < 0.001). The incidence of complications was 28.6%, with thrombosis/occlusion being the most frequent (33%). Thrombosis/occlusion were associated with two and three lumens (odds ratio 1.96, p < 0.001 and 4.63, p = 0.037, respectively). Lines placed by interventional radiology had decreased infiltration (odds ratio 0.20, p = 0.002) and lower migration/malposition (odds ratio 0.36, p < 0.001). The use of maintenance intravenous fluids (odds ratio 3.98, p = 0.008) and peripheral tip position (odds ratio 3.82, p = 0.001) were associated with increased infiltration. The probability of infection decreased over time (odds ratio 0.79, p < 0.001). CONCLUSION: Peripherally inserted central catheters in paediatric cardiac patients have complication rates similar to other paediatric populations. A prospective assessment of the factors associated with their complications in this patient population may be beneficial in improving outcomes.

Outcomes of paediatric cardiac patients after 30 minutes of cardiopulmonary resuscitation prior to extracorporeal support.

OBJECTIVES: To characterise the mortality and neurological outcomes of paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation and to identify risk factors associated with adverse outcomes in this population. MATERIALS AND METHODS: Observational retrospective cohort study in paediatric cardiac patients undergoing cardiopulmonary resuscitation for greater than 30 minutes prior to cannulation in a tertiary children's hospital, from July 2000 to July 2013. RESULTS: Seventy-three paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to cannulation were included in the study. Survival to hospital discharge was 43.8%, with 75% of survivors having either normal neurologic function or only mild disability. Multivariable logistic regression analysis demonstrated that increased use of calcium during resuscitation (odds ratio 14.5, p 0.01), cardiopulmonary resuscitation duration >50 minutes (odds ratio 4.12, p 0.03), >6 interruptions of chest compressions during cannulation (odds ratio 6.40, p 0.03), the need for continuous renal replacement therapy (odds ratio 11.1, p 0.001), and abnormal pupillary response during extracorporeal membrane oxygenation (odds ratio 33.9, p 0.006) were independent predictors for hospital mortality. CONCLUSION: Survival after cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation in our paediatric cardiac cohort was 43.8%. Factors associated with mortality included calcium use during resuscitation, longer cardiopulmonary resuscitation, increased chest compression pauses during cannulation, the use of continuous renal replacement therapy, and abnormal pupils during extracorporeal membrane oxygenation support. A prospective assessment of these factors in paediatric cardiac patients may be beneficial in improving outcomes.

Dead space fractions in neonates following first-stage palliation for hypoplastic left heart syndrome.

PURPOSE: (1) To characterise changes in dead space fraction during the first 120 post-operative hours in neonates undergoing stage 1 palliation for hypoplastic left heart syndrome, including hybrid procedure; (2) to document whether dead space fraction varied by shunt type (Blalock-Taussig shunt and Sano) and hybrid procedure; and (3) to determine the association between dead space fraction and outcomes. METHODS: Retrospective chart review in neonates undergoing stage 1 palliation for hypoplastic left heart syndrome in a cardiac intensive care unit over a consecutive 30-month period. A linear mixed model was used to determine the differences in dead space over time. Multivariable linear regression and a multivariable linear mixed model were used to assess the association between dead space and outcomes at different time points and over time, respectively. RESULTS: Thirty-four neonates received either a Blalock-Taussig shunt (20.5%), Sano shunt (59%), or hybrid procedure (20.5%). Hospital mortality was 8.8%. Dead space fractions in patients undergoing the hybrid procedure were significantly lower on day 1 (p = 0.01) and day 2 (p = 0.02) and increased over time. A dead space fraction >0.6 on post-operative days 3-5 was significantly associated with decreased duration of mechanical ventilation in all surgical groups (p 0.6 on post-operative days 3-5 was associated with lower duration of mechanical ventilation in all surgical groups. A more comprehensive, prospective assessment of dead space in this delicate patient population would likely be beneficial in improving outcomes.

