Marlous J. Madderom

Congenital diaphragmatic hernia with(out) ECMO: impaired development at 8 years.

Objective To evaluate developmental and social-emotional outcomes at 8 years of age for children with congenital diaphragmatic hernia (CDH), treated with or without neonatal extracorporeal membrane oxygenation (ECMO) between January 1999 and December 2003. Design Cohort study with structural prospective follow-up. Setting Level III University Hospital. Patients 35 children (ECMO: n=16; non-ECMO: n=19) were assessed at 8 years of age. Interventions None. Main outcome measures Intelligence and motor function. Concentration, behaviour, school performance, competence and health status were also analysed. Results Mean (SD) intelligence for the ECMO group was 91.7 (19.5) versus 111.6 (20.9) for the non-ECMO group (p=0.015). Motor problems were apparent in 16% of all participants and differed significantly from the norm (p=0.015) without differences between treatment groups. For all participants, problems with concentration (68%, p<0.001) and with behavioural attention (33%, p=0.021) occurred more frequently than in reference groups, with no difference between treatment groups. School performance and competence were not affected. Conclusions Children with CDH—whether or not treated with neonatal ECMO—are at risk for long-term morbidity especially in the areas of motor function and concentration. Despite their impairment, children with CDH have a well-developed feeling of self-competence.

Neuropsychological Follow-up After Neonatal ECMO

OBJECTIVE: To assess the longitudinal development of intelligence and its relation to school performance in a nationwide cohort of neonatal extracorporeal membrane oxygenation (ECMO) survivors and evaluate predictors of outcome at 8 years of age. METHODS: Repeated measurements assessed intelligence of neonatal ECMO survivors at 2, 5, and 8 years (n = 178) with the use of validated, standardized instruments. Selective attention (n = 148) and type of education were evaluated in the 8-year-olds. RESULTS: Intelligence remained stable and average across development (mean ± SD IQ: at 2 years, 102 ± 18; at 5 years, 100 ± 17; and at 8 years, 99 ± 17 [P = .15]). Children attending regular education without the need for help (n = 101; mean z score: –1.50 ± 1.93) performed significantly better on the selective attention task compared with those children who needed extra help (n = 65; mean z score: –2.54 ± 3.18) or those attending special education (n = 13; mean z score: –4.14 ± 3.63) (P = .03). However, only children attending special education had below-average intelligence (mean IQ: 76 ± 15), compared with average intelligence for those attending regular education, both with help (mean IQ: 95 ± 15) and without help (mean IQ: 105 ± 16). Compared with children with other diagnoses, children with congenital diaphragmatic hernia (CDH) scored significantly lower on both IQ (CDH, mean IQ: 93 ± 20; meconium aspiration syndrome, mean IQ: 100 ± 15; other diagnoses, mean IQ: 100 ± 19 [P = .04]) and selective attention (CDH, mean z score: –3.48 ± 3.46; meconium aspiration syndrome, mean z score: –1.60 ± 2.13; other diagnoses, mean z score: –1.65 ± 2.39 [P = .002]). CONCLUSIONS: For the majority of neonatal ECMO survivors, intelligence testing alone did not identify those at risk for academic problems. We propose internationally standardized follow-up protocols that focus on long-term, problem-oriented neuropsychological assessment.

Neonatal extracorporeal membrane oxygenation: impaired health at 5 years of age.

Objective: Children treated with neonatal extracorporeal membrane oxygenation may show physical and mental morbidity at a later age. We compared the health-related quality of life of these children with normative data. Design: Prospective longitudinal follow-up study. Setting: Outpatient clinic of a level III university hospital. Patients: Ninety-five 5-yr-old children who had received neonatal extracorporeal membrane oxygenation support between January 1999 and December 2005. Interventions: None. Measurements and Main Results: The pediatric quality of life inventory was administered at 5 yrs of age. The mothers (n = 74) as proxy-reporters assigned significantly lower health-related quality of life scores for their children than did the parents in the healthy reference group for the total functioning scale of the pediatric quality of life inventory (mean difference: 8.1; p < 0.001). Mothers’ scores for 31 children (42%) were indicative of impaired health-related quality of life (≥−1 SD below the reference norm). The children (n = 78) themselves scored significantly lower than did their healthy peers on total functioning (mean difference: 11.0; p < 0.001). Thirty-two children (41%) indicated an impaired health-related quality of life themselves. For the mother proxy- reports, the duration of extracorporeal membrane oxygenation support (R2 = 0.009; p = 0.010) and the presence of chronic lung disease (R2 = 0.133; p = 0.002) were negatively related to total functioning. Children with a disabled health status for neuromotor functioning, maximum exercise capacity, behavior, and cognitive functioning at 5 yrs of age had a higher odds ratio of also having a lower health-related quality of life. Health status had no influence on reported emotional functioning. Conclusions: Overall, children treated with extracorporeal membrane oxygenation in the neonatal period reported low health-related quality of life at 5 yrs of age. Because only emotional health-related quality of life was not associated with health status, the pediatric quality of life inventory might be a measure of health status rather than of health-related quality of life. In contrast with conclusions from others, we found that 5-yr-old children might be too young to rate their own health-related quality of life.

