Persisting Motor Function Problems in School-Aged Survivors of Congenital Diaphragmatic Hernia.

Background and Objectives: Children born with congenital diaphragmatic hernia (CDH) and treated with extracorporeal membrane oxygenation (ECMO), are at risk for motor function impairment during childhood. We hypothesized that all children born with CDH are at risk for persistent motor function impairment, irrespective of ECMO-treatment. We longitudinally assessed these children's motor function. Methods: Children with CDH with and without ECMO-treatment, born 1999-2007, who joined our structural prospective follow-up program were assessed with the Movement Assessment Battery for Children (M-ABC) at 5, 8, 12 years. Z-scores were used in a general linear model for longitudinal analysis. Results: We included 55 children, of whom 25 had been treated with ECMO. Forty-three (78%) were evaluated at three ages. Estimated mean (95% CI) z-scores from the general linear model were -0.67 (-0.96 to -0.39) at 5 years of age, -0.35 (-0.65 to -0.05) at 8 years, and -0.46 (-0.76 to -0.17) at 12 years. The 5- and 8-years scores differed significantly (p = 0.02). Motor development was significantly below the norm in non-ECMO treated patients at five years; -0.44 (-0.83 to -0.05), and at all ages in the ECMO-treated-patients: -0.90 (-1.32 to -0.49), -0.45 (-0.90 to -0.02) and -0.75 (-1.2 to -0.34) at 5, 8, and 12 years, respectively. Length of hospital stay was negatively associated with estimated total z-score M-ABC (p = 0.004 multivariate analysis). Conclusion: School-age children born with CDH are at risk for motor function impairment, which persists in those who received ECMO-treatment. Especially for them long-term follow up is recommended.

Associations of perioperative characteristics with motor function in preschool children born with esophageal atresia.

BACKGROUND: Children born with esophageal atresia experience long-term neurodevelopmental deficits, with unknown origin. AIMS: To find associations between perioperative variables during primary esophageal atresia repair and motor function at age 5 years. METHODS: This ambidirectional cohort study included children born with esophageal atresia who consecutively had been operated on in the Erasmus MC-Sophia Children's Hospital, University Medical Center, Rotterdam, from January 2007 through June 2013. The perioperative data of this cohort were collected retrospectively; the motor function data prospectively. RESULTS: After exclusion of patients with syndromal congenital diseases (n = 8) and lost to follow-up (n = 10), the data of 53 children were included. The mean (SD) total motor function impairment z-score at 5 years of age was -0.66 (0.99), significantly below normal (p < .001). In multivariable linear regression analysis, number of postoperative days endotracheal intubation (B = -0.211, 95% CI: -0.389 to -0.033, p = .021) was negatively associated with motor outcome, whereas high blood pressure (B = 0.022, 95% CI 0.001 to 0.042, p = .038) was positively associated. Preoperative nasal oxygen supplementation versus room air (B = 0.706, 95% CI: 0.132 to 1.280, p = .016) was positively associated with motor outcome, which we cannot explain. CONCLUSIONS: Motor function in 5-year-old esophageal atresia patients was impaired and negatively associated with the number of postoperative days of endotracheal intubation and positively associated with high blood pressure. Prospective studies with critical perioperative monitoring and monitoring during stay at the intensive care unit are recommended.

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 children born with severe anatomical anomalies and/or treated with neonatal extracorporeal membrane oxygenation. METHODS: We analysed data of 190/253 children (60.0% male) participating in our multidisciplinary follow-up programme who were routinely assessed at the ages of five, eight and/or 12 years. Parents completed the Checklist prior to assessment of the child's actual motor performance by a physical therapist using the MABC-2 Test. The sensitivity and specificity of the Checklist with a cut-off point of the 16th percentile were determined. RESULTS: The sensitivity of the MABC-2 Checklist was 57.1%, which implies that 42.9% of the children at risk for motor problems were not identified. The specificity was 79.1%. CONCLUSION: The low sensitivity of the MABC-2 Checklist suggests that this instrument does not suffice to screen for motor problems in children who survived neonatal critical illness. Yet, it may help to gain insight in parental perceptions of the child's motor performance and to provide tailored advice on lifestyle.