The Use of Extracorporeal Membrane Oxygenation in Life-Threatening Foreign Body Aspiration: Case Series, Review of Extracorporeal Life Support Organization Registry Data, and Systematic Literature Review.

BACKGROUND: Foreign body aspiration (FBA) is a common cause of morbidity and mortality in children < 3 years of age. Guidelines recommend performing a bronchoscopy in any suspected or confirmed FBA. Extracorporeal membrane oxygenation (ECMO) can be used as a rescue mode of support in children with life-threatening FBA for stabilization before, during, and after removal. CASE REPORT: We present a series of children with life-threatening FBA who were placed on ECMO for stabilization before or after FB removal and a review of the literature and the Extracorporeal Life Support Organization database. Foreign bodies were removed without complications, and all patients survived ECMO support and were promptly discharged home. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: ECMO can be safely used in the stabilization of children with life-threatening FBA before, during, and after bronchoscopic removal. ECMO should be considered in the stabilization of children presenting with FBA to facilitate removal.

Craniectomy and Traumatic Brain Injury in Children on Extracorporeal Membrane Oxygenation Support.

Severe trauma may cause refractory life-threatening respiratory failure requiring extracorporeal membrane oxygenation (ECMO). Concurrent traumatic brain injury, however, complicates the use of ECMO because of the major risk of intracranial bleeding with systemic anticoagulation. Craniotomy and/or craniectomy for hematoma evacuation during ECMO are extremely high-risk procedures secondary to ongoing anticoagulation, and there are only a few such case reports in the literature. We present the case of a child with multiple thoracic injuries and life-threatening respiratory failure supported on ECMO. She developed an intracranial hemorrhage while systemically heparinized that required emergent decannulation and bedside craniectomy for hematoma extraction. She survived with an excellent neurologic outcome. We also review the relevant literature regarding the use of ECMO in patients with polytrauma and the occurrence of craniectomy on extracorporeal support, with a focus on pediatric publications. Patients with polytrauma with brain injury can be supported on ECMO, but extreme precaution must be taken regarding anticoagulation. The intracranial complications of ECMO in this population are not infrequent, but our case report and review of the literature suggest that neurosurgical intervention should be considered in life-threatening conditions when no other alternatives are available.

A retrospective study of sedation and analgesic requirements of pediatric patients on extracorporeal membrane oxygenation (ECMO) from a single-center experience.

INTRODUCTION: The purpose of this study is to describe the sedative and analgesic requirements identifying factors associated with medication escalation in neonates and children supported on ECMO. METHOD: Observational retrospective cohort study in a tertiary pediatric intensive care unit from June 2009 to June 2013. RESULTS: One hundred and sixty patients were included in the study. Fentanyl and midazolam were the first line agents used while on ECMO. Higher opiate requirements were associated with younger age (p=0.01), thoracic cannulation (p=0.002), the use of dexmedetomidine (p=0.007) and prolonged use of muscle relaxants (p=0.03). Higher benzodiazepine requirements were associated with younger age (p=0.01), respiratory failure (p=0.02) and the use of second line agents (p=0.002). One third of the patients required second line agents as adjuvants for comfort without a decrease in opiate and/or benzodiazepine requirements. CONCLUSIONS: Providing comfort to subpopulations of pediatric ECMO patients seems to be more challenging. The use of second line agents did not improve comfort in our cohort. Prospective studies are required to optimize analgesia and sedation management in children on ECMO.

Successful engraftment after hematopoietic stem cell transplantation with infusion of donor stem cells through the extracorporeal membrane oxygenation circuit.

Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency due to mutations in the WAS gene expressed in hematopoietic cells. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice when an appropriate human leukocyte antigen-matched donor is available. The use of the extracorporeal membrane oxygenation (ECMO) circuit to infuse donor cells for HSCT has not been previously published in the literature. We describe a case of a child who had successful engraftment after HSCT with infusion of the donor stem cells through the ECMO circuit.

Extubation during pediatric extracorporeal membrane oxygenation: a single-center experience.

