Search

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.

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.

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.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.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.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.

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. 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 postmeningitic 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 1000 -1400 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 remain high above 500 ml/24h. 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. 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 ventriculo-atrial 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.

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.

Antimicrobial stewardship programmes (ASP) are aimed at optimising antimicrobial utilization. However, only few studies have focused on paediatric intensive care units (PICU), where inappropriate antibiotic use occurs frequently. We assessed the effect and safety of a once weekly paediatric infectious disease (PID) ward round with prospective audit and feedback on antibiotic consumption in a multidisciplinary PICU. This study was conducted within 6-months periods before and after the implementation of a weekly PID-ward round. Antimicrobial management and two main recommendations per patient were discussed and documented. The primary outcome was antimicrobial utilization, measured by days of therapy (DoT) and length of therapy (LoT) per 1000 patient days (PD) for all PICU stays. Secondary outcomes included PICU mean length of stay, total mortality, infection-related mortality and cost of therapy. 1964 PD were analyzed during the pre- and 1866 PD during the post-implementation phase. Adherence to the recommendations was 79%. An 18% reduction of DoT/1000 PD was observed in the post-implementation period (p = 0.005). LoT/1000 PD decreased by 11% (p = 0.09). Meropenem and vancomycin usage were reduced by 49% (p = 0.07) and 56% (p = 0.03), respectively. We conclude, that a once weekly PID-ward round with prospective audit and feedback is safe and effective and reduces antibiotic consumption in PICUs.

OBJECTIVE Arterial hypertension (AH) is the most common toxic effect of calcineurin inhibitor (CNI)–based immunosuppression in children after liver transplantation (LT). Activation of the renal sodium chloride cotransporter (NCC) by CNIs has been described as a major cause of CNI-induced AH. Thiazides, for example, hydrochlorothiazide (HCTZ), can selectively block the NCC and may ameliorate CNI-induced AH after pediatric LT. METHODS From 2005 thru 2015 we conducted a retrospective, single-center analysis of blood pressure in 2 pediatric cohorts (each n = 33) with or without HCTZ in their first year after LT. All patients received CNI-based immunosuppression. According to AAP guidelines, AH was defined as stage 1 and stage 2. Cohort 1 received an HCTZ-containing regimen to target the CNI-induced effect on the NCC, leading to AH. Cohort 2 received standard antihypertensive therapy without HCTZ. RESULTS In children who have undergone LT and been treated with CNI, AH overall was observed less frequently in cohort 1 vs cohort 2 (31% vs 44%; ns). Moreover, severe AH (stage 2) was significantly lower in cohort 1 vs 2 (1% vs 18%; p < 0.001). Multivariate analysis revealed HCTZ as the only significant factor with a protective effect on occurrence of severe stage 2 AH. While monitoring safety and tolerability, mild asymptomatic hypokalemia was the only adverse effect observed more frequently in cohort 1 vs 2 (27% vs 3%; p = 0.013). CONCLUSIONS Targeting NCC by HCTZ significantly improved control of severe CNI-induced AH and was well tolerated in children who underwent LT. This effect may reduce the risk of long-term end-organ damage and improve quality of life.

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.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.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, p0,001) and for complete blood sampling (2,62 ml±0,32 vs. 2,11 ml±0,35, p0,001) 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).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.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.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.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.Die Patientencharakteristik beider Gruppen zeigte keine relevanten Unterschiede. Blut, das für Blutgasanalysen (0,52 ml±0,16 vs. 0,38 ml±0,12, p0,001) und für vollständige Blutentnahmen (2,62 ml±0,32 vs. 2,11 ml±0,35, p0,001) 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).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.Wir konnten zeigen, dass es möglich ist, den Blutverlust durch Blutentnahme mit einem einfachen Schulungsprogramm für Mitarbeiter auf der Intensivstation deutlich zu reduzieren.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.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.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, p0,001) and for complete blood sampling (2,62 ml±0,32 vs. 2,11 ml±0,35, p0,001) 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).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.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.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.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.Die Patientencharakteristik beider Gruppen zeigte keine relevanten Unterschiede. Blut, das für Blutgasanalysen (0,52 ml±0,16 vs. 0,38 ml±0,12, p0,001) und für vollständige Blutentnahmen (2,62 ml±0,32 vs. 2,11 ml±0,35, p0,001) 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).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.Wir konnten zeigen, dass es möglich ist, den Blutverlust durch Blutentnahme mit einem einfachen Schulungsprogramm für Mitarbeiter auf der Intensivstation deutlich zu reduzieren.

