Perioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease.

Acquired von Willebrand syndrome (aVWS) has been reported in patients with congenital heart diseases associated with shear stress caused by significant blood flow gradients. Its etiology and impact on intraoperative bleeding during pediatric cardiac surgery have not been systematically studied. This single-center, prospective, observational study investigated appropriate diagnostic tools of aVWS compared with multimer analysis as diagnostic criterion standard and aimed to clarify the role of aVWS in intraoperative hemorrhage. A total of 65 newborns and infants aged 0 to 12 months scheduled for cardiac surgery at our tertiary referral center from March 2018 to July 2019 were included in the analysis. The glycoprotein Ib M assay (GPIbM)/von Willebrand factor antigen (VWF:Ag) ratio provided the best predictability of aVWS (area under the receiver operating characteristic curve [AUC], 0.81 [95% CI, 0.75-0.86]), followed by VWF collagen binding assay/VWF:Ag ratio (AUC, 0.70 [0.63-0.77]) and peak systolic echocardiographic gradients (AUC, 0.69 [0.62-0.76]). A cutoff value of 0.83 was proposed for the GPIbM/VWF:Ag ratio. Intraoperative high-molecular-weight multimer ratios were inversely correlated with cardiopulmonary bypass (CPB) time (r = -0.57) and aortic cross-clamp time (r = -0.54). Patients with intraoperative aVWS received significantly more fresh frozen plasma (P = .016) and fibrinogen concentrate (P = .011) than those without. The amounts of other administered blood components and chest closure times did not differ significantly. CPB appears to trigger aVWS in pediatric cardiac surgery. The GPIbM/VWF:Ag ratio is a reliable test that can be included in routine intraoperative laboratory workup. Our data provide the basis for further studies in larger patient cohorts to achieve definitive clarification of the effects of aVWS and its potential treatment on intraoperative bleeding.

Role of high-volume plasmapheresis in the management of paediatric acute liver failure.

OBJECTIVES: Paediatric acute liver failure (PALF) is a life-threatening disease. Management aims to support hepatic regeneration or to bridge to liver transplantation. High-volume plasmapheresis (HVP) removes protein-bound substances, alleviates inflammation, and improves survival in adult acute liver failure. However, experience with HVP in PALF is limited. Aim of this study is to report on feasibility, safety, efficacy and outcomes of HVP in PALF. METHODS: Retrospective observational study in children with PALF. HVP was performed upon identification of negative prognostic indicators, in toxic aetiology or multiorgan failure (MOF). Exchanged volume with fresh-frozen plasma corresponded to 1.5-2.0 times the patient's estimated plasma volume. One daily cycle was performed until the patient met criteria for discontinuation, that is, liver regeneration, liver transplantation, or death. RESULTS: Twenty-two children with PALF (body weight 2.5-106 kg) received 1-7 HVP cycles. No bleeding or procedure-related mortality occurred. Alkalosis, hypothermia and reduction in platelets were observed. Haemolysis led to HVP termination in one infant. Seven children (32%) survived with their native livers, 13 patients (59%) underwent liver transplantation. Two infants died due to MOF. Overall survival was 86%. International normalization ratio (INR), alanine aminotransaminases (ALT), bilirubin and inotropic support were reduced significantly (p < 0.05) after the first HVP-cycle (median): INR 2.85 versus 1.5; ALT 1280 versus 434 U/L; bilirubin 12.7 versus 6.7 mg/dL; norepinephrine dosage 0.083 versus 0.009 µg/kg/min. Median soluble-interleukin-2-receptor dropped significantly following HVP (n = 7): 2407 versus 950 U/mL (p < 0.02). CONCLUSIONS: HVP in PALF is feasible, safe, improves markers of liver failure and inflammation and is associated with lowering inotropic support. Prospective and controlled studies are required to confirm efficacy of HVP in PALF.

Chronic Pleural Effusion in Ventriculoperitoneal Shunt due to Diaphragmatic CSF Fistula: Report of a Case Treated by Endoscopic Choroid Plexus Coagulation and Literature Review.