OBJECTIVES: Describe aspects of one center's experience extubating infants and children during extracorporeal membrane oxygenation. DESIGN: Retrospective review of medical records. SETTING: Seventy-one-bed critical care service (PICU and cardiovascular ICU) in a large urban tertiary children's hospital. PATIENTS: Pediatric and neonatal patients supported on extracorporeal membrane oxygenation between 1996 and 2013 who were either not intubated or extubated greater than 24 hours during their extracorporeal membrane oxygenation course. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Sixteen of 511 patients on extracorporeal membrane oxygenation were extubated for at least 24 hours during their extracorporeal membrane oxygenation courses. Fourteen had respiratory failure and two had cardiac disease. Five patients died while on extracorporeal membrane oxygenation, but the cause of death was not related to complications associated with extubation. Extubated patients were supported a median of 19.7 days on extracorporeal membrane oxygenation, with a median extubation latency (time between cannulation and first extubation) of 6.2 days and a median extubation duration of 5.5 days. Mean time extubated was 43% of the total time on extracorporeal membrane oxygenation. Two patients were reintubated briefly or had a laryngeal mask airway placed for decannulation (n = 1). The remaining patients were extubated within 5 days of decannulation, weeks afterward (n = 2), transferred to outside facilities (n = 2), or died during extracorporeal membrane oxygenation support (n = 5). We also observed no complications directly attributable to extubation and spontaneous reaeration of consolidated lungs in acute respiratory distress syndrome in extubated patients on extracorporeal membrane oxygenation. CONCLUSION: Extubation and discontinuation of mechanical ventilation appear feasible in patients requiring long-term extracorporeal membrane oxygenation. Emergency procedure planning may need to be modified in extubated patients on extracorporeal membrane oxygenation.

Timing of Kidney Replacement Therapy Initiation and Survival During Pediatric Extracorporeal Membrane Oxygenation: An Extracorporeal Life Support Organization Registry Study.

To characterize kidney replacement therapy (KRT) and pediatric extracorporeal membrane oxygenation (ECMO) outcomes and to identify the optimal timing of KRT initiation during ECMO associated with increased survival. Observational retrospective cohort study using the Extracorporeal Life Support Organization Registry database in children (0-18 yo) on ECMO from January 1, 2016, to December 31, 2020. Of the 14,318 ECMO runs analyzed, 26% of patients received KRT during ECMO. Patients requiring KRT before ECMO had increased mortality to ECMO decannulation (29% vs. 17%, OR 1.97, P < 0.001) and to hospital discharge (58% vs. 39%, OR 2.16, P < 0.001). Patients requiring KRT during ECMO had an increased mortality to ECMO decannulation (25% vs. 15%, OR 1.85, P < 0.001) and to hospital discharge (56% vs. 34%, OR 2.47, P < 0.001). Multivariable logistic regression demonstrated that the need for KRT during ECMO was an independent predictor for mortality to ECMO decannulation (OR 1.49, P < 0.001) and to hospital discharge (OR 2.02, P < 0.001). Patients initiated on KRT between 24 and 72 hours after cannulation were more likely to survive to ECMO decannulation and showed a trend towards survival to hospital discharge as compared to those initiated before 24 hours and after 72 hours.

Time to Continuous Renal Replacement Therapy Initiation and 90-Day Major Adverse Kidney Events in Children and Young Adults.

