Long-term multidisciplinary follow-up programs in pediatric cardiac arrest survivors.

Long-term outcome studies after pediatric cardiac arrest (CA) are few. They require a CA registry and dedicated outcome teams. Learning about the long-term outcomes is very important for developing prognostication guidelines, improving post-cardiac care, counseling caregivers about the future of their child, and creating opportunities for therapeutic intervention studies to improve outcomes. Few PICUs worldwide provide a multidisciplinary follow-up program as routine practice at an outpatient clinic with standardized measurements, using validated instruments including neuropsychological assessments by psychologists. The primary goal of such a follow-up program should be to provide excellent care to children and their caregivers, thereby resulting in a high attendance. Pediatric psychologists, neurologists and pediatricians/pediatric intensivists should ideally be involved to screen for delayed development and psychosocial problems and offer appropriate care at the same time. Preferably, outcomes should consist of evaluation of morbidity (physical and neuropsychological), functional health and Health Related Quality Of Life (QoL) of the patient and their caregivers.

Trends in community response and long-term outcomes from pediatric cardiac arrest: A retrospective observational study.

AIM: This study aimed to investigate trends over time in pre-hospital factors for pediatric out-of-hospital cardiac arrest (pOHCA) and long-term neurological and neuropsychological outcomes. These have not been described before in large populations. METHODS: Non-traumatic arrest patients, 1 day-17 years old, presented to the Sophia Children's Hospital from January 2002 to December 2020, were eligible for inclusion. Favorable neurological outcome was defined as Pediatric Cerebral Performance Categories (PCPC) 1-2 or no difference with pre-arrest baseline. The trend over time was tested with multivariable logistic and linear regression models with year of event as independent variable. FINDINGS: Over a nineteen-year study period, the annual rate of long-term favorable neurological outcome, assessed at a median 2.5 years follow-up, increased significantly (OR 1.10, 95%-CI 1.03-1.19), adjusted for confounders. Concurrently, annual automated external defibrillator (AED) use and, among adolescents, initial shockable rhythm increased significantly (OR 1.21, 95% CI 1.10-1.33 and OR 1.15, 95% CI 1.02-1.29, respectively), adjusted for confounders. For generalizability purposes, only the total intelligence quotient (IQ) was considered for trend analysis of all tested domains. Total IQ scores and bystander basic life support (BLS) rate did not change significantly over time. INTERPRETATION: Long-term favorable neurological outcome, assessed at a median 2.5 years follow-up, improved significantly over the study period. Total IQ scores did not significantly change over time. Furthermore, AED use (OR 1.21, 95%CI 1.10-1.33) and shockable rhythms among adolescents (OR1.15, 95%CI 1.02-1.29) increased over time.

Association between shockable rhythms and long-term outcome after pediatric out-of-hospital cardiac arrest in Rotterdam, the Netherlands: An 18-year observational study.

INTRODUCTION: Shockable rhythm following pediatric out-of-hospital cardiac arrest (pOHCA) is consistently associated with hospital and short-term survival. Little is known about the relationship between shockable rhythm and long-term outcomes (>1 year) after pOHCA. The aim was to investigate the association between first documented rhythm and long-term outcomes in a pOHCA cohort over 18 years. METHODS: All children aged 1 day-18 years who experienced non-traumatic pOHCA between 2002-2019 and were subsequently admitted to the emergency department (ED) or pediatric intensive care unit (PICU) of Erasmus MC-Sophia Children's Hospital were included. Data was abstracted retrospectively from patient files, (ground) ambulance and Helicopter Emergency Medical Service (HEMS) records, and follow-up clinics. Long-term outcome was determined using a Pediatric Cerebral Performance Category (PCPC) score at the longest available follow-up interval through august 2020. The primary outcome measure was survival with favorable neurologic outcome, defined as PCPC 1-2 or no difference between pre- and post-arrest PCPC. The association between first documented rhythm and the primary outcome was calculated in a multivariable regression model. RESULTS: 369 children were admitted, nine children were lost to follow-up. Median age at arrest was age 3.4 (IQR 0.8-9.9) years, 63% were male and 14% had a shockable rhythm (66% non-shockable, 20% unknown or return of spontaneous circulation (ROSC) before emergency medical service (EMS) arrival). In adolescents (aged 12-18 years), 39% had shockable rhythm. 142 (39%) of children survived to hospital discharge. On median follow-up interval of 25 months (IQR 5.1-49.6), 115/142 (81%) of hospital survivors had favorable neurologic outcome. In multivariable analysis, shockable rhythm was associated with survival with favorable long-term neurologic outcome (OR 8.9 [95%CI 3.1-25.9]). CONCLUSION: In children with pOHCA admitted to ED or PICU shockable rhythm had significantly higher odds of survival with long-term favorable neurologic outcome compared to non-shockable rhythm. Survival to hospital discharge after pOHCA was 39% over the 18-year study period. Of survivors to discharge, 81% had favorable long-term (median 25 months, IQR 5.1-49.6) neurologic outcome. Efforts for improving outcome of pOHCA should focus on early recognition and treatment of shockable pOHCA at scene.