Improvement of exercise capacity following neonatal respiratory failure: A randomized controlled trial.

Exercise capacity deteriorates in school-aged children born with major anatomical foregut anomalies and/or treated with extracorporeal membrane oxygenation. The aim of the present study was to evaluate whether exercise capacity can be improved in the short term and long term in children born with anatomical foregut anomalies and/or treated with extracorporeal membrane oxygenation. Therefore, we evaluated two different interventions in this single-blinded randomized controlled trial. Forty participants were randomly assigned to group A: standardized anaerobic high-intensity interval training plus online lifestyle coaching program, B: online lifestyle coaching program only, or C: standard of care. Inclusion criteria were as follows: score ≤-1 standard deviation (SD) on the Bruce protocol. Exercise capacity was assessed at baseline (T0), after 3 months (T1), and after 12 months (T2). Exercise capacity improved over time: mean (SD) standard deviation score (SDS) endurance time: T0 -1.91 (0.73); T1 -1.35 (0.94); T2 -1.20 (1.03): both P < .001. No significant differences in maximal endurance time were found at T1 (group A-C: estimated mean difference (SDS): 0.06 P = .802; group B-C: -0.17 P = .733) or T2 (group A-C: -0.13 P = .635; group B-C: -0.18 P = .587). Exercise capacity improved significantly over time, irrespective of the study arm. Not only residual morbidities may be responsible for reduced exercise capacity. Parental awareness of reduced exercise capacity rather than specific interventions may have contributed. Monitoring of exercise tolerance and providing counseling on lifestyle factors that improve physical activity should be part of routine care, and aftercare should be offered on an individual basis.

Lung function in school-aged congenital diaphragmatic hernia patients; a longitudinal evaluation.

OBJECTIVE: Children with congenital diaphragmatic hernia (CDH) are at risk for pulmonary morbidity. Data on longitudinal evaluation of lung function in CDH are scarce. We hypothesized that CDH patients would have impaired lung function that worsens over time. We evaluated lung function and its determinants at ages 8 and 12 years. METHODS: Dynamic and static lung volumes, and diffusion capacity were measured. Extracorporeal membrane oxygenation (ECMO) treatment, the standardized European neonatal treatment protocol, patch repair, duration of ventilation, type of initial mechanical ventilation, and nitric oxide treatment were entered as covariates in linear mixed models with standard deviation score (SDS) lung function parameters (FEV1 , FEF 25-75 , and K CO ) as dependent variables. RESULTS: Seventy-six children (27 ECMO-treated) born between 1999 and 2009 performed 113 reliable lung function tests. Severity of airflow obstruction deteriorated significantly from age 8 to 12 years: estimated mean difference (95% confidence interval [CI]) SDS FEV1 was -0.57 (-0.79 to -0.36) and SDS FEF25-75 was -0.63 (-0.89 to -0.37), both P < .001. Static lung volumes were within normal range and unchanged over time: estimated mean difference (95% CI) SDS TLC -0.27 (-0.58 to 0.04); P = .085. SDS KCO was below normal at 8 and 12 years and remained stable: -0.06 (-0.22 to 0.35); P = .648. These observations were irrespective of ECMO treatment. FEV1 and FEF25-75 were negatively associated with duration of ventilation (P < .001). Baseline data were not related with TLC or KCO. CONCLUSIONS: CDH patients should be followed into adulthood as they are at risk for worsening airflow obstruction and decreased diffusion capacity at school age, irrespective of ECMO treatment.

Lung function, exercise tolerance, and physical growth of children with congenital lung malformations at 8 years of age.

