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Background and Aims

Recent years the number of children dependent on home mechanical ventilation (HMV) has substantially increased. Candidates for HMV are patients suffering from chronic respiratory failure due to either neurologic/muscular disease, or airway/lung pathologies.

Our aim was to study the special characteristics of pediatric patients receiving HMV upon discharge from Pediatric Intensive Care Unit (PICU).

Methods

A single-center retrospective study of all pediatric patients (age<18 years) admitted to PICU and discharged on HMV, from January 2018 to January 2021. Demographic data,underlying disorder,mode of ventilation,interaction with environment/others and comorbidities were recorded.

Results

Nine patients were included.Three of them had severe neurological disease and no interaction with the environment, thus it is questionable whether they were suitable candidates for HMV.

Conclusions

Although the decision for HMV in pulmonary diseases is defined by guidelines,there is no medical,legal or ethical consensus regarding futility in cases with severe neurological impairment.In these irreversible cases,it is debatable if HMV is in the child’s best interest.

Background and Aims

The threshold for resuscitation vary between 22 and 25 weeks gestational age (GA) in high-income countries. Norwegian consensus statement defines a “grey zone” at GA 23 to 24 weeks, which in practice has narrowed down to GA 23 weeks. In 2013-14, 80 % of live-born infants born at GA 23 weeks in Norway were transferred to a NICU, despite concerns about long-term impairment which is strongly associated with decreasing GA. Our aim was to explore how Norwegian residents in paediatrics perceive the beneficence of life support at different GAs, and compare the findings with existing guidelines and practice.

Methods

A questionnaire was distributed to paediatric residents during the national training course in neonatology in 2017, 2019 and 2020, asking whether they would want life support to be started for their own hypothetical infant born at GA 22, 23, 24, 25 and 26 weeks.

Results

80 residents (> 80 % of participants) responded the questionnaire. At GA 23 weeks, 58/80 (73 %) did not want life support, 22/80 (27 %) were uncertain and none answered affirmatively. Even at GA 25 weeks, only 47/80 (59 %) answered in favour of life support and 27/80 (34 %) were still uncertain.

Conclusions

We found a significant gap between common resuscitation practice for extreme premature infants, and what residents in paediatrics express that they would want for their own hypothetical baby. This points towards an important ethical tension. Are young doctors simply morally flawed? We will discuss potential reasons and ethical implications of our findings.

Background and Aims

Over recent years the paediatric profession has come to the forefront in the press, with a number of high-profile cases highlighting examples of discordance between clinical teams and patients’ families. As clinicians we endeavour to work with parents toward a shared goal. But what happens when there is disagreement? How do we tread the rocky ethical road that cases such as these set out for us? Our aim is to describe a collaborative way of working with all those involved which requires a shared understanding of multi professional and parental perspectives.

Methods

We present a case where the parents and clinicians disagreed over what was the most clinically appropriate and compassionate management for the child; an infant with a diagnosis of PURA syndrome, a rare syndrome associated with central hypoventilation, epilepsy and learning difficulties. Our challenge was to develop an ethical and clinically sound care plan for the now which took into account parental and professional concerns about her future quality of life (which remained relatively unknown). We consulted our hospital ethics committee to work towards a unified plan in the best interests of both the child and family.

Results

We present a framework for assessing moral balance in this difficult ethical situation. We also include the parents’ perspective on this case.

Conclusions

We hope this will provide a holistic framework for those facing such situations in the future.

Background and Aims

To assess brain injury MRI patterns and neonatal’ profiles in a large cohort of newborns > 36 weeks’ gestation in the context of neonatal encephalopathy (NE) treated with therapeutic hypothermia (TH).

Methods

French prospective observational population-based cohort including newborns with moderate and severe NE treated with TH. Images from brain MRI performed between birth and 12 days of life in surviving and deceased patients were analyzed in 7 brain regions according to Rutherford’s classification.