Long-term quality of life in children after open airway surgery for laryngotracheal stenosis.

OBJECTIVES: The purpose of this study is to evaluate the long-term health related quality of life (HRQoL) in a cohort of children surgically treated for laryngotracheal stenosis (LTS). STUDY DESIGN: Prospective cohort study. METHODS: Parents of children between 4 and 18 years at follow-up completed the Child Health Questionnaire Parent Form (CHQ-PF50). Children between 11 and 18 years at follow-up completed the Child Health Questionnaire Child Form (CHQ-CF87). Biographical and pre-operative data were extracted from the hospital records. Post-operative measurements consisted of the Bruce treadmill test and pulmonary function testing (PFT). RESULTS: Fifty-four parents completed the CHQ-PF50; twenty-one children completed the CHQ-CF87. The CHQ-PF50 was significantly worse than the norm population on the subscales physical functioning, role functioning: emotional/behavior, general health perceptions, family activities, parental impact: emotional, and time. CHQ-CF87 was significantly worse than the norm population on physical functioning and better on mental health. After multivariate analysis, presence of co-morbidities and glottic stenosis are the most important pre-operative factors for worse scores on general health. As post-operative measurements, the Bruce treadmill test and peak expiratory flow (PEF) correlate well with HRQoL physical subscales. CONCLUSIONS: At long-term follow-up after treatment for LTS, deficits in HRQoL may still exist. Presence of co-morbidities and glottic stenosis are important negative factors for long-term HRQoL. The Bruce treadmill test and peak expiratory flow on pulmonary function testing correlate well with physical subscales on HRQoL. A long-term multidisciplinary follow-up with assessment of HRQoL is advised in patients treated for LTS. LEVEL OF EVIDENCE: 2B, individual prospective cohort study.

Recovery in parents of children and adolescents who survived septic shock caused by Neisseria meningitidis: a cross-sectional study.

OBJECTIVES: To assess psychological distress, styles of coping and disease-related psychosocial limitations in parents of children and adolescents who survived meningococcal septic shock (MSS) 4-16 years ago. RESEARCH METHODOLOGY: An exploratory design using standardised questionnaires and interviews. SETTING: The psychological investigation took place in the department of Child and Adolescent Psychiatry of the ErasmusMC-Sophia Children's Hospital. MAIN OUTCOME MEASURES: 87 mothers and 77 fathers participated in this study. The General Health Questionnaire was used to assess parents' psychological distress; the Utrecht Coping List to assess styles of coping. A semi-structured disease-specific interview served to explore long-term disease-related psychosocial limitations for parents. RESULTS: MSS parents reported similar psychiatric symptoms and styles of coping in comparison to reference groups. Severity of illness and the child's age at time of illness were not significant predictors of parental psychological distress and styles of coping. The presence of somatic sequelae, cognitive or behavioural and emotional problems was not associated with the levels of parental psychiatric symptoms or styles of coping. The vast majority of parents reported no current disease-related psychosocial limitations due to the MSS. CONCLUSION: Parents of children who survived MSS show recovery. Nevertheless a minority still experiences emotional burden and disease-related limitations.

Long-term skin scarring and orthopaedic sequelae in survivors of meningococcal septic shock.

OBJECTIVE: To assess the incidence of skin scarring and orthopaedic sequelae (amputation, limb-length discrepancy) in patients who survived meningococcal septic shock (MSS) in childhood and to determine the severity and predictors of these sequelae. METHODS: 179 consecutive patients (170 of whom were eligible) with septic shock and purpura requiring intensive care between 1988 and 2001 in Rotterdam, the Netherlands were invited to visit a follow-up clinic 4-16 years after paediatric intensive care unit (PICU) discharge. RESULTS: 58 (48%) of 120 follow-up patients (median follow-up interval 10 years; median age at follow-up 14.5 years) had skin scarring due to purpura. This varied from barely visible to extremely disfiguring scars. Ten patients (8%) had undergone amputation(s) of extremities, ranging from one toe to both legs and one arm. Seven patients (6%) had lower limb-length discrepancy, in most cases together with angular deformity, requiring one or more late surgical intervention(s). Patients with scars or orthopaedic sequelae had significantly higher severity of illness scores, determined by the Pediatric Risk of Mortality score, Vasopressor score and Disseminated Intravascular Coagulation score. Gender or Neisseria meningitidis serogroup had no significant influence on the presence of scars or orthopaedic sequelae. Patients with lower limb-length discrepancy were significantly younger at the time of PICU admission. CONCLUSIONS: The incidence of long-term skin scarring and orthopaedic sequelae was high (48% and 14%, respectively) in patients who survived MSS in childhood. The severity of these sequelae varied from mild to severe. Patients with scars or orthopaedic sequelae had significantly higher severity of illness scores.