INTRODUCTION: Chronic pleural cerebrospinal fluid (CSF) effusion is a rare complication after ventriculoperitoneal (VP) shunt insertion and only 18 cases in children and adults have been described so far without catheter dislocation to the intrathoracic cavity. CASE PRESENTATION: We report on a 4-year-old girl with a complex history of underlying neurogenetic disorder, a hypoxic-ischemic encephalopathy after influenza A infection with septic shock and severe acute respiratory distress syndrome, followed by meningitis at the age of 10 months. In consequence, she developed a severe cerebral atrophy and post-meningitic hydrocephalus requiring placement of a VP shunt. At age 4, she was admitted with community-acquired mycoplasma pneumonia and developed increasing pleural effusions leading to severe respiratory distress and requiring continuous chest tube drainage (up to 1,000-1,400 mL/day) that could not be weaned. ß trace protein, in CSF present at concentrations >6 mg/L, was found in the pleural fluid at low concentrations of 2.7 mg/L. An abdomino-thoracic CSF fistula was finally proven by single photon emission computerized tomography combined with low-dose computer tomography. After shunt externalization, the pleural effusion stopped and the chest tube was removed. CSF production rate remains high above 500 mL/24 h. An atrial CSF shunt could not be placed, since a hemodynamically relevant atrial septum defect with frail circulatory balance would not have tolerated the large CSF volumes. Therefore, she underwent a total bilateral endoscopic choroid plexus laser coagulation (CPC) within the lateral ventricles via bi-occipital burr holes. Postoperatively CSF production rate went close to 0 mL and after external ventricular drain removal no signs and symptoms of hydrocephalus developed during a follow-up of now 2.5 years. CONCLUSION: In summary, pleural effusions in patients with VP shunt can rarely be caused by an abdomino-thoracic fistula, with non-elevated ß-trace protein in the pleural fluid. The majority of reported cases in literature were treated by ventriculoatrial shunt. This is the 2nd reported case, which has been successfully treated by radical CPC alone including the temporal horn choroid plexus, making the child shunt independent.

National Survey Regarding Motivation and Conditions of Physicians Working in a Pediatric Cardiac Intensive Care Unit.

BACKGROUND: The professional demands on the expertise in pediatric intensive care have continuously increased in recent years. Due to a lack of applicants, the staffing of a continuous shift service with qualified medical staff poses major challenges to the hospitals. METHODS: A web-based questionnaire with 27 predominantly matrix questions on working conditions and motivation for working in this area was sent to pediatric hospitals throughout Germany. RESULTS: 165 doctors responded to the survey. The average age of the participants was 35.2 years. The average weekend work load reported by 79% of the respondents was 2 weekends per month, 70% of the study participants performed five to seven night shifts per month. 92% of the respondents stated that they basically enjoyed working in the intensive care unit (ICU). When asked to prioritize the working conditions, an appreciative working atmosphere in the team was named as priority 1 by 57%, followed by good guidance in the independent performance of interventions (25%) and good working conditions (19%). DISCUSSION: The survey result shows that neither aspects of work-life balance nor payments are the key issues selecting the interesting, but physically and emotionally demanding job in pediatric ICU. CONCLUSION: When evaluating vocational training in pediatric intensive care medicine, the immediate working atmosphere in the team with mutual respect and understanding and the guidance in training are more important than the general conditions.

Implementation of a Blood-Saving Program in a Pediatric Intensive Care Unit to Reduce Diagnostic Blood Loss.