Aim: Benzodiazepines like midazolam are commonly used for long-term sedation of critically ill children requiring mechanical ventilation. Tolerance to midazolam may occur in these patients resultin...

Purpose Analgesia and sedation protocols are reported to reduce the requirement of sedative and analgesic agents, duration of mechanical ventilation, and length of pediatric intensive care unit (PICU) stay. However, these studies often were conducted based on inhomogeneous cohorts. The aim of this study was the evaluation of a nurse-driven analgesia and sedation protocol in a homogenous population of infants following corrective surgery for tetralogy of Fallot (TOF). Design and methods This retrospective analysis was conducted in a cardiac PICU of a tertiary referral center. Two cohorts of patients who underwent corrective surgery for TOF below the age of 7 months, were retrospectively evaluated before and after implementation of a nurse-driven analgesia and sedation protocol. We compared peak and cumulative doses of midazolam, morphine, and clonidine, length of PICU stay and time on mechanical ventilation. Results A total of 33 patients were included in the preimplementation period and 32 during the postimplementation period. Implementation of the nurse-driven analgesia and sedation protocol had no effect on time on mechanical ventilation (72 hr [24-141] vs. 49 hr [24-98]), but significantly on length of PICU stay (7 days [5-14] vs. 5 days [4-7]). Cumulative doses of midazolam (7.37 mg/kg [4.70-17.65] vs. 5.0 mg/kg [2.70-9.12]) as well as peak doses of midazolam (0.22 mg·kg-1 ·hr-1 [0.20-0.33] vs. 0.15 mg·kg-1 ·hr-1 [0.13-0.20]) and morphine (50.0 µg·kg-1 ·hr-1 [39.7-79.9] vs. 42.5 µg·kg-1 ·hr-1 [29.7-51.8]) were significantly reduced. The postimplemantation group showed no increase in postoperative complications and adverse events. Practice implications The implementation of a nurse-driven analgesia and sedation protocol is safe in infants following corrective surgery for TOF. It reduces significantly the length of PICU stay, cumulative and peak doses of midazolam and peak doses of morphine.

Essentials Bleeding complications during congenital heart disease surgery in neonatal age are very common. We report the perioperative incidence of acquired von Willebrand syndrome (aVWS) in 12 infants. aVWS was detected in 8 out of 12 neonates and infants intraoperatively after cardiopulmonary bypass. Ten patients received von Willebrand factor concentrate intraoperatively and tolerated it well. SUMMARY: Background Cardiac surgery of the newborn and infant with complex congenital heart disease (CHD) is associated with a high rate of intraoperative bleeding complications. CHD-related anatomic features such as valve stenoses or patent arterial ducts can lead to enhanced shear stress in the blood stream and thus cause acquired von Willebrand syndrome (aVWS). Objective To evaluate the intraoperative incidence and impact of aVWS after cardiopulmonary bypass (CPB) in neonates and infants with complex CHD. Patients/Methods We conducted a survey of patients aged < 12 months undergoing complex cardiac surgery in our tertiary referral center. Twelve patients, whose blood samples were analyzed for aVWS before CPB and immediately after discontinuation of CPB on a routine basis, were eligible for the analysis. von Willebrand factor antigen (VWF:Ag), ristocetin cofactor activity (VWF:RCo), collagen binding activity (VWF:CB), VWF:multimers and factor VIII activity (FVIII:C) were determined. Results aVWS was diagnosed by VWF multimer analysis in 10 out of 12 patients (83%) prior to surgery and intraoperatively at the end of CPB in 8 out of 12 patients (66%). Ten patients received VWF/FVIII concentrate intraoperatively as individual treatment attempts during uncontrolled bleeding. They tolerated it well without intraoperative thrombotic events. One patient suffered a transient postoperative cerebral sinuous vein thrombosis. Conclusions aVWS is of underestimated incidence in complex CHD surgery. These data may offer a new approach to reduce the risk of severe bleedings and to achieve hemostasis during high-risk pediatric cardiac surgery by tailoring the substitution with von Willebrand factor concentrate.