Results

575 newborns were eligible, 479 had an available MRI; 80 (16.7%) infants had died. MRI findings were normal for 231 (48.2%) infants; 155 (62.5%) infants had injuries in more than one region. Basal ganglia thalamus (BGT) and white matter injuries were predominant, reported for 33.7% and 32.4% infants, respectively. Infra-tentorial injuries were observed for 8.5% (brainstem) and 5.1% (cerebellum) of infants, respectively. Posterior-limb internal capsule and brainstem injuries were systematically associated with BGT injuries. Infants with severe injuries (BGT) more frequently had a warning event before birth, the poorest neonatal adaptation and severe acidemia, but almost 1 in 2 infants had the same associated factors as those with normal MRI findings.

Conclusions

This study updates the panorama of brain lesions in NE in the era of TH, with a large proportion of normal MRI findings in half of the infants and peculiar brain injuries associations. NE is mainly a diffuse brain pathology. MRI may assist to diagnose the hypoxic-ischemic nature of the encephalopathy. Prognostic markers for identifying brain-injured patients require further analysis.

Background and Aims

Large-sample data are lacking with regard to the clinical features and phenotypes, diagnosis and treatment strategies, and prognosis of CASPR2 autoimmunity in pediatric patients. The objective of this study was to identify the frequency of CASPR2 autoantibody disease and to better understand the clinical features and phenotypes associated with CASPR2 antibodies.

Methods

This study performed a multicenter retrospective analysis of the neuroimmune antibodies of children with autoimmune neurological disorders in the past 6 years.

Results

The seropositive rates of NMDAR-IgG and MOG-IgG were 8.09% (95/1174) and 14.53% (60/413), respectively. Only 12 patients (0.76%, 12/1579) were seropositive CASPR2-IgG. The minimum age was 5 months, and none of the patients had neoplasms. The most common clinical symptoms of CASPR2 autoimmunity included fever, seizures, disorders of consciousness, psychiatric symptoms, sleep disorders, peripheral nerve hyperexcitability/neuromyotonia, autonomic symptoms and movement disorders. Autoimmune encephalitis, intractable epilepsy and Morvan syndrome are the most common clinical phenotypes. Electroencephalography (EEG) and cerebrospinal fluid (CSF) examinations have poor saspecificity. The most commonly involved part identified by MRI is the cerebral cortex. Immunotherapy and symptomatic treatment are effective for CASPR2 autoimmunity. In this study, one patient with a poor response to conventional immunotherapy was cured after rituximab treatment.

Conclusions

CASPR2 autoimmune disease is very rare in children and is more common in males than in females. This disease can occur in infancy and autoimmune encephalitis, intractable epilepsy and Morvan syndrome are the most common clinical phenotypes. Immunotherapy and symptomatic treatment are effective for CASPR2 autoimmunity, and the overall prognosis is good.

Background and Aims

A relationship has recently been discovered between cleaved-tau (C-tau) and neuroglobin (Ngb) biomarkers and neural damage. We aimed to investigate; 1. their potential utility as a biomarker of neural damage caused by meningitis and/or increased intracranial pressure (IICP), and 2. simultaneous levels in CSF and blood.

Methods

This was a prospective observational study, funded by the scientific research project unit commission of Marmara University (SAG-TUP-131216-0524). Patients (1mo-18yrs) presenting (between 2015-2017) to the tertiary university hospital, with suspected meningitis and/or IICP, were included. Biomarkers were measured by the enzyme-linked immunosorbent assay method and analyzed in 4 groups according to final diagnoses.

Results

68 patients (median age:15 months) were included. There was no significant correlation between biomarker levels and symptom duration, CSF protein level, presence of high fever, and presence of seizures. There was a strong positive correlation between serum C-tau and Ngb levels in all groups (p <0.001). Median serum biomarker levels were higher than median CSF biomarker levels in all groups.