BACKGROUND: We aimed to reduce blood loss in the pediatric critical care unit (PICU) due to blood sampling in neonates and infants. Therefore, an educational program for our staff was established and evaluated. METHODS: Patients in a PICU of a tertiary referral center aged 0-12 months who underwent surgery of congenital heart disease on cardiopulmonary bypass were enrolled and divided into a pre- and a post-implementation group. We assessed frequency and types of postoperative blood samples, required blood volume, and amount of blood transfusions in the PICU within 5 days after cardiac surgery. RESULTS: Populations were similar prior and after the implementation. Blood drawn for blood gas analysis (0,52 ml±0,16 vs. 0,38 ml±0,12, p<0,001) and for complete blood sampling (2,62 ml±0,32 vs. 2,11 ml±0,35, p<0,001) could be successfully reduced after implementation of our blood-saving program. The daily diagnostic blood loss per patient was significantly reduced by approximately 35% (1,7 ml/kg/d±1,0 vs. 1,1 ml/kg/d±0,7, p=0,008). DISCUSSION: Our quality improvement program is feasible and effective to significantly reduce the blood loss due to blood sampling. Although the incidence of red blood cell transfusions was not significantly reduced, it is certainly beneficial to try to reduce diagnostic blood loss, especially in children with complex diseases requiring long-term intensive care treatment. CONCLUSION: We could demonstrate that it is possible to significantly reduce the blood loss due to blood sampling with a simple educational program for PICU staff. HINTERGRUND: Unser Ziel war es, den Blutverlust durch Blutabnahmen auf der pädiatrischen Intensivstation bei Neugeborenen und Säuglingen zu reduzieren. Deshalb wurde ein Schulungsprogramm für unsere Mitarbeiter etabliert und ausgewertet. METHODE: Patienten unserer pädiatrischen Intensivstation im Alter von 0-12 Monaten nach einer Operation eines angeborenen Herzfehlers mit Herz-Lungen-Maschine, wurden eingeschlossen und in eine Gruppe vor und nach der Implementierung des Schulungsprogramms zugeteilt. Wir haben die Häufigkeit und Art der postoperativen Blutproben, das benötigte Blutvolumen und die Menge der Bluttransfusionen auf der Intensivstation innerhalb von 5 Tagen nach der Herzoperation ausgewertet. ERGEBNISSE: Die Patientencharakteristik beider Gruppen zeigte keine relevanten Unterschiede. Blut, das für Blutgasanalysen (0,52 ml±0,16 vs. 0,38 ml±0,12, p<0,001) und für vollständige Blutentnahmen (2,62 ml±0,32 vs. 2,11 ml±0,35, p<0,001) entnommen wurde, konnte nach Umsetzung unseres Blutsparprogramms erfolgreich reduziert werden. Der tägliche diagnostische Blutverlust pro Patienten wurde signifikant um ca. 35% reduziert (1,7 ml/kg/d±1,0 vs. 1,1 ml/kg/d±0,7, p=0,008). DISKUSSION: Unser Schulungsprogramm für Mitarbeiter ist einfach umzusetzen und effektiv, den Blutverlust durch Blutentnahmen deutlich zu reduzieren. Obwohl die Inzidenz von Bluttransfusionen nicht signifikant reduziert wurde, ist es sicherlich erstrebenswert, den diagnostischen Blutverlust insbesondere bei Kindern mit komplexen Krankheiten, die eine langfristige Intensivbehandlung erfordern, zu reduzieren. SCHLUSSFOLGERUNG: Wir konnten zeigen, dass es möglich ist, den Blutverlust durch Blutentnahme mit einem einfachen Schulungsprogramm für Mitarbeiter auf der Intensivstation deutlich zu reduzieren.

Cerebral Oxygen Metabolism Before and After RBC Transfusion in Infants Following Major Surgical Procedures.

OBJECTIVE: Although infants following major surgery frequently require RBC transfusions, there is still controversy concerning the best definition for requirement of transfusion in the individual patient. The aim of this study was to determine the impact of RBC transfusion on cerebral oxygen metabolism in noncardiac and cardiac postsurgical infants. DESIGN: Prospective observational cohort study. SETTING: Pediatric critical care unit of a tertiary referral center. PATIENTS: Fifty-eight infants (15 after pediatric surgery and 43 after cardiac surgery) with anemia requiring RBC transfusion were included. INTERVENTIONS: RBC transfusion. MEASUREMENTS AND MAIN RESULTS: We measured noninvasively regional cerebral oxygen saturation and microperfusion (relative cerebral blood flow) using tissue spectrometry and laser Doppler flowmetry before and after RBC transfusion. Cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen were calculated. Fifty-eight RBC transfusions in 58 patients were monitored (15 after general surgery, 24 after cardiac surgery resulting in acyanotic biventricular physiology and 19 in functionally univentricular hearts including hypoplastic left heart following neonatal palliation). The posttransfusion hemoglobin concentrations increased significantly (9.7 g/dL vs 12.8 g/dL; 9.7 g/dL vs 13.8 g/dL; 13.1 g/dL vs 15.6 g/dL; p < 0.001, respectively). Posttransfusion cerebral oxygen saturation was significantly higher than pretransfusion (61% [51-78] vs 72% [59-89]; p < 0.001; 58% [35-77] vs 71% [57-88]; p < 0.001; 51% [37-61] vs 58% [42-73]; p = 0.007). Cerebral fractional tissue oxygen extraction decreased posttransfusion significantly 0.37 (0.16-0.47) and 0.27 (0.07-039), p = 0.002; 0.40 (0.2-0.62) vs 0.26 (0.11-0.57), p = 0.001; 0.42 (0.23-0.52) vs 0.32 (0.1-0.42), p = 0.017. Cerebral blood flow and approximated cerebral metabolic rate of oxygen showed no significant change during the observation period. The increase in cerebral oxygen saturation and the decrease in cerebral fractional tissue oxygen extraction were most pronounced in patients after cardiac surgery with a pretransfusion cerebral fractional tissue oxygen extraction greater than or equal to 0.4. CONCLUSION: Following RBC transfusion, cerebral oxygen saturation increases and cerebral fractional tissue oxygen extraction decreases. The data suggest that cerebral oxygenation in postoperative infants with cerebral fractional tissue oxygen extraction greater than or equal to 0.4 may be at risk in instable hemodynamic or respiratory situations.