Importance: In clinical trials, the early or accelerated continuous renal replacement therapy (CRRT) initiation strategy among adults with acute kidney injury or volume overload has not demonstrated a survival benefit. Whether the timing of initiation of CRRT is associated with outcomes among children and young adults is unknown. Objective: To determine whether timing of CRRT initiation, with and without consideration of volume overload (VO; <10% vs ≥10%), is associated with major adverse kidney events at 90 days (MAKE-90). Design, Setting, and Participants: This multinational retrospective cohort study was conducted using data from the Worldwide Exploration of Renal Replacement Outcome Collaborative in Kidney Disease (WE-ROCK) registry from 2015 to 2021. Participants included children and young adults (birth to 25 years) receiving CRRT for acute kidney injury or VO at 32 centers across 7 countries. Statistical analysis was performed from February to July 2023. Exposure: The primary exposure was time to CRRT initiation from intensive care unit admission. Main Outcomes and measures: The primary outcome was MAKE-90 (death, dialysis dependence, or persistent kidney dysfunction [>25% decline in estimated glomerular filtration rate from baseline]). Results: Data from 996 patients were entered into the registry. After exclusions (n = 27), 969 patients (440 [45.4%] female; 16 (1.9%) American Indian or Alaska Native, 40 (4.7%) Asian or Pacific Islander, 127 (14.9%) Black, 652 (76.4%) White, 18 (2.1%) more than 1 race; median [IQR] patient age, 8.8 [1.7-15.0] years) with data for the primary outcome (MAKE-90) were included. Median (IQR) time to CRRT initiation was 2 (1-6) days. MAKE-90 occurred in 630 patients (65.0%), of which 368 (58.4%) died. Among the 601 patients who survived, 262 (43.6%) had persistent kidney dysfunction. Of patients with persistent dysfunction, 91 (34.7%) were dependent on dialysis. Time to CRRT initiation was approximately 1 day longer among those with MAKE-90 (median [IQR], 3 [1-8] days vs 2 [1-4] days; P = .002). In the generalized propensity score-weighted regression, there were approximately 3% higher odds of MAKE-90 for each 1-day delay in CRRT initiation (odds ratio, 1.03 [95% CI, 1.02-1.04]). Conclusions and Relevance: In this cohort study of children and young adults receiving CRRT, longer time to CRRT initiation was associated with greater risk of MAKE-90 outcomes, in particular, mortality. These findings suggest that prospective multicenter studies are needed to further delineate the appropriate time to initiate CRRT and the interaction between CRRT initiation timing and VO to continue to improve survival and reduce morbidity in this population.

Familial hypocalciuric hypercalcemia: new mutation in the CASR gene converting valine 697 to methionine.

Familial hypocalciuric hypercalcemia is an uncommon cause of hypercalcemia that arises from mutations in the calcium-sensing receptor gene. Inactivation of this receptor leads to a decreased receptor sensitivity to calcium, determining that higher concentrations of calcium are needed to inhibit the release of parathormone in the parathyroid glands. Patients usually are asymptomatic. Diagnosis is usually made casually after a routine blood analysis. The syndrome is characterized by mild or moderate hypercalcemia, hypocalciuria, and normal or slightly increased levels of parathormone. The degree of hypercalcemia depends on the type of mutation. The accurate diagnosis is important since it is a benign disorder that does not require medical or surgical treatment. We report a 9-year-old female with persistent hypercalcemia in several routine blood analyses, who was diagnosed with familial hypocalciuric hypercalcemia after genetic studies were performed. A new mutation determining a nucleotide change c.2089G>A in the calcium-sensing receptor gene (exon 7) was detected. This mutation was also found in the patient's mother and brother.

Cangrelor Dosing and Monitoring for Prevention of Acute Systemic-to-Pulmonary Artery Shunt Thrombosis in Neonates.

OBJECTIVE: Systemic-to-pulmonary artery shunts are amongst the most common palliative procedures performed in neonates with congenital heart defects. These procedures require immediate postoperative thromboprophylaxis to prevent life-threatening shunt thrombosis. The novel use of intravenous P2Y12 platelet receptor antagonists has led to a need for dosing recommendations and monitoring. This study aims to determine cangrelor dosing in neonates through laboratory assessment of P2Y12 receptor reactivity and adverse events. METHODS: Observational retrospective cohort study on the use of cangrelor for thromboprophylaxis in the immediate postoperative period of neonates undergoing placement of systemic-to-pulmonary artery shunts in a tertiary children's hospital from March 2020 to March 2021. RESULTS: Ten neonates receiving cangrelor post systemic-to-pulmonary artery shunt placement were included in the study. Median age and weight were 4 days (IQR, 2.75-5.25) and 3.49 kg (IQR, 3.1-3.75), respectively. Five (50%) patients received a 3.5-mm shunt, while the remaining patients received a 4-mm shunt. For thrombin inhibition, 5 (50%) patients received heparin and 5 (50%) received bivalirudin. Median cangrelor dose was 0.1 mcg/kg/min (IQR, 0.1-0.1). Median achieved P2Y12 reaction units (PRU) at this cangrelor dose was 127.5 (IQR, 72.5-173.75). No shunt thrombosis occurred in these patients; however, there was 1 minor hemorrhagic event. CONCLUSIONS: Our study suggests that a cangrelor dose of 0.1 mcg/kg/min is associated with therapeutic PRU and prevents shunt thrombosis in neonates post systemic-to-pulmonary artery shunt, with minimal hemorrhagic complications.