OBJECTIVE: To improve counseling on congenital lung malformations (CLM) by describing long-term outcomes of children either operated on or managed by observation. STUDY DESIGN: We analyzed lung function (spirometry), exercise tolerance (Bruce treadmill), and physical growth of 8-year-old children with CLM who participated in our longitudinal prospective follow-up program. The data are shown as median standard deviation scores (SDS) with IQR, or estimated marginal means (95% CI) on the basis of general linear models. RESULTS: Twenty-nine (48%) of the 61 children had required surgery at a median age of 108 (IQR: 8-828) days, and 32 (52%) were managed by observation. In the surgery group, all lung function measurements (except for forced vital capacity [FVC]) were significantly below 0 SDS, with median FEV1 -1.07 (IQR: -1.70 to -0.56), FEV1 /FVC -1.49 (-2.62 to -0.33), and FEF25%-75% -1.95 (-2.57 to -0.63) (all P < 0.001). Children in the observation group had normal FEV1 and FVC, whereas FEV1 /FVC (-0.81 (-1.65 to -0.14)) and FEF25%-75% (-1.14 (-1.71 to -0.22)) were significantly below 0 SDS (both P < 0.001). Mean exercise tolerance was significantly below 0 SDS in both groups (observation: -0.85 (95% CI: -1.30 to -0.41); surgery: -1.25 (-1.69 to -0.80)); eight (28%) children in the observation group and ten (40%) in the surgery group scored <-1 SDS. Physical growth was normal in both groups. CONCLUSION: Children with CLM may be at risk for reduced lung function and exercise tolerance, especially those who required surgery. As little pulmonary morbidity was found in children with asymptomatic CLM, this study supports a watchful waiting approach in this group.

Congenital diaphragmatic hernia and exercise capacity, a longitudinal evaluation.

OBJECTIVE: Children with congenital diaphragmatic hernia (CDH) suffer from long-term pulmonary morbidity. Longitudinal data of exercise capacity in these children are lacking. We hypothesized that exercise capacity would be impaired in children with CDH and deteriorates over time. We evaluated exercise capacity and its determinants in CDH patients longitudinally until 12 years of age. DESIGN: Prospective longitudinal follow-up study in tertiary university hospital. PATIENTS: One hundred and fourteen children with CDH born between 1999 and 2012. METHODS: Exercise capacity was evaluated using the Bruce treadmill-protocol at the ages of 5, 8, and 12 years. Primary outcome parameter was standard deviation score (SDS) of maximal endurance time. Data were analyzed by using linear mixed models. RESULTS: A total of 107 children (30 treated with extracorporeal membrane oxygenation [ECMO]) performed 191 reliable exercise tests. At ages 5, 8, and 12 years, the mean (95%CI) SDS endurance time was -0.44 (-0.65 to -0.24); -1.01 (-1.23 to -0.78); -1.10 (-1.40 to -0.80), respectively, all less than zero (P < 0.001). Exercise capacity declined significantly over time irrespective of ECMO-treatment (5-12 years: non-ECMO P = 0.015; ECMO P = 0.006). Duration of initial hospital stay and diffusion capacity corrected for alveolar volume were associated with SDS endurance time (P < 0.001 and P = 0.039). CONCLUSIONS: In CDH patients exercise capacity deteriorates between 5 and 12 years of age, irrespective of ECMO-treatment. CDH patients may benefit from long-term assessments of exercise capacity with timely intervention.

Determinants of exercise capacity in school-aged esophageal atresia patients.