Multicenter Experience With Mechanical Circulatory Support Using a New Diagonal Pump in 233 Children.

Technological innovations in pediatric extracorporeal life support circuits can reduce system-related complications and may improve patients' outcome. The Deltastream DP3 (Medos Medizintechnik AG, Stolberg, Germany) is a novel rotational pump with a diagonally streamed impeller that can be used over a broad range of flows. We collected patient data from seven pediatric centers to conduct a retrospective cohort study. We examined 233 patients whose median age was 1.9 (0-201) months. The DP3 system was used for cardiopulmonary support as veno-arterial extracorporeal membrane oxygenation (ECMO) in 162 patients. Respiratory support via veno-venous ECMO was provided in 63 patients. The pump was used as a ventricular assist device in eight patients. Median supporting time was 5.5 (0.2-69) days and the weaning rate was 72.5%. The discharge home rate was 62% in the pulmonary group versus 55% in the cardiac group. Extracorporeal cardiopulmonary resuscitation was carried out in 24 patients (10%) with a survival to discharge of rate of 37.5%. About 106 (47%) children experienced no complications, while 33% suffered bleeding requiring blood transfusion or surgical intervention. Three patients suffered a fatal cerebral event. Renal replacement therapy was performed in 28% and pump or oxygenator exchange in 26%. Multivariable analysis identified system exchange (OR 1.94), kidney failure (OR 3.43), and complications on support (OR 2.56) as risk factors for dismal outcome. This novel diagonal pump has demonstrated its efficacy in all kinds of mechanical circulatory and respiratory support, revealing good survival rates.

Suspicion of respiratory tract infection with multidrug-resistant Enterobacteriaceae: epidemiology and risk factors from a Paediatric Intensive Care Unit.

BACKGROUND: Multidrug-resistant (MDR) infections are a serious concern for children admitted to the Paediatric Intensive Care Unit (PICU). Tracheal colonization with MDR Enterobacteriaceae predisposes to respiratory infection, but underlying risk factors are poorly understood. This study aims to determine the incidence of children with suspected infection during mechanical ventilation and analyses risk factors for the finding of MDR Enterobacteriaceae in tracheal aspirates. METHODS: A retrospective single-centre analysis of Enterobacteriaceae isolates from the lower respiratory tract of ventilated PICU patients from 2005 to 2014 was performed. Resistance status was determined and clinical records were reviewed for potential risk factors. A classification and regression tree (CRT) to predict risk factors for infection with MDR Enterobacteriaceae was employed. The model was validated by simple and multivariable logistic regression. RESULTS: One hundred sixty-seven Enterobacteriaceae isolates in 123 children were identified. The most frequent isolates were Enterobacter spp., Klebsiella spp. and E.coli. Among these, 116 (69%) isolates were susceptible and 51 (31%) were MDR. In the CRT analysis, antibiotic exposure for ≥ 7 days and presence of gastrointestinal comorbidity were the most relevant predictors for an MDR isolate. Antibiotic exposure for ≥ 7 days was confirmed as a significant risk factor for infection with MDR Enterobacteriaceae by a multivariable logistic regression model. CONCLUSIONS: This study shows that critically-ill children with tracheal Enterobacteriaceae infection are at risk of carrying MDR isolates. Prior use of antibiotics for ≥ 7 days significantly increased the risk of finding MDR organisms in ventilated PICU patients with suspected infection. Our results imply that early identification of patients at risk, rapid microbiological diagnostics and tailored antibiotic therapy are essential to improve management of critically ill children infected with Enterobacteriaceae.

Management of postoperative junctional ectopic tachycardia in pediatric patients: a survey of 30 centers in Germany, Austria, and Switzerland.

