Vascular rehabilitation in children with chronic intestinal failure reduces the risk of central-line associated bloodstream infections and catheter replacements.

BACKGROUND & AIMS: Children with chronic intestinal failure (IF) require a long-term central venous catheter (CVC) for provision of parenteral nutrition. Vascular, mechanical and infectious complications such as central line-associated bloodstream infections (CLABSI) may lead to progressive loss of venous access sites. Handling and management of CVCs therefore play an important role. Our vascular rehabilitation concept (VRC) is a core component of our intestinal rehabilitation program (IRP) and consists of an education program, optimization of skin care, catheter fixation and lock solution, and the use of hybrid technique for catheter placement. Aim of this study is to analyse the effectiveness of our VRC on CLABSI rates and need for CVC replacements. METHODS: Retrospective analysis of all children treated in our IRP that were followed up between 2018 and 2023. RESULTS: A total of 117 children with chronic IF could be included for analysis of 248864 catheter days (CD). 91 patients were referred from other hospitals (127117 CD before and 89359 CD after entry into our IRP). Children receiving primary care at our IRP (32388 CD) showed a significantly lower CLABSI and line replacement rate than patients referred from external centers (p < 0.001). After entering our IRP, CLABSI rates and need for CVC replacements per 1000 CD decreased significantly in referred patients: 1.19 to 0.26 and 1.77 to 0.59, respectively (p < 0.001). CONCLUSION: Management of paediatric chronic IF patients by an IRP with a vascular rehabilitation concept significantly lowers the rate of CLABSI episodes and the need for catheter replacements.

The effect of intraoperative cerebral oxygen desaturations on postoperative cerebral oxygen metabolism in neonates and infants a pilot study.

INTRODUCTION: Cerebral oxygen desaturation during pediatric surgery has been associated with adverse perioperative outcomes. The aim of this pilot study was to analyze the frequency and severity of intraoperative cerebral oxygen desaturations and their impact on postoperative cerebral oxygen metabolism in neonates and infants undergoing pediatric surgery. METHODS: In a prospective pilot study, intra- and postoperative regional cerebral oxygen saturation and blood flow were measured noninvasively using a device combining laser Doppler flowmetry and white-light-spectrometry. Thirty-seven consecutive neonates and infants undergoing noncardiac surgery under general anesthesia for more than 30 min and necessity for invasive arterial blood pressure monitoring were included. Patients with pre-known congenital structural heart disease or cerebral disease were excluded. Continuously brain monitor recording was started in sedated patients before induction of anesthesia (preoperative baseline) and was completed 1 h postoperatively in the PICU in sedated, intubated, and mechanically ventilated states at the PICU (postoperative state). Baseline and postoperative state for cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen were calculated. RESULTS: Seventeen (46%) of the 37 studied neonates and infants suffered from intraoperative periods of regional cerebral oxygen desaturation below 20% of the baseline (event group). Severity of cerebral desaturations was median 4.0%min/h [range 0.1-58.7; interquartile range [IQR] 0.99-21.29]. In the event group, the duration of surgery was significantly longer (median 135 min [range 11-260; IQR 113.5-167.0] vs median 46.5 min [range 11-180; IQR 30.5-159.3]; difference of -62.94; 95% confidence interval [CI] -105.17 to -20.71; p = .021). In the event group, cerebral fractional tissue oxygen extraction (median 0.41 [range 0.20-0.55; IQR 0.26-0.44] vs. median 0.27 [range 0.11-0.41; IQR 0.20-0.31]; difference of -0.11; 95% CI -0.17 to -0.05; p = .001) and approximated cerebral metabolic rate of oxygen (median 6.15 arbitrary unit [range 2.69-12.07; IQR 5.12-7.21] vs. median 4.14 arbitrary unit [range 1.78-7.86; IQR 3.82-6.31]; difference of -1.76; 95% CI -3.03 to -0.49; p = .009) were significantly higher and the cerebral regional oxygen saturation (median 58.99% [range 44.87-79.1; IQR 54.26-72.61] vs median 70.94% [range 57.9-86.13; IQR 67.07-76.59]; difference of 10.01; 95% CI 4.13-15.90; p = .002) significantly lower after surgery compared to the nonevent group. DISCUSSION: The increase of approximated cerebral metabolic rate of oxygen could indicate an elevated oxidative energy metabolism in the "stressed" brain, due to repair processes. The increased cerebral fractional tissue oxygen extraction fits with the decreased NIRS cerebral oxygenation. Our data suggest that an increase in cerebral oxygen metabolism was the cause. CONCLUSION: Cerebral oxygen desaturation during major surgery in neonates and infants is associated with early postoperative increased cerebral oxygen extraction and possibly increased cerebral oxygen metabolism.