BACKGROUND AND AIMS: Data on long-term outcome of exercise capacity in school-aged children with esophageal atresia (EA) are scarce. We evaluated maximal exercise capacity and its relation to lung function. Moreover, we studied other possible determinants of exercise capacity and lung function. METHODS: Exercise capacity of 63 children with EA born 1999-2007 was evaluated at the age of 8 years with the Bruce-protocol. Dynamic and static lung volumes, bronchodilator response and diffusion capacity were measured. Furthermore, perinatal characteristics, hospital admissions for lower respiratory tract infections (RTIs), RTIs treated with antibiotics in the past year, symptoms of gastroesophageal reflux, weight-for-height, and sports participation were evaluated as other potential determinants. RESULTS: Exercise capacity was significantly below normal: mean (SD) SDS -0.91 (0.97); P < 0.001. All spirometric parameters were significantly below normal with significant reversibility of airflow obstruction in 13.5% of patients. Static lung volumes were significantly decreased (mean (SD) SDS TLChe -1.06 (1.29); P < 0.001). Diffusion capacity corrected for alveolar volume was normal (mean (SD) SDS KCO -0.12 (1.04)). Exercise capacity was positively associated with total lung capacity and negatively with SDS weight-for-height. Spirometric parameters were negatively associated with congenital cardiac malformation, duration of ventilation, and persistent respiratory morbidity. CONCLUSION: Eight-year-old children with EA had reduced exercise capacity which was only associated with the reduction in TLChe and higher SDS weight-for-height. We speculate that diminished physical activity with recurrent respiratory tract infections may also play a role in reduced exercise capacity. This should be subject to further research to optimize appropriate intervention.

Developmental problems in patients with oesophageal atresia: a longitudinal follow-up study.

OBJECTIVE: To longitudinally evaluate motor development and predictive factors in school-age children with oesophageal atresia. DESIGN: Cohort study with prospective longitudinal follow-up. SETTING: Outpatient clinic of a tertiary university paediatric hospital. PATIENTS: Children with oesophageal atresia born between January 1999 and May 2006 were assessed at 5 and 8 years of age. INTERVENTIONS: None. MAIN OUTCOME: Motor performance was evaluated at 5 and 8 years using the Movement Assessment Battery for Children (M-ABC). Additionally, we evaluated perinatal characteristics, duration of anaesthesia within the first 24 months, socioeconomic status, sports participation and school performance at time of follow-up and intelligence and sustained attention at the age of 8 years. RESULTS: In 5-year-olds (n=54), the mean (SD) z-score M-ABC was slightly, but significantly lower than age-predicted normative values (-0.75 (0.83), p<0.001). In 8-year-olds (n=49), the z-score M-ABC was -0.53 (0.91) (p<0.001), intelligence was normal, but sustained attention was impaired: z-score speed (-1.50 (1.73)) and raw score attentional fluctuation (3.99 (1.90)) (both p<0.001). Motor problems mainly concerned gross motor performance. Duration of anaesthesia and sustained attention were negatively associated with motor development; sports participation was positively associated. CONCLUSIONS: Longer duration of anaesthesia and sustained attention problems were associated with gross motor problems in school-age patients with oesophageal atresia. Parental awareness of risks for motor problems may provide the opportunity to offer timely intervention.

Perceived Motor Competence Differs From Actual Performance in 8-Year-Old Neonatal ECMO Survivors.

OBJECTIVE: To assess perceived motor competence, social competence, self-worth, health-related quality of life, and actual motor performancein 8-year-old survivors of neonatal extracorporeal membrane oxygenation (ECMO). METHODS: In a prospective nationwide study, 135 children completed the extended version of the "athletic competence" domain of the Self Perception Profile for Children (SPPC) called the m-CBSK (Motor supplement of the Competentie BelevingsSchaal voor Kinderen) to assess perceived motor competence, the SPPC, and the Pediatric Quality of Life Inventory (PedsQL), andwere tested with the Movement Assessment Battery for Children. SD scores (SDS) were used to compare with the norm. RESULTS: The mean (SD) SDS for perceived motor competence, social competence, and self-worth were all significantly higher than the norm: 0.18 (0.94), P = .03; 0.35 (1.03), P < .001; and 0.32 (1.08), P < .001, respectively. The total PedsQL score was significantly below the norm: mean (SD) SDS: -1.26 (1.53), P < .001. Twenty-two percent of children had actual motor problems. The SDS m-CBSK and actual motor performance did not correlate (r = 0.12; P = .17). The SDS m-CBSK significantly correlated with the athletic competence domain of the SPPC (r = 0.63; P < .001). CONCLUSIONS: Eight-year-old ECMO survivors feel satisfied with their motor- and social competence, despite impaired PedsQL scores and motor problems. Because motor problems in ECMO survivorsdeteriorate throughout childhood, clinicians should be aware that these patients may tend to "overrate" their actual motor performance. Education andstrict monitoring of actual motor performanceare important to enable timelyintervention.