Postoperative junctional ectopic tachycardia (JET) is a frequent complication after pediatric cardiac surgery. Current recommendations on how and when to treat JET are inconsistent. We evaluated the management strategies of postoperative JET in German-speaking countries. We sent an online survey to 30 centers of pediatric cardiology that perform surgery for congenital heart defects in Germany (24), Austria (4), and Switzerland (2). The survey asked 18 questions about how and in what treatment sequence postoperative JET was managed. All 30 centers completed the survey (100% return rate). There was general agreement that the management of JET is based on administration of antiarrhythmic drugs, body surface cooling, and temporary pacing. Many centers presented treatment algorithms based on published literature, all centers named amiodarone as the first drug of choice. Significant disagreement was found concerning the timing and sequential order of additional therapeutic measures and particularly about the dosing of amiodarone and the role of R-wave synchronized atrial pacing. CONCLUSION: This survey reveals that from center to center, the treatment of postoperative JET may vary substantially. Future work should focus on those treatment modalities where a high rate of variation is found. Such studies may be of value to achieve commonly adopted treatment recommendations. What is known: • Treatment of postoperative junctional ectopic tachycardia is predominantly based on administration of antiarrhythmic drugs, therapeutic cooling, and temporary pacing. • Amiodarone is the antiarrhythmic drug of choice in this context. What is new: • Dosing and duration of administration of amiodarone differ relevantly from center to center. • The sequential order of drug administration, therapeutic cooling, and pacing is not consistent.

Severe Upper Airway Obstruction After Intraoperative Transesophageal Echocardiography in Pediatric Cardiac Surgery: A Retrospective Analysis.

OBJECTIVES: The aim of this study was to evaluate if there is a correlation between the use of intraoperative transesophageal echocardiography and an increased rate of extubation failure and to find other risk factors for severe upper airway obstructions after pediatric cardiac surgery. DESIGN: Retrospective analysis. SETTING: Cardiac PICU. PATIENTS: Patients 24 months old or younger who underwent surgery for congenital heart disease with cardiopulmonary bypass were retrospectively enrolled and divided into two groups depending on whether they received an intraoperative transesophageal echocardiography or not. We analyzed all cases of early reintubations within 12 hours after extubation due to a documented upper airway obstruction. INTERVENTION: None. MEASUREMENTS AND MAIN RESULTS: From a total of 424 patients, 12 patients (2.8%) met our criteria of early reintubation due to upper airway obstruction. Ten of 207 children in the transesophageal echocardiography group had to be reintubated, whereas only two of the 217 children in the control group had to be reintubated (4.8% vs 0.9%; p = 0.018). Logistic regression analysis showed a significant correlation between use of intraoperative transesophageal echocardiography and extubation failure (odds ratio, 5.64; 95% CI, 1.18-27.05; p = 0.030). There was no significant relationship among sex (odds ratio, 4.53; 95% CI, 0.93-22.05; p = 0.061), weight (odds ratio, 1.07; 95% CI, 0.82-1.40; p = 0.601), duration of surgery (odds ratio, 1.04; 95% CI, 0.74-1.44; p = 0.834), duration of mechanical ventilation (odds ratio, 1.00; 95% CI, 0.99-1.00; p = 0.998), and occurrence of trisomy 21 (odds ratio, 3.47; 95% CI, 0.83-14.56; p = 0.089). CONCLUSIONS: Although the benefits of intraoperative transesophageal echocardiography during pediatric cardiac surgery are undisputed, it may be one factor which could increase the rate of severe upper airway obstruction after extubation with the need for reintubation. We suggest to take precautions before extubating high-risk patients, especially in young male children with genetic abnormalities after cardiac surgery with cardiopulmonary bypass.

Elevated intrathoracic CO2 pressure during thoracoscopic surgery decreases regional cerebral oxygen saturation in neonates and infants-A pilot study.