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.

Technological innovations in pediatric extra-corporeal 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.

SummaryBackground 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.

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.Retrospective analysis.Cardiac PICU.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.None.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).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.

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 4 mm Hg should be avoided during thoracoscopic surgery.

OBJECTIVES Cerebral protection is a major issue in the treatment of infants with complex congenital heart disease. We tested a new device combining tissue spectrometry and laser Doppler flowmetry for non-invasive determination of cerebral oxygen metabolism following cardiac surgery in infants. METHODS We prospectively measured regional cerebral oxygen saturation cSO 2 and microperfusion (rcFlow) in 43 infants 12-24 h following corrective ( n = 30) or palliative surgery ( n = 13) of congenital heart defects. For comparison, cerebral blood flow (CBF) was determined by colour duplex sonography of the extracranial cerebral arteries. Cerebral fractional tissue oxygen extraction, approximated cerebral metabolic rate of oxygen (aCMRO 2 ) and cerebral metabolic rate of oxygen (CMRO 2 ) were calculated. RESULTS cSO 2 was lower [54.6% (35.7-64.0) vs 59.7% (44.5-81.7); P

Background/Purpose Among the options for esophageal replacement in long-gap esophageal atresia (LGEA), gastric transposition (GT) is accessible for an endoscopic approach. Here we report a novel technique and functional results after laparoscopic-assisted gastric transposition (LAGT), including pyloric dilatation in patients with LGEA. Methods Retrospective analysis of 14 children undergoing LAGT. Surgical steps included the release of the gastrostomy, transumbilical ante-situ section of the stomach including pyloric balloon-dilation, and laparoscopically controlled transhiatal retromediastinal blunt dissection followed by LAGT for cervical anastomosis to the proximal esophagus. Results The median age at LAGT was 110 days (33–327 days), bodyweight 5.3 kg (3.1–8.3 kg). Operation time was 255 min (180–436 min); one conversion was necessary. The duration of ventilation was 4 days (1–14 days). Postpyloric feeding was started after 2 days, and oral feeding after 13 days. Complications were recurrent pleural effusion or pneumothorax and transient Horner syndrome or transient incomplete paresis of the recurrence nerve. After a median follow-up of 60 months (13–240 months), all children have a patent upper GI tract, show weight gain, and are fed without delayed gastric emptying, dumping, or reflux. Severe (n = 1) or mild (n = 2) anastomotic or pyloric (n = 5) stenosis was resolved with endoscopic dilatations. Conclusions Functional outcome after LAGT in patients with LGEA is good. The laparoscopic retromediastinal dissection preserves thoracal structures and increases patients' safety. The technique of pyloric dilatation might also prevent dumping syndrome. Type of Study Case Series with no Comparison Group. Level of Evidence Level IV.

Aim: Midazolam like other benzodiazepines is supposed to be neurotoxic in small children and to represent a risk factor for the development of delirium. The aim of this study was to evaluate whethe...

Data are still lacking about the use of terlipressin or vasopressin in the treatment of pediatric patients who are in a state of therapy-refractory shock. The aim of this study was to evaluate the effect of terlipressin on hemodynamics in children with distributive shock and to describe any severe side effects. Consecutive patients (n = 20) with catecholamine-resistant distributive shock who were treated with terlipressin were retrospectively enrolled in this study. We analyzed response in terms of mean arterial blood pressure, heart rate, vasoactive inotropic score (VIS), urinary output, and serum lactate. The hemodynamics of 12 children significantly improved within 6 h of commencing terlipressin (mean blood pressure increase of ≥20 % without VIS increase, or mean blood pressure increase of ≥10 % with VIS decrease of ≥10 %). The hemodynamics of eight patients did not improve, regardless of treatment dosage or duration. More children died in the responders group (n = 7 [58.3 %]) than in the non-responders group (n = 2 [25.0 %]), but this was not statistically significant. Two patients (one in each group) who received high dosages of terlipressin developed rhabdomyolysis. One case of Takotsubo cardiomyopathy was observed, which could be related to terlipressin. Although treatment with terlipressin resulted in rapid positive hemodynamic responses in some children, it did not seem to have a positive effect in other pediatric patients. Therefore, the possible benefits of terlipressin should be always weighed against potential severe adverse effects.