Motor performance after neonatal extracorporeal membrane oxygenation: a longitudinal evaluation.

OBJECTIVE: To assess longitudinally children's motor performance 5 to 12 years after neonatal extracorporeal membrane oxygenation (ECMO) and to evaluate associations between clinical characteristics and motor performance. METHODS: Two hundred fifty-four neonatal ECMO survivors in the Netherlands were tested with the Movement Assessment Battery for Children at 5, 8, and/or 12 years. Percentile scores were transformed to z scores for longitudinal evaluation (norm population mean = 0 and SD = 1). Primary diagnoses: meconium aspiration syndrome (n = 137), congenital diaphragmatic hernia (n = 49), persistent pulmonary hypertension of the newborn (n = 36), other diagnoses (n = 32). RESULTS: Four hundred fifty-six tests were analyzed. At 5, 8, and 12 years motor performance was normal in 73.7, 74.8, and 40.5%, respectively (vs 85% expected based on reference values; P < .001 at all ages). In longitudinal analyses mean (95% confidence interval [CI]) z scores were -0.42 (-0.55 to -0.28), -0.25 (-0.40 to -0.10) and -1.00 (-1.26 to -0.75) at 5, 8, and 12 years, respectively. Mean score at 8 years was significantly higher than at 5 years (difference 0.16, 95% CI 0.02 to 0.30), and mean score at 12 years was significantly lower than at both other ages (differences -0.59 and -0.75; 95% CI -0.33 to -0.84 and -0.49 to -1.00, respectively). Children with congenital diaphragmatic hernia encountered problems at all ages. The presence of chronic lung disease was negatively related with outcome. CONCLUSIONS: Motor problems in neonatal ECMO survivors persist throughout childhood and become more obvious with time.

Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation.

BACKGROUND: Survival rates of patients with congenital diaphragmatic hernia (CDH) have improved to up to 80%. Little is known about long-term consequences of the disease and its treatment. We evaluated lung function and respiratory symptoms longitudinally in a previously studied cohort of CDH patients and age-matched non-CDH patients who underwent similar neonatal intensive care treatment. STUDY DESIGN: We tested 27 young adults [mean (SD) age: 26.8 years (2.9)] with CDH and 30 non-CDH patients. Dynamic and static lung volumes, midexpiratory flows, and diffusion capacity were expressed as mean (SD) standard deviation scores. Prevalence of respiratory symptoms was evaluated with the European Community Respiratory Health Survey. RESULTS: All data are expressed as mean (SD). FEF(25-75) in CDH patients had slightly deteriorated since childhood (CDH: -0.7 (1.4) vs. -1.6 (1.5), P < 0.001; non-CDH patients: 0.2 (1.4) vs. -0.3 (1.6), P = 0.038, ns). Diffusion capacity decreased in both groups (CDH: DL(CO) c 0.2 (1.1) vs. -1.5 (1.1), P <0.001; non-CDH: DL(CO) c 0.1 (0.4) vs. -1.1 (1.1), P < 0.001). Lung volumes were normal in both groups. The prevalence of asthma was higher than in the normal population (27.6% in patients and 30% in controls, P < 0.001). CONCLUSIONS: Airflow obstruction and diffusion capacity deteriorated mildly from childhood into adulthood in survivors of CDH. The improved survival of patients with more severe forms of diaphragmatic hernia calls for long-term follow-up of lung function.

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.

Early developmental assessment of children with major non-cardiac congenital anomalies predicts development at the age of 5 years.