Conclusions

To our knowledge, this is the first study to evaluate C-tau and Ngb simultaneously in human CSF and serum. Contrary to the previous literature, the prediction of C-tau and Ngb in defining neural damage in meningitis and/or IICP was found to be insufficient.

Background and Aims

Cerebral autoregulation (CA) is a physiological mechanism aimed to preserve cerebral blood flow and its impairment has been associated with unfavourable neurological outcome after severe TBI in adult and paediatric population. Despite a growing interest in neonatal CA monitoring, limited ICP-based data are reported.

Methods

We analysed three consecutive cases of ICP-monitored neonates admitted to our PICU. Patient #1 and #2 were full-term neonates presenting cranial fractures and epidural hematomas after accidental fall in their first week of life. Patient #3 was a neonate who underwent an emergency unilateral decompressive craniectomy (DC) after a devasting car accident.

Pressure Reactivity index (PRx) was continuously monitored processing ABP and ICP signals with ICM+ Software (Cambridge University, UK) on an observational basis.

Patients received standard care following current guidelines for paediatric severe TBI management.

Results

PRx was monitored for a total of 410 hours. In case #1 values suggest transient CA impairment on day 2 (see Figure) associated with acceptable ICP and CPP (good recovery at hospital discharge). In #2 no sustained PRx>0.3 was detected, including when ICP was in the upper limits (good recovery at hospital discharge). In #3 displayed ICP and PRx values were globally low but their accuracy and interpretation after DC were questionable (patient died on day 48). Optimal CPP determination could be possible during a relevant part of the monitoring period.

Conclusions

CA assessment is feasible in ICP-monitored neonates and PRx may provide useful information for ICP/CPP threshold tailoring in the context of multi-modal neuromonitoring.

Background and Aims

Cerebral vascular stroke (CVS) in children is a challenging medical emergency. A paediatric CVS protocol was started four years ago in our region by a multidisciplinary Paediatric Stroke Working Group.

The primary aim was to describe the clinical characteristics, management and short term outcomes of children presenting with acute neurological symptoms where CVS protocol was activated and ischemic stroke was identified. The secondary aim was to assess whether the stroke mimics also benefited from an early management.

Methods

Observational retrospective single-centre study through patient databases from January 2016 to December 2020. A standardized data extraction checklist was used. Neonatal (<28 days), brain-trauma related and in-hospital strokes were excluded.

Results

The protocol was activated 10 times. One brain-trauma-related and one in-hospital stroke were excluded. Median time to image was 240 minutes (85-510). Acute ischemic stroke was confirmed in 3 patients, median age 8 years. All had hemiparesis, 1 language disorder and visual disturbance and 2 headache. The mean PedNIHSS score was 15. Two patients were eligible for recannalization therapy: one had anterior circulation stroke, another had posterior circulation stroke; times to treatment were 420 and 230 minutes respectively. They did not experience any short term complication and had mild neurological deficits at hospital discharge. The third patient had a wake-up stroke, with diffused intracranial vascular irregularity and eventually died. The stroke mimics consisted of 2 focal crisis, one migraine, one hypoglycaemia and one brain tumour.

Conclusions

CVS is an infrequent medical emergency. Our multidisciplinary protocol offers effective and quick response to ischemic stroke.

Background and Aims

Evidence about decompressive craniotomy (DC) to lower mortality in children with severe traumatic brain injury (TBI) is scarce. We aimed to investigate the effect of DC on mortality in children with severe TBI.

Methods

We included patients < 18 years from Germany, Austria, and Switzerland with abbreviated injury scores (AIS) for the head ≥ 3 from the German trauma registry.

Results

2507 patients were included. DC was performed in 402 (16%) patients. Patients undergoing DC had higher AIS head scores, predicted mortality (RISC II-Score), and ISS.