Thoracic lymphatic anomalies in patients with univentricular hearts: correlation of morphologic findings in isotropic T2-weighted MRI with the outcome after fontan palliation.

Objectives: In this study we examined the correlation between the extent of thoracic lymphatic anomalies in patients after surgical palliation by total cavopulmonary connection (TCPC) and their outcome in terms of clinical and laboratory parameters. Materials and methods: We prospectively examined 33 patients after TCPC with an isotropic heavily T2-weighted MRI sequence on a 3.0 T scanner. Examinations were performed after a solid meal, slice thickness of 0.6 mm, TR of 2400 ms, TE of 692 ms, FoV of 460 mm, covering thoracic and abdominal regions. Findings of the lymphatic system were correlated with clinical and laboratory parameters obtained at the annual routine check-up. Results: Eight patients (group 1) showed type 4 lymphatic abnormalities. Twentyfive patients (group 2) presented less severe anomalies (type 1-3). In the treadmill CPET, group 2 reached step 7.0;6.0/8.0 vs. 6.0;3.5/6.8 in group 1 (p = 0.006*) and a distance of 775;638/854 m vs. 513;315/661 m (p = 0.006*). In the laboratory examinations, group 2 showed significantly lower levels of AST, ALT and stool calprotectin as compared to group 1. There were no significant differences in NT-pro-BNP, total protein, IgG, lymphocytes or platelets, but trends. A history of ascites showed 5/8 patients in group 1 vs. 4/25 patients in group 2 (p = 0.02*), PLE occurred in 4/8 patient in group 1 vs. 1/25 patients in group 2 (p = 0.008*). Conclusion: In the long-term follow-up after TCPC, patients with severe thoracic and cervical lymphatic abnormalities showed restrictions in exercise capacity, higher liver enzymes and an increased rate of symptoms of imminent Fontan-failure such as ascites and PLE.

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.

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.

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.

Current Knowledge Regarding Long-Term Consequences of Pediatric Intensive Care: A Staff Survey in Intensive Care Units in German-Speaking Countries.

Background: The Post Intensive Care Syndrome (PICS) describes new impairments of physical, cognitive, social, or mental health after critical illness. In recent years, prevention and therapy concepts have been developed. However, it is unclear whether and to what extent these concepts are known and implemented in hospitals in German-speaking countries. Methods: We conducted an anonymous online survey in German-speaking pediatric intensive care units on the current state of knowledge about the long-term consequences of intensive care treatment as well as about already established prevention and therapy measures. The request to participate in the survey was sent to the heads of the PICUs of 98 hospitals. Results: We received 98 responses, 54% of the responses came from nurses, 43% from physicians and 3% from psychologist, all working in intensive care. As a main finding, our survey showed that for only 31% of the respondents PICS has an importance in their daily clinical practice. On average, respondents estimated that about 42% of children receiving intensive care were affected by long-term consequences after intensive care. The existence of a follow-up outpatient clinic was mentioned by 14% of the respondents. Frequent reported barriers to providing follow-up clinics were lack of time and staff. Most frequent mentioned core outcome parameters were normal developmental trajectory (59%) and good quality of life (52%). Conclusion: Overall, the concept of PICS seems to be underrepresented in German-speaking pediatric intensive care units. It is crucial to expand knowledge on long-term complications after pediatric critical care and to strive for further research through follow-up programs and therewith ultimately improve long-term outcomes.

