Children with Anatomical Congenital Anomalies; a Portrait Follow-up over five years

Annually some 5.000 newborns (2 - 3% of all births) in the Netherlands present with major anatomical congenital anomalies.140,395 The causes of most of these are still unknown. So far, genetic or chromosomal causes have been implicated in 13 - 14% of all anomalies,17,120,214,249,337 and environmental factors, including maternal disease, can explain another 6 - 7%.149,345,472 Some 20% are known to be multifactorial. Many of these anomalies require immediate surgical correction in order for these children to survive. The pediatric surgeon Ravitch classified six of these anomalies as the so called index diagnoses.351 These are esophageal atresia/tracheo-esophageal fistula, congenital diaphragmatic hernia, intestinal atresias, Hirschsprung's disease, anorectal malformations, and abdominal wall defects (gastroschisis and omphalocele). They occur either in isolated form or as part of more complex syndromes.59 Multiple congenital anomalies refers to any combination of two or more major anomalies. As a governmental policy patients presenting with an index diagnosis are to be treated in one of six pediatric surgical centers in the Netherlands.170 Improved antenatal detection, surgical techniques, and peri-operative care have increased these children's chances of survival.210 Survivors may show considerable morbidity, however, and many will have to rely on the healthcare system for life

Assessment and significance of long-term outcomes in pediatric surgery

Treatment modalities for newborns with anatomical congenital anomalies have greatly improved over the past decades, with a concomitant increase in survival. This review will briefly discuss specific long-term outcomes to illustrate, which domains deserve to be considered in long-term follow-up of patients with anatomical congenital anomalies. Apart from having disease-specific morbidities these children are at risk for impaired neurodevelopmental problems and school failure, which may affect participation in society in later life. There is every reason to offer them long-term multidisciplinary follow-up programs. We further provide an overview of the methodology of long-term follow-up, its significance and discuss ways to improve care for newborns with anatomical congenital anomalies from childhood into adulthood. Future initiatives should focus on transition of care, risk stratification, and multicenter collaboration

Introduction

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

Diagnosis-related deterioration of lung function after extracorporeal membrane oxygenation.

The aim of the study was to assess lung function longitudinally after neonatal extracorporeal membrane oxygenation (ECMO), and to identify any effects of diagnosis and perinatal characteristics. 121 neonatal ECMO-treated children (70 with meconium aspiration syndrome, 20 congenital diaphragmatic hernia and 31 with other diagnoses) performed a total of 191 lung function measurements at 5, 8 and/or 12 yrs. We assessed dynamic and static lung volumes, reversibility of airway obstruction and diffusion capacity. Mean SDS forced expiratory volume in 1 s (FEV(1)) at 5 yrs before and after bronchodilation (-0.51 and 0.07) was significantly higher than at 8 (-0.79 and -0.4; p<0.04) and 12 yrs (-1.10 and -0.52; p<0.003). Mean SDS for all spirometric parameters before and after bronchodilation were significantly lower in the congenital diaphragmatic hernia group compared with the other diagnostic groups (all p</=0.025). A significant volume of trapped air was observed in 86% patients with congenital diaphragmatic hernia, 50% with meconium aspiration syndrome and 58% with other diagnoses. After bronchodilation, mean SDS FEV(1) and forced vital capacity were negatively influenced by duration of ventilation (both p<0.001) and duration of ECMO (p=0.003 and p=0.02, respectively). Long-term pulmonary sequelae after neonatal ECMO-treatment mainly occur in congenital diaphragmatic hernia patients and tend to deteriorate over time

Motor-function and exercise capacity in children with major anatomical congenital anomalies