BACKGROUND: Intraoperative hypercapnia and acidosis are risk factors during thoracoscopy in neonates and infants. METHODS: In a prospective pilot study, we evaluated the effects of thoracoscopy in neonates and infants on cerebral microcirculation, oxygen saturation, and oxygen consumption. Regional cerebral oxygen saturation and blood flow were measured noninvasively using a new device combining laser Doppler flowmetry and white light spectrometry. Additionally, cerebral fractional tissue oxygen extraction and approximated oxygen consumption were calculated. Fifteen neonates and infants undergoing thoracoscopy were studied using the above-mentioned method. The chest was insufflated with carbon dioxide with a pressure of 2-6 mm Hg. Single lung ventilation was not used. As control group served 15 neonates and infants undergoing abdominal surgery. RESULTS: Data are presented as median and range. The 95% confidence intervals for differences of means (95% CI) are given for the mean difference from baseline values. We observed a correlation between intrathoracic pressure exceeding 4 mm Hg and transient decrease in regional cerebral oxygen saturation of 12.7% (95% CI: 9.7-17.2, P<.001). Peripheral oxygen saturation was normal at the same time. Intraoperative increase in arterial paCO2 (median maximum value: 48.8 mm Hg, range: [36.5-65.4]; 95% CI: -16.0 to -3.0, P=.002) and decrease in arterial pH (median minimum value: 7.3, range: [7.2-7.4]; 95% CI: 0.04-0.12, P=.008) were observed during thoracoscopy with both parameters recovering at the end of the procedure. Periods of regional cerebral oxygen saturation below 20% from baseline were significantly more frequent during thoracoscopy as compared to the control group (median maximum value: 1.3%min/h, range: [0.0-66.2] vs median maximum value: 0.0%min/h, range: [0.0-4.0]; 95% CI: -16.6 to -1.1, P=.028). CONCLUSION: We suggest that thoracoscopic surgery in neonates and infants, although generally safe, may be associated with a decrease in regional cerebral oxygen saturation correlating with the applied intrathoracic pressure. According to our data an inflation pressure >4 mm Hg should be avoided during thoracoscopic surgery.

Nurse-driven analgesia and sedation in pediatric patients with univentricular hearts requiring extracorporeal life support after first-stage palliation surgery: A pilot study.

BACKGROUND: Few data are available regarding requirements of sedation and analgesia in children during extracorporeal life support. AIMS: The aim of this study was to evaluate if children with functionally univentricular hearts on extracorporeal life support after first-stage palliation surgery have higher requirement of analgesics and sedatives compared with children without extracorporeal life support using a goal-directed nurse-driven analgesia and sedation protocol. METHODS: This prospective observational matched case-control pilot study was conducted at a cardiac pediatric intensive care unit of a tertiary referral center. Seventeen patients with functionally univentricular hearts including hypoplastic left heart syndrome who were on extracorporeal life support after first-stage palliation surgery were enrolled from July 2012 to January 2017. Seventeen matched patients served as controls. Doses of morphine, midazolam, clonidine, and muscle relaxants as well as sedation scores (COMFORT behavior scale and the nurse interpretation of sedation scale) were assessed according to a nurse-driven protocol every 8 hours up to 120 hours after first-stage palliation surgery. RESULTS: Sedation scores were equal in the extracorporeal life support group and in the control group at most points in time. There was no significant difference in cumulative doses of morphine and midazolam. However, children of the extracorporeal life support group received higher doses of midazolam and morphine at some points in time. CONCLUSION: A nurse-driven protocol for analgesia and sedation of children with extracorporeal life support is feasible. Patients with extracorporeal life support do not need deeper sedation levels and have not higher cumulative sedation requirements than children without extracorporeal life support.

Nurse-driven pediatric analgesia and sedation protocol reduces withdrawal symptoms in critically ill medical pediatric patients.

BACKGROUND: While several analgesia and sedation guidelines and protocols have been developed and implemented for adults, there is still little evidence of clinical use of analgesia and sedation protocols and the impact on withdrawal symptoms in critically ill children. OBJECTIVE: The aim of this study was to evaluate the effects of a nurse-driven goal-directed analgesia and sedation protocol for mechanically ventilated pediatric patients (pASP) on duration of mechanical ventilation, pediatric intensive care unit (PICU) length of stay, total doses of opioids and benzodiazepines, and occurrence of withdrawal symptoms. PATIENTS AND METHODS: This is a before and after protocol implementation study in a 14-bed medical-surgical-cardiac pediatric intensive care unit at a university children's hospital. A total of 337 medical pediatric patients requiring mechanical ventilation with PICU length of stay for at least 24 h were included. Prior to implementation of the protocol, analgesia and sedation was managed by the attending physician's order. Afterwards, postimplementation, nurses managed analgesia and sedation following a pASP, including COMFORT 'behavioral' Scale, Nurse Interpretation Sedation Scale, and Sophia Observation Withdrawal Symptoms Scale. RESULTS: One hundred and sixty-five patients were included in the 15-month period before and 172 patients were included in the 15-month period after implementation of the pASP. Median duration of mechanical ventilation was 2.02 (0.96-25.0) days in the group preceding protocol implementation and 1.71 (0.96-66.0) days afterwards (P = 0.23). Median PICU length of stay was 5.8 (1-37.75) days in the preimplementation and 5.0 (1-120) days in the postimplementation group (P = 0.14). Total doses of opioids and benzodiazepines were 3.9 mg·kg(-1) ·day(-1) (0.1-70) vs 3.1 mg·kg(-1) ·day(-1) (0.05-56); P = 0.38 and 5.9 mg·kg(-1) ·day(-1) (0-82.0) vs 4.2 mg·kg(-1) ·day(-1) (0-66); P = 0.009 after implementation. Incidence of withdrawal was significantly lower over the postimplementation period (12.8% vs 23.6%; P = 0.005). CONCLUSION: Implementation of a nurse-driven pASP reduced the total dose of benzodiazepines and the occurrence of withdrawal symptoms significantly.