Revascularization of Left Subclavian to Common Carotid Artery Prepares for Covered Stent Implantation in Patients With Complex Aortic Coarctation.

Seven adult patients underwent a two-stage treatment of complex coarctation (CoA), including surgical revascularization of the left subclavian artery (LSA) to left common carotid artery (LCCA), followed by transcatheter covered stent implantation. The majority of patients (5 of 7, 71%) received 1 covered stent (covered Cheatham Platinum stent: 8 zig/45 mm [n = 2], 10 zig/60 mm [n = 1], 10 zig/65 mm [n = 1]; BeGraft: 24/48 mm [n = 2]). In 1 patient (14%), the implantation of 2 covered stents (BeGraft 20/48 mm) was necessary. During a median follow-up of 2.4 years (interquartile range, 0.1 to 4.9 years), complications occurred in 3 of 7 patients (43%), including an asymptomatic but severe stenosis of the LSA bypass (n = 1), a recoarctation with a mild endoleak (n = 1), and a severe endoleak (n = 1). Surgical revascularization of the LSA to the LCCA can successfully prepare for covered stent implantation in complex CoA in adult patients. This two-stage approach was feasible and safe with complications occurring in 3 of 7 patients (43%). All complications were managed by catheter reintervention only.

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.

Spectrum of Interventional Procedures During Hybrid Central Line Placement in Pediatric Intestinal Rehabilitation Patients With End-Stage Vascular Access.

Background: Loss of available central vein access sites for parenteral nutrition delivery represents one of the main indications for intestinal transplantation in children with intestinal failure. Placement of central venous catheters can be challenging in advanced loss of patent venous pathways. We recently described the hybrid technique (interventional plus surgical approach) of central line placement in children. The aim of this study was to describe and analyze the interventions used during the hybrid procedures regarding feasibility, safety and outcome. Methods: We retrospectively analyzed the course of all children in our intestinal rehabilitation program undergoing hybrid central line placement. We evaluated patients' conditions, interventional techniques and surgical peculiarities as well as outcome. Results: 203 children were treated in our intestinal rehabilitation program between 2010 and 2021. Due to loss of venous access, hybrid technique was performed in 53 children during 76 interventions. In 40 cases the same vessel was reused via Seldinger technique. Among the 30 ultrasound-guided new vessel punctures, 12 were performed by puncture of collateral vessels. Extended interventions due to thoracic central venous obstruction and/or thrombosis requiring additional access via a femoral vein for rehabilitation of the vascular system was performed during 29 procedures including catheter extraction (1), angioplasties (18), stent placement (1), revascularization (5) and thrombectomy (4). Placement of a central line was not possible in 6 children which eventually underwent extended thoracic/vascular surgery: in three children the previously placed catheter could not be removed, in one child, placement of a thrombectomy-catheter was not possible because of inferior vena cava occlusion, and in two children, revascularization failed. Intestinal transplantation was considered in one patient because of impending loss of vascular access. Two self-limiting minor extravasations and one intervention-associated pericardial effusion occurred. Conclusions: Hybrid interventions for central venous catheter placement and vascular rehabilitation enable a high success rate in children with intestinal failure and end-stage vascular access, circumventing the need for intestinal transplantation or advanced surgery. The relevant procedures are complex and require a foresighted and individualized approach with a wide range of interventional techniques. If performed with expertise, this combined interventional/surgical approach is feasible and safe.

Implementation and Evaluation of Resuscitation Training for Childcare Workers.

