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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.

__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.

__Purpose:__ The aim of the study was to compare long-term respiratory morbidity in children after repair of esophageal atresia (EA) or congenital diaphragmatic hernia (CDH). __Patients and Methods:__ Children were seen at 6, 12, and 24 months and 5 years within a prospective longitudinal follow-up program in a tertiary children's hospital. Respiratory morbidity and physical condition were evaluated at all moments. At age 5 years, pulmonary function and maximal exercise performance were tested. __Results:__ In 3 of 23 atresia patients and 10 of 20 hernia patients, bronchopulmonary dysplasia was developed. Seventeen atresia and 11 hernia patients had recurrent respiratory tract infections mainly in the first years of life. At age 5, 25% of EA and CDH patients measured showed reduced forced expiratory volume in 1 second (z-score < -2). Both atresia and hernia patients showed impaired growth, with catch-up growth at 5 years in patients with EA but not in those with hernia. Maximal exercise performance was significantly below normal for both groups.

Introduction: Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) is a cardio-pulmonary bypass technique to provide life support in acute reversible cardio-respiratory failure when conventional management is not successful. Most neonates receiving ECMO suffer from meconium aspiration syndrome (MAS), congenital diaphragmatic hernia (CDH), sepsis or persistent pulmonary hypertension (PPH). In five-year-old children who underwent VA-ECMO therapy as neonates, we assessed motor performance related to growth, intelligence and behaviour, and the association with the primary diagnosis. Methods: In a prospective population-based study (n = 224) 174 five-year-old survivors born between 1993 and 2000 and treated in the two designated ECMO centres in the Netherlands (Radboud University Medical Centre Nijmegen and Sophia Children's Hospital, Erasmus MC - University Medical Center Rotterdam) were invited to undergo follow-up assessment including a paediatric assessment, the movement assessment battery for children (MABC), the revised Amsterdam intelligence test (RAKIT) and the child behaviour checklist (CBCL). Results: Twenty-two percent of the children died before the age of five, 86% (n = 149) of the survivors were assessed. Normal development in all domains was found in 49% of children. Severe disabilities were present in 13%, and another 9% had impaired motor development combined with cognitive and/or behavioural problems. Chi-squared tests showed adverse outcome in MABC scores (P < 0.001) compared with the reference population in children with CDH, sepsis and PPH, but not in children with MAS. Compared with the Dutch population height, body mass index (BMI) and weight for height were lower in the CDH group (P < 0.001). RAKIT and CBCL scores did not differ from the reference population. Total MABC scores, socio-economic status, growth and CBCL scores were not related to each other, but negative motor outcome was related to lower intelligen

Background: The objective of this study was to validate the Impact of a Child with Congenital Anomalies on Parents (ICCAP) questionnaire. ICCAP was newly designed to assess the impact of giving birth to a child with severe anatomical congenital anomalies (CA) on parental quality of life as a result of early stress. Methods: At 6 weeks and 6 months after birth, mothers and fathers of 100 children with severe CA were asked to complete the ICCAP questionnaire and the SF36. The ICCAP questionnaire measures six domains: contact with caregivers, social network, partner relationship, state of mind, child acceptance, and fears and anxiety. Reliability (i.e. internal consistency and test-retest) and validity were tested and the ICCAP was compared to the SF-36. Results: Confirmatory factor analysis resulted in 6 six a priori constructed subscales covering different psychological and social domains of parental quality of life as a result of early stress. Reliability estimates (congeneric approach) ranged from .49 to .92. Positive correlations with SF-36 scales ranging from .34 to .77 confirmed congruent validity. Correlations between ICCAP subscales and children's biographic characteristics, primary CA, and medical care as well as parental biographic and demographic variables ranged from -.23 to .58 and thus indicated known-group validity of the instrument. Over time both mothers and

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.

Introduction: Extracorporeal membrane oxygenation (ECMO) is a supportive cardiopulmonary bypass technique for babies with acute reversible cardiorespiratory failure. We assessed morbidity in ECMO survivors at the age of five years, when they start primary school and major decisions for their school careers must be made. Methods: Five-year-old neonatal venoarterial-ECMO survivors from the two designated ECMO centres in The Netherlands (Erasmus MC - Sophia Children's Hospital in Rotterdam, and University Medical Center Nijmegen) were assessed within the framework of an extensive follow-up programme. The protocol included medical assessment, neuromotor assessment, and psychological assessment by means of parent and teacher questionnaires. Results: Seventeen of the 98 children included in the analysis (17%) were found to have neurological deficits. Six of those 17 (6% of the total) showed major disability. Two of those six children had a chromosomal abnormality. Three were mentally retarded and profoundly impaired. The sixth child had a right-sided hemiplegia. These six children did not undergo neuromotor assessment. Twenty-four of the remaining 92 children (26%) showed motor difficulties: 15% actually had a motor problem and 11% were at risk for this. Cognitive delay was identified in 11 children (14%). The mean IQ score was within the normal range (IQ = 100.5). Conclusion: Neonatal ECMO in The Netherlands was found to be associated with considerable morbidity at five years of age. It appeared feasible to have as many as 87% of survivors participate in follow-up assessment, due to cooperation between two centres and small travelling distances. Objective evaluation of the long-term morbidity associated with the application of this highly invasive technology in the immediate neonatal period requires an interdisciplinary follow-up programme with nationwide consensus on timing and actual testing protocol.