Background Choosing the correct insertion depth of tracheal tubes is crucial for successful airway management in paediatrics. Currently used formulas are based on patient characteristics such as age, body weight and height. The aim of the study is to devise and evaluate more suitable body surface area based diagrams for predicting the correct tracheal insertion depth. Methods Calculated insertion depth according to currently used formulas, primary insertion depth and insertion depth corrected by chest radiography (‘gold standard') were collected from 237 children. Age, body weight, height and body surface area were noted. Body surface area based diagrams were devised and prospectively evaluated in another set of 123 paediatric patients. Results Tracheal tube position according to currently used formulas had to be corrected in 37% of all intubations. New body surface area based diagrams were created. In 20.3%, depth of the tracheal tube had to be corrected according to the new body surface area based diagrams. Conclusions The body surface area based diagrams may be a reliable tool for predicting the correct tracheal insertion depth in children.

Der padiatrische Teil der S3-Leitlinie „Analgesie, Sedierung und Delirmanagement in der Intensivmedizin“ hat das Ziel, Kolleginnen und Kollegen, die in die Betreuung von Neugeborenen und Kindern auf Intensivstationen involviert sind, eine umfassende evidenz- und konsensus-basierte Handlungsgrundlage zu geben. Zugleich sollen Impulse fur weitere Forschungsaktivitaten gegeben werden. In der Uberarbeitung von 2015 werden klinisch relevanten Schlusselfragen entsprechend der aktuellen Studienlage aus pflegerischer und arztlicher Sicht neu beleuchtet.

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.

Early differentiation between sepsis and systemic inflammatory response syndrome (SIRS) is useful for therapeutic management in neonates and infants after surgery.To compare the early (first 2 days) diagnostic value of interleukin-6 (IL-6), procalcitonin (PCT), and C-reactive protein (CRP) after surgery in the differentiation of subsequent SIRS and septic complications.IL-6, PCT, and CRP were measured 0, 24, and 48 hours after surgery in neonates and infants with clinical suspicion of postoperative sepsis. Sensitivity, specificity, and predictive values for SIRS/septic complications were calculated.A total of 31 out of 205 neonates and infants showed clinical signs for postoperative sepsis and underwent sepsis work-up. Nine patients developed septic complications, sixteen patients met criteria for SIRS, and six patients showed an uneventful postoperative course during the first five postoperative days. IL-6, PCT, and CRP levels increased in all subgroups after surgery and were significantly higher in the sepsis group (p 0.05). IL-6 peaked immediately, CRP at 24 to 48 hours, and PCT at 24 hours after surgery. Sensitivity and specificity (area under the curve) for IL-6 (cutoff 673 ng/dL) were 94.4 and 75% (86.2%), for CRP (cutoff 1.48 mg/dL) 76.2 and 75.0% (88.1%), and for PCT (cutoff 16.1 mg/L) 66.7 and 57.1% (65.6%).IL-6 appears to be an early marker for severe bacterial infections with high sensitivity. IL-6 and CRP were the most reliable markers for the discrimination between SIRS and sepsis within the postoperative period.

The purpose of this study was to assess the use of continuous epidural analgesia in pediatric patients undergoing major abdominal tumor surgery.Children undergoing major abdominal tumor surgery at our institution between 2008 and 2012 (n=40) received continuous epidural analgesia via an epidural catheter. Surgical trauma scores, pain scores, and clinical data of the children were compared to a pair-matched historical control group operated on between 2002 and 2007 without epidural analgesia.Pain levels in the study group on day 1 and 3 after surgery were lower compared to the control group. The differences did, however, not reach statistical significance (p=0.15 and 0.09). Children in the study group received significantly fewer additional doses of piritramide or morphine (45% versus 82%, p0.001). Despite significantly higher surgical trauma scores in the study group (p=0.018), there were no statistical differences regarding clinical parameters, such as mechanical ventilation time, time on intensive care unit, and total hospital stay. There were no catheter-related complications.Continuous epidural analgesia is beneficial for children undergoing complex abdominal tumor surgery with regard to pain levels, postoperative recovery, and general clinical course. Expertise of the managing team, a careful patient selection, and a continuous quality assessment are essential for success.