Selumetinib for Refractory Pulmonary and Gastrointestinal Bleeding in Noonan Syndrome.

A 15-year-old-boy with Noonan syndrome and status post orthoptic heart transplant developed mixed mitral valve disease and underwent mechanical mitral valve replacement 6 months before presentation with acute respiratory distress. He developed massive pulmonary hemorrhage that required veno-venous extracorporeal membrane oxygenation (ECMO) support. He had a prolonged anticoagulation free ECMO course of 4 weeks, with ongoing recurrent pulmonary hemorrhage and underwent several rounds of coil embolization of aortopulmonary collaterals. ECMO course was complicated by significant nasopharyngeal bleeding that required embolization of the sphenopalatine artery. Shortly after decannulation, he developed massive gastrointestinal and peritoneal hemorrhage that was treated by embolization of the left gastric artery and a branch of the internal iliac artery. His bleeding was attributed to neo-angiogenesis. Initial treatment with propranolol was unsuccessful. Subsequent treatment with interferon α 2b demonstrated efficacy, but severe neutropenia required cessation of therapy. Because functional alterations of the rat sarcoma virus-mitogen activated protein kinase signaling pathway and protein tyrosine phosphatase nonreceptor type (PTPN11) mutations in Noonan syndrome are known to be associated with neo-angiogenesis, we used the mitogen-activated protein kinase inhibitor selumetinib as a gene-targeted therapy with the hope of controlling bleeding and inhibiting neo-angiogenesis. After initiation of selumetinib, bleeding stopped and allowed the patient to be discharged from the hospital on dipyridamole as antiplatelet prophylaxis for his mechanical mitral valve. He had no further bleeding episodes through 1 year after hospital discharge.

PFAPA syndrome in siblings. Is there a genetic background?

UNLABELLED: "PFAPA syndrome" is an autoinflammatory entity composed of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. There have been many reports of children with the disease, but only occasionally have been described in siblings, and no specific genetic mutation has been determined yet. Corticosteroids are the mainstay in the treatment of the acute attacks. The role of surgery in long-term follow-up (tonsillectomy with or without adenoidectomy) is controversial. We report two brothers affected with the syndrome, in whom corticosteroids as the only treatment led to an improvement. A genetic work-up was performed, making very unlikely other possible syndromes of recurrent fever. CONCLUSION: PFAPA syndrome is the most common recurrent periodic fever disorder described in childhood. Its genetic background has not been elucidated yet. Our contribution with two siblings affected with PFAPA syndrome further support the genetic basis for the entity.

Early Fluid Accumulation and Intensive Care Unit Mortality in Children Receiving Extracorporeal Membrane Oxygenation.

Purpose of this study was to evaluate the impact of early fluid accumulation and renal dysfunction on mortality in children receiving extracorporeal membrane oxygenation (ECMO). Retrospective cohort study of neonatal and pediatric patients who received ECMO between January 2010 and December 2012 in a tertiary level multidisciplinary pediatric intensive care unit (ICU). Ninety-six patients were included, and forty-six (48%) of them received continuous renal replacement therapy (CRRT) during ECMO. Overall mortality was 38.5%. Proportion of patients with acute kidney injury (AKI) at ICU admission was 33% and increased to 47% at ECMO initiation. High-risk diagnoses, extracorporeal cardiopulmonary resuscitation (ECPR), and venoarterial (VA)-ECMO were more common among nonsurvivors. Nonsurvivors had significantly higher proportion of AKI at ICU admission (OR: 2.59, p = 0.04) and fluid accumulation on ECMO day 1 (9% vs. 1%, p = 0.05) compared with survivors. Multivariable logistic regression analysis (adjusted for a propensity score based on nonrenal factors associated with increased mortality) demonstrated that fluid accumulation on ECMO day 1 is significantly associated with increased ICU mortality (OR: 1.07, p = 0.04). Fluid accumulation within the first 24 hours after ECMO cannulation is significantly associated with increased ICU mortality in neonatal and pediatric patients. Prospective studies evaluating the impact of conservative fluid management and CRRT during the initial phase of ECMO may help further define this relationship.