Background and Objective: Children spend a large amount of time in daycare centers or schools. Therefore, it makes sense to train caregivers well in first-aid measures in children. The aim of this study is to evaluate whether a multimodal resuscitation training for childcare workers can teach adherence to resuscitation guidelines in a sustainable way. Materials and Methods: Caregivers at a daycare center who had previously completed a first-aid course received a newly developed multimodal resuscitation training in small groups of 7-8 participants by 3 AHA certified PALS instructors and providers. The 4-h focused retraining consisted of a theoretical component, expert modeling, resuscitation exercises on pediatric manikins (Laerdal Resusci Baby QCPR), and simulated emergency scenarios. Adherence to resuscitation guidelines was compared before retraining, immediately after training, and after 6 months. This included evaluation of chest compressions per round, chest compression rate, compression depth, full chest recoil, no-flow time, and success of rescue breaths. For better comparability and interpretation of the results, the parameters were evaluated both separately and summarized in a resuscitation score reflecting the overall adherence to the guidelines. Results: A total of 101 simulated cardiopulmonary resuscitations were evaluated in 39 participants. In comparison to pre-retraining, chest compressions per round (15.0 [10.0-29.0] vs. 30.0 [30.0-30.0], p < 0.001), chest compression rate (100.0 [75.0-120.0] vs. 112.5 [105-120.0], p < 0.001), correct compression depth (6.7% [0.0-100.0] vs. 100.0% [100.0-100.0], p < 0.001), no-flow time (7.0 s. [5.0-9.0] vs. 4.0 s. [3.0-5.0], p < 0.001), success of rescue breaths (0.0% [0.0-0.0] vs. 100.0% [100.0-100.0], p < 0.001), and resuscitation score were significantly improved immediately after training (3.9 [3.2-4.9] vs. 6.3 [5.6-6.7], p < 0.001). At follow-up, there was no significant change in chest compression rate and success of rescue breaths. Chest compressions per round (30.0 [15.0-30.0], p < 0.001), no-flow time (5.0 s. [4.0-8.0], p < 0.001), compression depths (100.0% [96.7-100.0], p < 0.001), and resuscitation score worsened again after 6 months (5.7 [4.7-6.4], p = 0.03). However, the results were still significantly better compared to pre-retraining. Conclusion: Our multimodal cardiopulmonary resuscitation training program for caregivers is effective to increase the resuscitation performance immediately after training. Although the effect diminishes after 6 months, adherence to resuscitation guidelines was significantly better than before retraining.

A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment. CASE PRESENTATION: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months. CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.

Implementation of a Delirium Bundle for Pediatric Intensive Care Patients.

BACKGROUND AND OBJECTIVE: Delirium represents the most common form of acute cerebral dysfunction in critical illness. The prevention, recognition, and treatment of delirium must become the focus of modern pediatric intensive care, as delirium can lead to increased morbidity and mortality. The aim of this study is to evaluate the impact of a delirium bundle consisting of mainly non-pharmacological measures. MATERIAL AND METHODS: This is a pre-/post-implementation study in an interdisciplinary pediatric intensive care unit of a tertiary care university hospital. In the pre-implementation period, pediatric intensive care delirium was monitored using the Sophia Observation withdrawal Symptoms and Pediatric Delirium scale. After introduction of a delirium bundle consisting of non-pharmacological prevention and treatment measures a period of 4 months was interposed to train the PICU staff and ensure that the delirium bundle was implemented consistently before evaluating the effects in the post-implementation period. Data collection included prevalence of delirium and withdrawal, length of PICU stay, duration of mechanical ventilation, and cumulative dose of sedatives and analgesics. RESULTS: A total of 792 critically ill children aged 0-18 years were included in this study. An overall delirium prevalence of 30% was recorded in the pre-implementation group and 26% in the post-implementation group (p = 0.13). A significant reduction in the prevalence of pediatric delirium from was achieved in the subgroup of patients under 5 years of age (27.9 vs. 35.8%, p = 0.04) and in patients after surgery for congenital heart disease (28.2 vs. 39.5%, p = 0.04). Young age, length of PICU stay, and iatrogenic withdrawal syndrome were found to be risk factors for developing delirium. CONCLUSIONS: Based on a validated delirium monitoring, our study gives new information regarding the prevalence of pediatric delirium and the characteristics of intensive care patients at risk for this significant complication. Especially young patients and patients after surgery for congenital heart disease seem to benefit from the implementation of non-pharmacological delirium bundles. Based on our findings, it is important to promote change in pediatric intensive care-toward a comprehensive approach to prevent delirium in critically ill children as best as possible.