Intraoperative hypercapnia and acidosis are risk factors during thoracoscopy in neonates and infants.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.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 paCOWe 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 pressure4 mm Hg should be avoided during thoracoscopic surgery.

Drug rotation to prevent opioid tolerance is well recognized in chronic pain management. However, ketamine infusion as a counter measure for opioid tolerance is rarely described in mechanically ventilated children developing tolerance from prolonged opioid infusion. We performed a retrospective study in a 14-bed medical-surgical-cardiac pediatric intensive care unit. Thirty-two mechanically ventilated children who had developed tolerance from prolonged intravenous infusion of opioids received a continuous intravenous infusion of ketamine as an opioid substitute for more than 2 days, scheduled in a drug rotation protocol. Thirty-two children (median age 2.5 years, range 0.1–16.0; weight 11.2 kg [3.8–62.0]) were included. Patients had received continuous intravenous infusion of opioids and benzodiazepines for 16.0 days (4.0–34.0) when drug rotation was started. The median dose of continuous intravenous infusion of ketamine was 4.0 mg·kg−1·h−1 (1.8–6.0) and the median duration was 3.0 days (2.0–6.0). After having restarted opioids, fentanyl doses were significantly lower compared with the time before the drug rotation began (after, 2.9 µg·kg−1·h−1 [0.8–4.9] vs before, 4.15 µg·kg−1·h−1 [1.2–10.0]; p

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.

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.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.

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.

Background We demonstrated recently that the implementation of a nurse-driven analgesia and sedation protocol (pediatric analgesia and sedation protocol [pASP]) for mechanically ventilated nonpostsurgical patients reduces the total dose of benzodiazepines and the withdrawal symptoms significantly. It has not been investigated if these results can also be expected in the group of patients undergoing surgery. Objectives To evaluate the effects of the pASP in mechanically ventilated postsurgical children regarding drug dosage, duration of mechanical ventilation, length of stay, and rate of withdrawal symptoms. Methods This is a two-phase prospective observational control study. The preimplementation group was managed by the physician's order and the postimplementation group was managed with the pASP including COMFORT-B, nurse interpretation of sedation, and Sophia observation withdrawal symptoms scale scoring. Measurements and Main Results One hundred and sixteen patients were included before and one hundred and ten patients after implementation. The pASP had no effect on length of pediatric intensive care unit stay or duration of mechanical ventilation. The protocol reduced total (5.0 mg/kg [0.5–58.0] vs 4.0 mg/kg [0.0–47.0]; p = 0.021) and daily doses (4.4 mg/kg/d [1.1–33.9] vs 2.9 mg/kg/d [0.0–9.9]; p Conclusion The nurse-driven analgesia and sedation protocol is an effective procedure to reduce the total doses of benzodiazepines and occurrence of withdrawal symptoms in postsurgical children, which are naïve to opioids or benzodiazepines.

The pathophysiology of acute kidney injury (AKI) after cardiopulmonary bypass surgery for congenital heart disease is not completely understood. The aim of this study was to carry out a prospective analysis of the diagnostic value of non-invasive monitoring of renal oxygenation and microcirculation by combining laser Doppler flowmetry and tissue spectrometry.In 50 neonates and infants who underwent repair (n = 31) or neonatal palliation (n = 19) of congenital heart disease with cardiopulmonary bypass, renal oxygenation, and microcirculatory flow, the approximate renal metabolic rate of oxygen and Doppler-based renal resistive index were determined after surgery. Correlations between these parameters and the occurrence of AKI according to the Pediatric Risk, Injury, Failure, Loss, End Stage Renal Disease criteria were investigated.Acute kidney injury occurred in 45% of patients after repair and in 32% after palliation. Renal oxygenation was significantly lower and the approximate renal metabolic rate of oxygen significantly higher in patients with AKI (P 0.05). The microcirculatory flow was significantly higher in patients with AKI after neonatal palliation (P 0.05), whereas renal resistive index was significantly higher in patients with AKI after repair (P 0.05). The sensitivity of renal oxygenation, metabolic rate of oxygen, microcirculation, and resistive index in predicting AKI was 78-80, 73-78, 64-83, and 71-74%, respectively, with a specificity of 63-65, 54-75, 64-78, and 46-74% (area under the curve: 0.73-0.75, 0.68-0.83, 0.52-0.68, and 0.60-0.75), respectively.Monitoring of renal oxygen metabolism allows early prediction of AKI in infants after cardiac surgery. In contrast, renal resistive index does not allow prediction of AKI after neonatal palliation with aortopulmonary shunt establishment.