Refractory congestive heart failure following delayed pericardectomy in a 12-year-old child with Mulibrey nanism due to a novel mutation in TRIM37.

UNLABELLED: Mulibrey nanism (MUL) is a rare autosomal recessive disorder with severe primordial growth retardation and multiorgan involvement, caused by mutations in TRIM37. Early clinical detection is important since more than 50 % of the patients develop congestive heart failure. We report a 12-year-old patient who presented in infancy with severe growth retardation, dysmorphic features, and cleft palate. Clinical diagnosis of MUL was established at the age of 5 years. Postmortem, molecular diagnostic confirmed MUL as a novel 1-bp deletion (c.1233delA) in exon 14 of the TRIM37 coding region. Cardiac examination at the age of 6 years revealed constrictive pericarditis with significant elevation of atrial filling pressures, consecutive hepatomegaly, and protein loosing enteropathy. Since the parents refused pericardectomy, surgery was delayed until the age of 12 years, when congestive heart failure deteriorated. Despite pericardectomy, the boy died from persistent right heart failure. CONCLUSION: Our report underlines the necessity of early clinical diagnosis of Mulibrey nanism. Careful cardiologic examination is required to detect constrictive pericarditis, which is a major factor of mortality in these patients. Pericardectomy should be performed early, to avoid sequelae of persisting congestive heart failure.

Recurrent pulmonary haemorrhage in an infant with tetralogy of Fallot and absent pulmonary valve: interventional treatment by coil occlusion of systemic-to-pulmonary collateral arteries.

Although clinically silent in the majority of cases, enlarged bronchial arteries or systemic-to-pulmonary collateral arteries may complicate congenital heart disease in infants, causing significant left-to-right shunting with subsequent pulmonary congestion and respiratory compromise. So far, pulmonary haemorrhage, a well-known complication in older patients with cyanotic congenital heart disease, has not been described in infancy. We describe the case of a 6-month-old girl with tetralogy of Fallot and absent pulmonary valve who developed haemoptysis with severe respiratory distress following corrective surgery of the cardiac malformation. High-resolution computed tomography of the thorax followed by selective angiography revealed a systemic-to-pulmonary collateral artery originating from the left internal mammary artery. Pulmonary haemorrhage stopped immediately following coil occlusion of the collateral. A second episode of pulmonary haemorrhage occurred at the age of 9 months during mechanical ventilation for treatment of pneumonia. Repeat angiography revealed two more collateral vessels. Again coil occlusion resulted in prompt resolution of pulmonary haemorrhage. According to our experience, enlarged bronchial arteries or systemic-to-pulmonary collateral arteries should be considered in infants with cyanotic heart disease with unexplained pulmonary congestion or prolonged respiratory problems.

Successful on-ECLS Repair of CDH and Omphalocele in a Newborn.

Both congenital diaphragmatic hernias (CDHs) and omphaloceles show relevant overall mortality rates as individual findings. The combination of the two has been described only sparsely in the literature and almost always with a fatal course. Here, we describe a term neonate with a rare high-risk constellation of left-sided CDH and a large omphalocele who was successfully treated on extracorporeal life support (ECLS). Prenatally, the patient was diagnosed with a large omphalocele and a left CDH with a lung volume of ∼27% and an observed to expected lung-to-head ratio of 30%. Due to respiratory insufficiency, an ECLS device was implanted. As weaning from ECLS was not foreseeable, the female infant underwent successful surgery on ECLS on the ninth day of life. Perioperative high-frequency oscillatory ventilation and circulatory and coagulation management under point-of-care monitoring were the main anesthesiological challenges. Over the following 3 days, ECLS weaning was successful, and the patient was extubated after another 43 days. Surgical treatment on ECLS can expand the spectrum of therapy in high-risk constellations if potential risks are minimized and there is close interdisciplinary cooperation.

