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 [corrected] . 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.

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.

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.

Interdisciplinary structural follow-up of surgical newborns: a prospective evaluation.

BACKGROUND: Information on physical and developmental outcomes of children with anatomical congenital anomalies (CAs) may indicate the need for early intervention and reduce impact on the child's life and parental burden. METHODS: From 1999 to 2003, 101 children with CA (76.5% of initial survivors) were seen 6-monthly in a tertiary children's hospital. Growth, neurologic outcome, mental and psychomotor development as determined with the Bayley Scales of Infant Development, and categorization of predictive sociodemographic and medical variables of the children were evaluated prospectively and longitudinally. RESULTS: Congenital diaphragmatic hernia (CDH) and esophageal atresia patients showed impaired growth, that is, both height for age (-1.5 standard deviation score [SDS]) and weight for height (-1.0 SDS). Overall neurologic outcome was normal, however, suspect or abnormal for 40% of CDH patients. Overall mental development was normal, but psychomotor scores were significantly lower than the norm (95% confidence interval, 83.8-92.2 at 6 months and 87.9-98.5 at 24 months). Sex, maternal age, socioeconomic status, CA, severity-of-disease covariables, and need of medical appliances at home could predict negative outcome significantly (P < .05). CONCLUSIONS: The CA survivors show impaired growth and psychomotor developmental delay up to age 2 years. This warrants specific follow-up programs and infrastructure for these patients.

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.

Impact of a child with congenital anomalies on parents (ICCAP) questionnaire; a psychometric analysis.

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 fathers showed changes on subscales (Cohen's d varied from .07 to .49), while the test-retest reliability estimates varied from .42 to .91. CONCLUSION: The ICCAP is a reliable and valid instrument for clinical practice. It enables early signaling of parental quality of life as a result of early stress, and thus early intervention.

Telephone helpline for parents of children with congenital anomalies.

AIM: This paper is a report of a study to evaluate how often and for what reason parents of children born with severe anatomical congenital anomalies use a 24-hour telephone helpline, and to identify differences between callers and non-callers. BACKGROUND: Children born with severe congenital anomalies often remain dependent on medical care and technology after discharge. Adequate medical consultation in the home situation may lower parental burden. METHOD: Observational study of telephone contacts from 2000 to 2006 with parents of congenital anomaly patients discharged home after neonatal intensive care admission. Frequency of telephone calls was categorized per type of anomaly. Resulting interventions in terms of consultation and hospital admission were recorded. Finally, costs of personnel needed to provide 24-hour telephone helpline availability were calculated. FINDINGS: A total of 670 calls occurred outside office hours, from 34.4% of all parents; 23.7% of these calls concerned feeding problems. Parents of children with oesophageal atresia, urogenital malformation and congenital diaphragmatic hernia called most frequently (44.3-50.6%). Non-callers were more often first-time parents, divorced/separated or immigrants. Nurses handled 24.5% of calls by themselves and 20.2% of calls led to emergency room consultations resulting in 4.9% admissions. First contact took place at median age 3 months, last contact at median age 8 months. Total personnel costs amounted to euro27,191 per year. CONCLUSION: A 24-hour telephone helpline provides easy access to medical and supportive care for parents of children with congenital anomalies at relatively low cost. Nurses can effectively run this telephone helpline with paediatrician back-up.

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.