We report a 12-year-old boy who underwent stent implantation into the left main bronchus at the age of 9.5 years for palliation of severe bronchial stenosis. He had developed complete obstruction of the stent by granulation tissue resulting in respiratory deterioration and the requirement of mechanical ventilation. The stent obstruction was treated at the age of 11.5 years by cryorecanalization. In this technique, cooling of the tip of the cryoprobe is used to induce adherence of the obstructing tissue allowing the subsequent removal of tissue particles by retraction of the probe. Under general anesthesia, a miniaturized cryoprobe was advanced via the working channel of a flexible bronchoscope. Repeat maneuvers of freezing and retraction resulted in complete recanalization of the stent. Repeat bronchoscopies 4 and 12 weeks later revealed recurrent formation of some granulation tissue which was removed by repeat cryorecanalization. Seven months after the initial procedure there was a complete patency of the stent. According to our experience, cryorecanalization is a safe and effective alternative for the treatment of stent obstruction by granulation tissue. Due to the introduction of a miniaturized probe, this method is well applicable in children since it can be performed via the working channel of a flexible pediatric bronchoscope.

Introduction: At present no evidence-based medical treatment for persistent atelectasis in pediatric non-cystic fibrosis (CF) patients is available. Method: To evaluate the use of intratracheally inst

Patients with Fontan circulation represent a significant cardiorespiratory risk during spinal surgery. We report about two patients with severe scoliosis and Fontan circulation and their successful operative treatment. The case report will be compared with the national and international literature.

Intermittent chest pain is a common symptom in adolescent patients, which can be attributed to musculoskeletal disorders in the majority of cases. While most patients have a benign course of their pain episodes, the differential diagnosis includes diseases which are associated with serious and even life-threatening complications. Therefore, careful examination and systematic diagnostic evaluation is important. We report on an adolescent, who presented with severe chest pain and distinct electrocardiographic findings caused by Mycoplasma pneumoniae myocarditis.

Little is known about which paediatric patients respond to hydrocortisone rescue therapy (HRT) with improvement of haemodynamic stability in refractory hypotension after cardiopulmonal bypass. Data were gathered retrospectively from children who received HRT in refractory hypotension after cardiopulmonary bypass in the period from 2000 to 2010. One hundred and sixty-six out of 1,273 children, 150 1 year and 16 1 year were enrolled. HRT improved haemodynamics significantly, increased blood pressure, decreased the vasoactive-inotropic score and plasma lactate concentrations in all children1 year and in 82 % (123 out of 150) of the infants1 year. Non-responders1 year were significantly younger, lighter, mostly male infants and had longer cardiopulmonary bypass support time. Serum lactate and paediatric risk of mortality score were significantly higher in non-responders at time of initiation of HRT. Mortality was significantly higher in non-responders versus responders (2.44 vs. 13.5 %; p = 0.0008). HRT caused no adverse effects like electrolyte disturbances or hyperglycaemia. HRT in refractory hypotension after paediatric cardiac surgery is safe but not all infants1 year show haemodynamic response to HRT. Non-response to HRT is associated with significantly higher mortality.

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.

At present no evidence-based medical treatment for persistent atelectasis in pediatric non-cystic fibrosis (CF) patients is available.To evaluate the use of intratracheally instilled recombinant human deoxyribonuclease (rhDNase) in intubated and ventilated pediatric patients, we performed a single-center observational study on 46 pediatric intensive care patients who had received intratracheal DNase. Patients were classified, according to radiologic findings of atelectasis (group 1) or infiltrates. As controls we examined a historical control group of 17 patients with atelectasis after cardiac surgery, who had been treated with NaCl 0.9% and matched for age and diagnosis with 21 patients from group 1 (subgroup 1a). Radiologic improvement and inflammatory markers in both serum and tracheal aspirates were measured.In group 1, 35 patients had 51 atelectases/dystelectases episodes at baseline. 67 % of patients showed radiologic signs of improvement after 24h treatment with rhDNase. In subgroup 1a, 16 patients had complete resolution of atelectases and minimal change in dystelectases after a treatment of 24 hours rhDNase, compared with the control group of 17 patients, who had 7 atelectases and 10 dystelectases at baseline and an improvement in only 1 out of 17 (6 %) patients after 24h.Intratracheal instillation of rhDNase is an effective adjunct to conservative therapy of atelectases in children. Further randomized controlled prospective studies are necessary.