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.

Severe adverse events in children with tracheostomy and home mechanical ventilation - Comparison of pediatric home care and a specialized pediatric nursing care facility.

BACKGROUND: Advances in medical care and ventilator technologies increase the number of children with tracheostomy and home mechanical ventilation (HMV). Data on severe adverse events in home care and in specialized nursing care facilities are limited. PATIENTS AND METHODS: Retrospective analysis of incidence and type of severe adverse events in children with tracheostomy and HMV in home care compared to a specialized nursing care facility over a 7-year period. RESULTS: 163.9 patient-years in 70 children (home care: 110.7 patient-years, 24 patients; nursing care facility: 53.2 patient-years, 46 patients) were analyzed. In 34 (48.6%) patients tracheostomy was initiated at the age of <1 year. 35 severe adverse events were identified, incidence of severe adverse events per patient-year was 0.21 (median 0.0 (0.0-3.0)). We observed no difference in the rate of severe adverse events between home care and specialized nursing care facility (0.21 [y-1]; median 0.0 (0.0-3.0) versus 0.23 [y-1]; median 0.0 (0.0-1.6); p = 0.690), however, significantly more tracheostomy related incidents and infections occurred in the home care setting. Young age (<1 year) (Odds ratio 3.27; p = 0.045) and feeding difficulties (nasogastric tubes and percutaneous endoscopic gastrostomy) (Odds ratio 9.08; p = 0.016) significantly increased the risk of severe adverse events. Furthermore, the rate of severe adverse events was significantly higher in patients with a higher nursing score. CONCLUSION: Pediatric home mechanical ventilation via tracheostomy is rarely associated with emergencies or adverse events in home care as well as in a specialized nursing care facility setting.

Coronary Artery Anomalies and Their Impact on the Feasibility of Percutaneous Pulmonary Valve Implantation.

One of the major obstacles preventing successful percutaneous pulmonary valve implantation (PPVI) is related to the close proximity of coronary artery branches to the expected landing zone. The aim of this study was to assess the frequency of coronary artery anomalies (CAAs) especially those associated with major coronary branches crossing the right ventricular outflow tract (RVOT) and to describe their relevance for the feasibility of percutaneous pulmonary valve implantation (PPVI). In our retrospective single-center study 90 patients were evaluated who underwent invasive testing for PPVI in our institution from 1/2010 to 1/2020. CAAs were identified in seven patients (8%) associated with major branches crossing the RVOT due to origin of the left anterior descending (LAD) or a single coronary artery from the right aortic sinus. In 5/7 patients with CAAs balloon testing of the RVOT and selective coronary angiographies revealed a sufficiently large landing zone distal to the coronary artery branch. While unfavorable RVOT dimensions prevented PPVI in one, PPVI was performed successfully in the remaining four patients. The relatively short landing zone required application of the "folded" melody technique in two patients. All patients are doing well (mean follow-up 3 years). CAAs associated with major coronary branches crossing the RVOT can be expected in about 8% of patients who are potential candidates for PPVI. Since the LAD crossed the RVOT below the plane of the pulmonary valve successful distal implantation of the valve was possible in 4/7 patients. Therefore these coronary anomalies should not be considered as primary contraindications for PPVI.

Catheter insertion depths in less-invasive surfactant administration.