Growing Up After Critical Illness: Verbal, Visual-Spatial, and Working Memory Problems in Neonatal Extracorporeal Membrane Oxygenation Survivors.

Objectives:To assess neuropsychologic outcome in 17- and 18-year–old neonatal extracorporeal membrane oxygenation survivors. Design:A prospective longitudinal follow-up study. Setting:Follow-up program at the Erasmus MC-Sophia Children’s Hospital in Rotterdam, The Netherlands. Patients:Thirty adolescents 17 or 18 years old, treated between 1991 and 1997, underwent neuropsychologic assessment. Interventions:None. Measurements and Main Results:Attention, memory, executive functioning, visual-spatial functions, social-emotional functioning, and behavior were assessed with validated instruments, and data were compared with reference data. Included predictors for analysis of adverse outcome were diagnosis, age at start extracorporeal membrane oxygenation, convulsions, and use of antiepileptics. Adolescents’ performance (expressed as mean [SD] z score) was significantly lower than the norm on short-term and long-term verbal memory (z score = −1.40 [1.58], p = 0.016; z score = −1.54 [1.67], p = 0.010, respectively), visual-spatial memory (z score = −1.65 [1.37], p = 0.008; z score = −1.70 [1.23], p = 0.008, respectively), and working memory (32% vs 9% in the norm population). Parents reported more problems for their children regarding organization of materials (z score = −0.60 [0.90]; p = 0.03) and behavior evaluation (z score = −0.53 [0.88]; p = 0.05) on a questionnaire. Patients reported more withdrawn/depressed behavior (z score = −0.47 [0.54]; p = 0.02), somatic complaints (z score = −0.43 [0.48]; p = 0.03), and social problems (z score = −0.41 [0.46]; p = 0.04). Patients reported more positive feelings of self-esteem and an average health status. Conclusions:Adolescents treated with neonatal extracorporeal membrane oxygenation are at risk of verbal, visual-spatial, and working memory problems. Future research should focus on 1) the longitudinal outcome of specific neuropsychologic skills in adolescence and adulthood; 2) identifying risk factors of neuropsychologic dysfunction; 3) evaluating to what extent “severity of illness” is responsible for acquired brain injury; and 4) effects of timely cognitive rehabilitation.

Sensorineural hearing loss and language development following neonatal extracorporeal membrane oxygenation

Objectives: To determine the prevalence of hearing loss in school-age children who have undergone neonatal extracorporeal membrane oxygenation treatment and to identify any effects of hearing loss on speech and language development. Design: Prospective longitudinal follow-up study within the framework of a structured post-extracorporeal membrane oxygenation follow-up program. Setting: Outpatient clinic of a level III university hospital. Results: Tone audiometry was performed by standardized protocol in 136 children aged 5–12 yrs. Hearing loss was considered clinically significant when >20 dB. Hearing was normal in 75.7% of children. Five children (3.7%) had bilateral sensorineural or combined hearing loss; three of them received special audiological care (2.2% of total sample). Of the 24 children with congenital diaphragmatic hernia, 19 (79.2%) had normal hearing and only two (8.3%) had mild sensorineural hearing loss, unilateral in one of them. Follow-up at 24 months of age had shown normal verbal and nonverbal developmental scores. Language development and intelligence median (range) scores at 5 yrs of age were also normal: receptive language development 104 (55–133), syntactical development 104 (68–132), and lexical development 101 (50–141) for 89 children; intelligence quotient 104 (68–132) for 106 children. Scores did not differ among those with normal hearing, mild hearing loss, and moderate-to-severe hearing loss (p = 0.800, p = 0.639, p = 0.876, and p = 0.886, for the respective developmental tests). Conclusions: We found normal language development and intelligence in a cohort of neonatal extracorporeal membrane oxygenation survivors. The prevalence of bilateral sensorineural hearing loss was in accordance with that of a larger series in the United States—which exceeds the prevalence in the normal population.

Abstract P-526: CEREBRAL WHITE MATTER MICROSTRUCTURE CHANGES AND MEMORY IMPROVEMENT FOLLOWING WORKING-MEMORY TRAINING IN SURVIVORS OF NEONATAL CRITICAL ILLNESS

During July 2013 and June 2016, 128 consecutive children (71 boys and 57 girls) in PICU underwent VEEG monitoring for 202 times. The median age was 1.4 years (from 26 days to 17.4 years). Electrographic seizures occurred in 42 patients (32.8%), of which 17 cases (40.5%) had electrographic status epilepticus. Severe abnormal EEG background was associated with electrographic seizures (P<0.001). Patients with electrographic status epilepticus had greater odds of in-hospital death.