Bridging bronchus (BB) is a rare, congenital bronchial anomaly that is frequently associated with congenital cardiac malformations, especially left pulmonary artery sling. It represents an anomalous bronchus to the right originating from the left main bronchus. Discrimination from other bronchial anomalies is important, since BB is frequently associated with bronchial stenoses due to abnormal cartilage rings. This case study describes the findings of bronchoscopy, bronchography and multidetector computed tomography (MDCT) in three patients. Bronchoscopy was helpful in the description of the severity and length of bronchial stenoses. However, it was not possible to establish a diagnosis of BB based on this method in two patients, since it is difficult or even impossible to differentiate the bifurcation from the pseudocarina. It was not possible to establish the correct diagnosis in all patients based on bronchography or MDCT. MDCT was able to depict the relationship of bronchial and vascular structures, which is particularly important in patients with pulmonary artery sling. Multidetector computed tomography is preferable to bronchography as it is less invasive and due to its short acquisition time it can be performed in children with severe respiratory disease. In the current authors' experience, detection of cartilage rings still requires flexible bronchoscopy.

The clinical presentation of otogenic dural sinus thrombosis (DST) as a complication of acute otitis media (AOM) can be masked by antibiotic treatment. Morning episodes of vomiting and/or headache, visual impairment and a history of AOM seem to be indicative of otogenic hydrocephalus. We therefore advocate that the MRI scans of patients with similar symptoms should be carefully studied to facilitate the early diagnosis of a potentially life-threatening complication.To describe the frequency, pathognomonic signs, clinical course and outcome of otogenic hydrocephalus and DST as complications of AOM in pediatric patients.We undertook a retrospective chart review of all pediatric patients (age 1-14 years) treated for otitis media and its complications at an academic medical center between 1999 and 2003. The main outcome measures were otologic and ophthalmologic findings and CT and MRI scans at the beginning of treatment and 3 months later.We report on five cases with otogenic DST following AOM. All but one of them presented initially with diplopia caused by otogenic hydrocephalus. In four cases the otologic complaints had already disappeared by the time of MRI confirmation of the diagnosis. Only one child was referred with severe otologic symptoms. Management included systemic antibiotics, short-term heparin anticoagulation and surgical decompression. In our cases, even after intensive i.v. antibiotic treatment, only surgery led to a significant improvement in the clinical condition.

Recombinant human deoxyribonuclease I (dornase alfa) is currently used as an inhaled mucoactive agent in the treatment of cystic fibrosis. In a randomized, placebo-controlled, double-blind clinical study in 100 infants, we investigated whether the therapeutic use of dornase alfa can be extended to ventilated, fluid-restricted children to reduce reintubation rate, ventilation duration, pediatric intensive care unit (PICU) stay, and ventilation complications. While reintubation rates were similar for dornase alfa 7% vs. placebo 9% (odds ratio, 0.77; confidence interval, 0.11-4.9), the incidence of atelectasis (6 vs. 17, respectively; P-value 0.051), median ventilation time (2.2 vs. 3.4 days, respectively; P-value 0.043), median length of PICU stay (7 vs. 8 days, respectively; P-value 0.051), and mean costs (4,830 vs. 6,320, respectively) were lower in the dornase alfa group. No adverse effects were observed, even in critically ill patients. We found that dornase alfa was beneficial and safe. Our findings also indicate that dornase alfa is possibly of value from the first day of mechanical ventilation onward, particularly when longer ventilation (>3 days) is expected in fluid-restricted children after cardiac surgery.

In our study of 136 patients with conotruncal malformations, anomalies of the subclavian arteries were present in >60% of children with monosomy 22q11. Because cervical origin of the right subclavian artery was exclusively found in patients with monosomy 22q11, this anomaly might represent a specific marker for this microdeletion.