INTRODUCTION: Less-invasive surfactant administration (LISA) under continuous positive airway pressure is increasingly used for the treatment of neonatal respiratory distress. Different procedures are described, but data on the optimal catheter insertion depth are sparse. OBJECTIVE: To generate data for recommending an optimal catheter insertion depth in LISA. METHODS: We examined 112 anterior-posterior chest X-rays from intubated infants and determined the carina's vertebral projection, whenever possible. After that, distances between the middle of cervical vertebra 4 (C4) and thoracic vertebra 2 and the middle of C4 to thoracic vertebra 3, respectively, were measured. Results were plotted against infant's weight. RESULTS: A weight-based chart and recommendations for the optimal intratracheal catheter position in infants with a body weight between 350 and 4000 g were created. CONCLUSIONS: Generated data offer standardisation and may thus help to find a balance between risk of surfactant reflux and unilateral surfactant administration.

Nasal insertion depths for neonatal intubation.

AIM: Data on the depth of nasal intubation in neonates are rare, although this is the preferred route in some countries. Therefore, recommendations on optimal nasal intubation depths based on gestational age (GA) and weight are desirable. METHODS: We determined the distances between the middle of thoracic vertebrae 2 (T2) and the tip of the endotracheal tube in 116 X-rays from nasally intubated neonates. The intubation depth (tip to nostril distance) that was documented in the digital patient's file was then corrected for this distance to reach an optimal nasal insertion depth. Results were plotted against the infant's GA and weight. RESULTS: GA-based and birthweight-based charts and formulas for the nasal intubation depth in infants with a GA between 24 and 43 weeks and body weight between 400 and 4500 g were created. CONCLUSIONS: Generated data may help in predicting optimal insertion depths for nasal intubation in neonates.

Ventriculoatrial malalignment in atrioventricular septal defect resulting in uniatrial biventricular connection: surgical options.

BACKGROUND: Uniatrial biventricular connection (UBC) is a rare cardiovascular anomaly characterized by absence of one atrioventricular connection and drainage of the other atrium via a solitary atrioventricular valve into both ventricles. The absent atrioventricular connection may affect either the left or right atrium. Because of the absence of one atrioventricular connection hearts with UBC have been classified among functionally univentricular hearts requiring palliative treatment according to the Fontan principle. AIMS/OBJECTIVE: We report two further patients with UBC. In one of these patients careful echocardiographic examination of the atrioventricular junction in early infancy revealed the possibility of biventricular repair based on the favorable anatomy of the atrioventricular valve and balanced ventricles in the presence of an inlet ventricular septal defect (VSD). CASE PRESENTATIONS: Both patients presented in the neonatal period for evaluation of complex congenital heart disease. The anatomy of the atrioventricular valves in our patients was indistinguishable from atrioventricular septal defects exhibiting the morphology of a common valve with superior and inferior bridging leaflets. The common atrioventricular valve was connected exclusively to the right atrium draining into both ventricles while the left atrium drained into the right atrium via a secundum atrial septal defect. In one of our patients biventricular repair with good longterm-result was performed by reseptation of the atria, patch repair of the VSD and septation of the atrioventricular valve. The second patient underwent univentricular palliation according to the Fontan principle. DISCUSSION/CONCLUSION: The echocardiographic findings in our patients suggest that at least some patients with UBC represent a variant of atrioventricular septal defects associated with extreme ventriculoatrial malalignment resulting in fusion of the deviated primary atrial septum with the lateral aspect of the atrioventricular junction. This offers the option of septation of the common atrioventricular valve and biventricular repair in patients with adequate size of both ventricles. Exact echocardiographic analysis of the morphology of the atrioventricular valve is essential to distinguish these patients with a morphologically common atrioventricular valve in early infancy from other variants of absent atrioventricular connection and to select those who are suitable for biventricular repair.

A nurse-driven analgesia and sedation protocol reduces length of PICU stay and cumulative dose of benzodiazepines after corrective surgery for tetralogy of Fallot.

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.