Mortality depended on the severity of head injury (AIS head 3, 3%, AIS head 6: 100%) and was higher in patients undergoing DC (21% vs. 14%). In children > 6 years undergoing DC, mortality was lower than predicted (mortality/RISC II for ages 6-9, 10-13, 14-17 years: 19%/22%, 5%/15%, 9%/23%). In patients with AIS head 3 and 4, mortality was higher in the DC group (10 and 11%) than in the medical treatment group (3% and 7%). In AIS head 5, mortality was lower in the DC group (32% vs. 45%). Analyses to rule out concomitant injuries affecting mortality for AIS head 3 and 4, did not change results. The adjusted odds ratio for mortality was 1.20 (0.74-1.95) and for mortality and vegetative state 1.56 (1.01-2.40).

Conclusions

We showed worse outcomes for AIS 3 and 4 and favorable outcomes for AIS 5 after DC. The adjusted odds ratios did not favour DC. Subgroups of patients that benefit from DC need to be identified in larger trials.

Background and Aims

There is an increasing number of chronic critically ill (CCI) patients in pediatric intensive care unit (PICU). Due to the complexity of care, efficient interprofessional collaboration is paramount but to date the evidence is scare.

The aim of this study is to describe the extend of interprofessional collaboration amongst PICU teams when working with CII patients and their families and to identify the predictors of good interprofessional collaboration.

Methods

This observational study took place in eight Swiss PICUs. All healthcare professionals working in PICU for at least three months and caring for CCI children were invited to participate in an online survey. It included sociodemographic and professional characteristics as well as the multidimensional Assessment of Interprofessional Team Collaboration Scale II. Items of the domains of partnership, cooperation, and coordination were rated on a Likert scale from 1 (the lowest) to 5 (the highest). Descriptive and regression analyses were performed.

Results

458 questionnaires were completed from 8 PICUs (45% Response rate). The majority of the participants were nurses (60%) and physicians (20%), female (88%), specialized in PICU (73%) and working fulltime (54%). The mean total collaboration score was 3.60 ±0.51, for partnership 3.75 ±0.57, for cooperation 3.74 ±0.50, and for coordination 3.28 ±0.65. Regression analyses shows the medical profession was the only predictor of better interprofessional collaboration (Coef. 0.28 (SE.08) p=0.001).

Conclusions

Overall there is room for interprofessional collaboration improvement, especially in the coordination domain. The whole team would benefit from clarification about what it really means working together. Interprofessional critical care education is warranted.

Background and Aims

To describe staff response to communication simulation with use of care bundle tools at a paediatric intensive care unit(PICU).

Methods

DIdactics with communication techniques such as "ask-tell-ask" and mneumonics were used, followed by simulation in a facilitated environment with role-play scenarios and group reflection.

Staff were invited to attend themed workshops on one of three domains of communication: delivering significant news, conflict resolution and leadership in difficult conversations. 10-item questionnaires on self-perceived confidence were collected before and after each session.

Results

Each 45-minute workshop was attended by 2-3 participants and 2 facilitators, delivered as part of palliative care study days, PICU bootcamps or masterclass. 57 PICU nurses (band 5-8) and 23 doctors (trainees and consultants) participated in 30 sessions between June2018 and September2020. Faculty were from disciplines of intensive care, palliative care and clinical psychology. The mean pre-workshop and post-workshop confidence levels on "preparing, delivering and supporting parents/carers with significant news" were 6/10 and 8/10 respectively.

Pre-workshop feedback indicated anxiety around handling emotions, limited experience with "breaking bad news" and worry about meeting expectations of parents. Partipants reported positively on small group size, role-playing, timing of sessions and discussion opportunity in a safe space. The workshop style and contents were marked as "excellent", and 100% participants indicated "definitely to attend another similar session in the future".

Conclusions

Communication training delivered as a care bundle using reflective learning and instructive tool can improve confidence levels for professionals. It is anticipated that proceduralization will foster improvement for staff in managing challenging communication on PICU.