Long-term cognitive functioning in children and adolescents who survived septic shock caused by Neisseria meningitidis.

AIMS: To assess long-term cognitive functioning and its predictors, in children and adolescents who survived meningococcal septic shock (MSS) 4 to 16 years ago. METHODS: The Wechsler Intelligence Scale for Children-third edition was used to measure intellectual functioning and neuropsychological tests were used to measure attention, verbal memory, visual-motor integration, and executive skills. RESULTS: Overall, results of the total MSS sample (N=77) as to neuropsychological functioning were similar to those of normative reference groups. On social and practical reasoning and visual-motor integration, however, MSS children obtained poorer outcomes compared to normative data. Two children had mental retardation (estimated IQ<70) due to the MSS. The percentage of children with mental retardation or borderline intellectual functioning (15%) was similar to that in the general population (16%). Eighteen children (23%) had a z score<-2, indicating unusual poor functioning, on one or more domains of neuropsychological functioning (selective attention, sustained attention, and executive functioning). Compared to normative data, significantly more children had received special education services in the past. Older age at time of follow-up was the most important significant predictor of poorer long-term cognitive functioning. CONCLUSION: Overall, long-term outcomes as to cognitive functioning of the total MSS sample were similar to those of normative reference groups, but MSS children showed long-term impairments on social and practical reasoning, visual-motor integration, attention, and executive functioning. Older age at time of follow-up was a significant predictor.

Behavioural, emotional, and post-traumatic stress problems in children and adolescents, long term after septic shock caused by Neisseria meningitidis.

OBJECTIVES: To assess the occurrence of a wide range of behavioural, emotional, and post-traumatic stress problems in children and adolescents, long term after septic shock caused by Neisseria meningitidis (MSS). DESIGN: This study included 6- to 17-year-old patients who survived MSS and were admitted to the PICU of the Medical Centre between 1988 and 2001. To assess behavioural, emotional, and post-traumatic stress problems, the Child Behaviour Checklist (CBCL), the Teacher's Report Form (TRF), and the Youth Self-Report (YSR) were used. METHODS: Parents of 89 MSS children, aged 6-17 years, completed the CBCL. Teachers of 65 same-aged MSS children completed the TRF, and 45 11- to 17-year-old MSS children completed the YSR. These data were compared with those from the normative reference groups. RESULTS: Overall, the proportions of MSS children scoring in the deviant range for problem behaviour were comparable to the proportions in the reference groups, according to parents', teachers', and self-reports. As to the level of emotional and behavioural problems, mothers of the MSS children reported more somatic complaints regarding their children in comparison with the reference groups. Severity of illness was not a significant predictor of behavioural, emotional, and post-traumatic stress problems. Age at the time of illness was a significant predictor of behavioural, emotional, and post-traumatic stress problems in MSS children, indicating that the younger the child at the time of illness, the more problems were reported by parents at follow-up. CONCLUSION: Overall, the results showed long-term behavioural, emotional, and post-traumatic stress outcomes for MSS children, which were comparable to those in the general population.

[Respiratory insufficiency caused by acute disseminated encephalomyelitis in a child]. / Respiratoire insufficiëntie bij een kind door acute disseminerende encefalomyelitis.

A 1.5-year-old boy presented with progressive ataxia and meningeal irritation after a period of general malaise and fever. He was eventually admitted to a paediatric intensive care unit for respiratory insufficiency. A diagnosis of acute disseminated encephalomyelitis (ADEM) with pontine involvement was made. The patient was mechanically ventilated and treated with immunoglobulins and corticosteroids, after which he recovered almost completely. ADEM is characterised by rapidly progressive demyelination of the central nervous system. The exact incidence and aetiology are unknown. The disorder is considered to be an autoimmune reaction, and current treatment is aimed at the suppression of this reaction. Despite the dramatic clinical and radiological presentation of ADEM, the prognosis is favourable in most cases.

[Diagnostic image (137) An infant with fever and pancytopenia. Hemophagocytic lymphohistiocytosis]. / Diagnose in beeld (137). Een zuigeling met koorts en pancytopenie. Hemofagocytaire lymfohistiocytose.

A 2-month-old male infant with consanguineous parents had fever of unknown origin, pancytopenia, and hepatosplenomegaly. Laboratory tests, peripheral blood smear and bone marrow revealed haemophagocytic lymphohistiocytosis.