__Background:__ Children with major anatomical congenital anomalies (CA) often need prolonged hospitalization with surgical interventions in the neonatal period and thereafter. Better intensive care treatment has reduced mortality rates, but at the cost of more morbidity. __Aim:__ To study motor-function and exercise capacity in five-year-old children born with CA, and to determine whether motor-function and exercise capacity differ according to primary diagnosis. __Study design:__ Descriptive study. __Subjects:__ One-hundred-and-two children with the following CA: congenital diaphragmatic hernia (CDH) n=24, esophageal atresia (EA) n=29, small intestinal anomalies (SIA) n=25, and abdominal wall defects (AWD) n=24. __Outcome measures:__ Overall and subtest percentile scores of the Movement-Assessment Battery for Children (M-ABC) were used to measure motor skills. Endurance time on the Bruce treadmill test was used to determine maximal exercise capacity. __Results:__ Motor-function: Seventy-three children (71.6%) had an overall percentile score within the normal range, 18 (17.6%) were classified as borderline, and 11 (10.8%) had a motor problem. This distribution was different from that in the reference population (Chi square: p=0.001). Most problems were encountered in children with CDH and EA (p=0.001 and 0.013, respectively). Ball skills and balance were most affected. __Exercise capacity:__ Mean standard deviation score (SDS) endurance time=-. 0.5 (SD: 1.3); p=0.001; due to poor exercise performance in CDH and EA patients. __Conclusions:__ Children with major anatomical CA and especially those with CDH and EA are at risk for delayed motor-function and disturbed exercise capacity

Sensorineural hearing loss and language development following neonatal extracorporeal membrane oxygenation

OBJECTIVE: To determine the prevalence of hearing loss in school-age children who have undergone neonatal extracorporeal membrane oxygenation (ECMO) 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-ECMO 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 to 12 years. Hearing loss was considered clinically significant when >20dB. Hearing was normal in 75.7% of children. Five children (3.7%) had bilateral sensorineural or combined hearing loss; 3 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 2 (8.3%) had mild SNHL, unilateral in one of them. Follow-up at 24 months of age had shown normal verbal and non-verbal developmental scores. Language development and intelligence median (range) scores at 5 years 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 was 104 (68-132) in 106 children. Scores did not differ between those with normal hearing, and those with mild hearing loss, or those with 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 ECMO survivors. The prevalence of bilateral sensorineural hearing loss was in accordance with that of larger series in the United States – which exceeds the prevalence in the normal population

Growth and development after oesophageal atresia surgery: Need for long-term multidisciplinary follow-up

Abstract Survival rates in oesophageal atresia patients have reached over 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We reviewed the literature on the long-term morbidity of these patients and discuss mainly issues of physical growth and neurodevelopment. We conclude that growth problems–both stunting and wasting–are frequently seen, but that sufficient longitudinal data are lacking. Therefore, it is unclear whether catch-up growth into adolescence and adulthood occurs. Data on determinants of growth retardation are also lacking in current literature. Studies on neurodevelopment beyond preschool age are scarce but oesophageal atresia patients seem at risk for academic problems and motor function delay. Many factors contribute to the susceptibility to growth and development problems and we propose a multidisciplinary follow-up schedule into adulthood future care which may help improve quality of life

Verbal and visual-spatial memory problems at adolescent age after neonatal extracorporeal membrane oxygenation

__Objectives__ To assess neuropsychological 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 neuropsychological 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 anti-epileptics. 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 neuropsychological skills in adolescence and adulthood; 2) identifying risk factors of neuropsychological dysfunction; 3) evaluating to what extent “severity of illness” is responsible for acquired brain injury; and 4) effects of timely cognitive rehabilitation

Neuropsychological Follow-up After Neonatal ECMO

OBJECTIVE: To assess the longitudinal development of intelligence and its relation to school abstract 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

A parent-reported standardised checklist is not sensitive to screen for motor problems at school age following neonatal critical illness

Aim: As nowadays more children survive neonatal critical illness, evaluation of long-term morbidities becomes more important. We determined whether the parent-reported Movement Assessment Battery for Children-Second Edition (MABC-2) Checklist is a proper tool to screen for motor problems in school-aged chi