AIM: the aim of this study was to evaluate cognitive and motor development in children with major congenital anomalies and the predictability of development at age 5 years. METHOD: a prospective, longitudinal follow-up study was undertaken. The Dutch version of the Bayley Scales of Infant Development - Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) - were administered at the ages of 6, 12, and 24 months. The Revised Amsterdam Children's Intelligence Test - IQ and the Movement Assessment Battery for Children - Total impairment score (TIS) were used at age 5 years. A total of 117 children participated in the study. After excluding 12 children who had a major chromosomal or syndromal abnormality, the analysis was limited to 105 children (50 females, 55 males). Seven groups of congenital anomalies were distinguished: (1) small intestinal anomalies; (2) abdominal wall defects, comprising gastroschisis and omphalocele; (3) oesophageal atresia; (4) congenital diaphragmatic hernia; (5) Hirschsprung disease; (6) anorectal malformations; and (7) miscellaneous diagnoses. Logistic regression analyses served to determine the ability of MDI and PDI to predict IQ and TIS at age 5 years. RESULTS: at age five, 83.7% of 104 children had an IQ of 85 or above and 16.3% an IQ of less than 85. TIS was normal in 71.3% of 87 children, while 17.2% demonstrated a borderline score and 11.5% a definite motor problem. MDI and PDI scores showed equal sensitivity to predict IQ (p=0.004 at 6 and 12mo, p=0.001 at 24mo) and TIS (p<0.001 at 6 and 12mo, p=0.002 at 24mo). MDI and PDI were positively correlated with IQ and TIS; TIS was positively correlated with IQ. INTERPRETATION: IQ scores at 5 years of age corresponded to Dutch population scores, but TIS scores differed significantly. Early development of children with major congenital anomalies is predictive of development at 5 years, which can guide individualized follow-up for this vulnerable group of children.

Motor-function and exercise capacity in children with major anatomical congenital anomalies: an evaluation at 5 years of age.

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.

Exercise testing of pre-school children using the Bruce treadmill protocol: new reference values.

The Bruce treadmill protocol is an often-used exercise test for children and adults. Few and mainly old normative data are available for young children. In this cross-sectional observational study we determined new reference values for the original Bruce protocol in children aged 4 and 5 years. Furthermore, we compared the original protocol with the so-called 'half Bruce' protocol. In the Netherlands this half-Bruce protocol is often used for young children because of the rather large increments in workload in the original protocol. Seventy-eight healthy Dutch children participated. The maximal endurance time was the criterion of exercise capacity. The new reference values for the original Bruce protocol are presented as reference centiles. The mean (SD) endurance time using the original protocol was 10.2 (SD 1.5) min.; this was 9.4 (1.3) min. for the half-Bruce protocol. The mean difference was 50 seconds (95% CI: 29-71 s, P < 0.001). So, for children aged 4 and 5 years the endurance times obtained with the original and half-Bruce protocol are different and should not be considered interchangeable. Our new reference values can be used as reference values for the original Bruce protocol.

A prospective comparative evaluation of persistent respiratory morbidity in esophageal atresia and congenital diaphragmatic hernia survivors.

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. CONCLUSIONS: Esophageal atresia and CDH are associated with equal risk of long-term respiratory morbidity, growth impairment, and disturbed maximal exercise performance. Prospective follow-up of EA patients aimed at identifying respiratory problems other than tracheomalacia should be an integral part of interdisciplinary follow-up programs.

Motor performance in five-year-old extracorporeal membrane oxygenation survivors: a population-based study.

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 intelligence quotient (IQ) scores (r = 0.48, P < 0.001). CONCLUSIONS: The ECMO population is highly at risk for developmental problems, most prominently in the motor domain. Adverse outcome differs between the primary diagnosis groups. Objective evaluation of long-term developmental problems associated with this highly invasive technology is necessary to determine best evidence-based practice. The ideal follow-up programme requires an interdisciplinary team, the use of normal-referenced tests and an international consensus on timing and actual outcome measurements.

Follow-up of newborns treated with extracorporeal membrane oxygenation: a nationwide evaluation at 5 years of age.

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.