Sedation practices during high dose rate brachytherapy for children with urogenital and perianal rhabdomyosarcoma.

BACKGROUND: A novel concept for an organ-preserving treatment of pediatric urogenital and perianal rhabdomyosarcoma includes high dose rate brachytherapy following surgical tumor resection. For the duration of the brachytherapy of 6 days plus 2-day recovery break the patients are not allowed to move and are kept under deep sedation, which can lead to difficult weaning from mechanical ventilation, withdrawal, delirium, and prolonged hospital stay. The aim of this study was to evaluate a protocol which includes a switch from fentanyl to ketamine 3 days prior to extubation to help ensure a rapid extubation and transfer from PICU. METHODS: Patients who underwent surgical tumor resection of rhabdomyosarcoma and subsequent brachytherapy were treated according to a standardized protocol. We evaluated doses of fentanyl, midazolam and clonidine, time of extubation, length of PICU stay and occurrence of withdrawal symptoms and delirium. We compared fentanyl dose at time of extubation, duration of weaning from mechanical ventilation and time to discharge from PICU with patients after isolated severe traumatic brain injury. RESULTS: Twentytwo patients (age 39.9 ± 29.8 months) were treated in our PICU to undergo brachytherapy. Extubation was performed 21.6 ± 13.5 h after the last brachytherapy session with an average fentanyl dose of 1.5 ± 0.5 µg/kg/h and patients were discharged from PICU 58.4 ± 30.3 h after extubation, which all is significantly lower compared to the control group (extubation after 88.0 ± 42.2 h, p < 0.001; fentanyl dose at the time of extubation 2.5 ± 0.6 µg/kg/h, p < 0.001; PICU discharge after 130.1 ± 148.4 h, p < 0.009). Withdrawal symptoms were observed in 9 patients and delirium in 13 patients. CONCLUSION: A standardized analgesia and sedation protocol including an opioid break, scoring systems to detect withdrawal symptoms and delirium, and tapering plans contributes to successful early extubation and discharge from PICU after long-term deep sedation.

Modified ABCDEF-Bundles for Critically Ill Pediatric Patients - What Could They Look Like?

Background and Significance: Advances in pediatric intensive care have led to markedly improved survival rates in critically ill children. Approximately 70% of those children survive with varying forms of complex chronic diseases or impairment/disabilities. Length of stay, length of mechanical ventilation and number of interventions per patient are increasing with rising complexity of underlying diseases, leading to increasing pain, agitation, withdrawal symptoms, delirium, immobility, and sleep disruption. The ICU-Liberation Collaborative of the Society of Critical Care Medicine has developed a number of preventative measures for prevention, early detection, or treatment of physical and psychiatric/psychological sequelae of oftentimes traumatic intensive care medicine. These so called ABCDEF-Bundles consist of elements for (A) assessment, prevention and management of pain, (B) spontaneous awakening and breathing trials (SAT/SBT), (C) choice of analgesia and sedation, (D) assessment, prevention and management of delirium, (E) early mobility and exercise and (F) family engagement and empowerment. For adult patients in critical care medicine, research shows significant effects of bundle-implementation on survival, mechanical ventilation, coma, delirium and post-ICU discharge disposition. Research regarding PICS in children and possible preventative or therapeutic intervention is insufficient as yet. This narrative review provides available information for modification and further research on the ABCDEF-Bundles for use in critically ill children. Material and Methods: A narrative review of existing literature was used. Results: One obvious distinction to adult patients is the wide range of different developmental stages of children and the even closer relationship between patient and family. Evidence for pediatric ABCDEF-Bundles is insufficient and input can only be collected from literature regarding different subsections and topics. Conclusion: In addition to efforts to improve analgesia, sedation and weaning protocols with the aim of prevention, early detection and effective treatment of withdrawal symptoms or delirium, efforts are focused on adjusting ABCDEF bundle for the entire pediatric age group and on strengthening families' decision-making power, understanding parents as a resource for their child and involving them early in the care of their children.