Neurodevelopmental Outcomes in Extracorporeal Membrane Oxygenation Patients: A Pilot Study.

In this pilot study, we evaluated the long-term neurodevelopmental outcomes in neonatal and pediatric patients supported by extracorporeal membrane oxygenation (ECMO) and aimed to identify the role of post-ECMO magnetic resonance imaging (MRI) in predicting neurodevelopmental outcomes. Twenty-nine patients were evaluated using the Ages and Stages Questionnaire, Third Edition (ASQ-3) screening tool. Thirteen were evaluated during their visit at the neurodevelopmental clinic and 16 were interviewed via phone. We also reviewed the post-ECMO MRI brain of these patients and scored the severity of their injury based on the neuroimaging findings. In our cohort of 29 patients, 10 patients (34%) had developmental delay. Of those with developmental delay, 80% were newborns. Sixty-seven percent of patients with developmental delay had moderate to severe MRI abnormalities as compared with only 18% with no developmental deficits (p = 0.03). The younger the age at the time of placement on ECMO, the higher the chances of impaired neurodevelopmental outcome. Long-term follow-up of patients who have survived ECMO, with standardized neuropsychologic testing and post-ECMO imaging, should become the standard of care to improve long-term outcomes. Significant abnormalities on brain MRIs done before discharge correlated with developmental delay on follow-up.

Tailored Management of Airway Abnormalities in Children with Congenital Single Functional Lung.

OBJECTIVES: Pediatric patients born with single functional lung can be associated with symptomatic airway abnormalities. Management of such patients is not only technically demanding but is also ethically challenging. This study reports our experience of managing symptomatic airway abnormalities in pediatric patients with congenital single functional lung. METHODS: Observational retrospective cohort study performed at a tertiary children's hospital from January 2001 to September 2017. All children (0 to 18 years old) with congenital single functional lung (agenesis and hypoplasia) presenting with symptomatic airway abnormalities (long segment congenital tracheal stenosis and tracheomalacia) and requiring surgical interventions were included in the study. Children with single functional lung secondary to non-congenital causes were excluded. RESULTS: A total of 16 patients with single functional lung (agenesis=8 and hypoplasia=8) and airway abnormalities (long segment congenital tracheal stenosis=12 and tracheomalacia =4) were eligible for the study. Lung abnormalities were common on the right side (n = 10, 62.5%). Associated abnormalities (cardiac and non-cardiac) were seen in 11 patients (68.8%). Surgical interventions for airway abnormalities, alone or in combination, included slide tracheoplasty (n=12), aortopexy with or without pericardiopexy (n=7), excision of rudimentary lung (n=4) and placement of intrathoracic tissue expanders to reposition the mediastinum (n=3). Nine patients (56.3%) underwent a one-stage repair while staged repairs (airway and cardiac) were performed in 7 (43.7%). Fourteen patients (87.5%) survived to hospital discharge. Of the survivors, 9 (64.2%) had stable airways not requiring respiratory support at home. CONCLUSION: Management of pediatric patients with airway abnormalities in the setting of congenital single functional lung is feasible with acceptable surgical outcomes. This is facilitated by staged repairs and repositioning of mediastinum before a definitive airway repair in patients with significant comorbidities. Treatment should not be deferred to these patients if there are no obvious contraindications. TYPE OF STUDY: Retrospective Case Control Study LEVEL OF EVIDENCE: Level III.