Congenital Diaphragmatic Hernia Survivors Research Articles

Educational level and socioeconomic status in patients born with congenital diaphragmatic hernia: A population-based study

BackgroundNeurodevelopmental dysfunction is one of the most disabling outcomes for congenital diaphragmatic hernia (CDH) survivors and may have a long lasting impact in adult life. AimTo evaluate to which extent being born with CDH has an impact on the educational level and socioeconomic status as a proxy for neurocognitive development. Material and methodsNationwide, population-based prospective study of newborn children in Sweden from 1982 to 2015. School grades, highest educational level and income were assessed through Swedish public registries. Children above 15 years of age with CDH were compared with randomly selected controls. ResultsA significantly higher number of cases (17% vs 10%) did achieve neither a school nor a university degree. Among those who achieved a degree there was no difference in the highest level of education. The qualification points in elementary school did not differ, but in high school female cases had significantly lower qualification points than female controls. There were no differences in individual disposable income between cases and controls. However, males had higher income compared to females. Prematurity and a long hospital stay had a negative impact on educational level. ConclusionsA higher proportion of children born with CDH compared to controls do not achieve a school degree. Among those who achieved a degree, the school achievements and educational level were similar to controls. Prematurity and a long hospital stay are risk factors for not achieving an educational degree. Type of studyPrognosis study (high-quality prospective cohort study with 99% of patients followed to the study end point). Level of evidenceLevel I.I for a prognosis study — This is a high-quality, prospective cohort study with 99% of patients followed to the study end point.

Asthma Medication Use in Congenital Diaphragmatic Hernia Survivors: A Retrospective Population Level Data Analysis.

The purpose of this study was to determine if congenital diaphragmatic hernia (CDH) survivors had worse long-term respiratory outcomes compared with age-matched controls, as measured by inhaled bronchodilator use, inhaled steroid use, and asthma-related physician visits. We performed a retrospective case-control study of infants with isolated CDH from 1991 to 2013. The primary outcome measures were inhaled bronchodilator prescriptions, inhaled steroid prescriptions, and asthma-related physician visits between 0 and 5 years of age and between 5 and 10 years of age. Subgroup analysis compared the same outcomes for CDH patients grouped by: birth weight, gestational age, side of defect, defect size, liver herniation, hernia sac, and pulmonary hypertension. Fifty-six patients with CDH and 753 age-matched controls met the inclusion criteria for the 0 to 5 years of age analysis. Between 0 and 5 years of age, more CDH survivors were prescribed an inhaled bronchodilator (odds ratio [OR] = 2.47[1.38-4.48], p = 0.001) and inhaled steroid (OR = 2.03[1.07-3.74], p = 0.03), and had an asthma-related physician visit (OR = 1.92[1.00-3.56], p = 0.04). Thirty-eight cases and 491 controls met the inclusion criteria for the 5 to 10 years of age analysis. Between 5 and 10 years of age, CDH survivors were not more likely to be prescribed inhaled bronchodilators, inhaled steroids, or have an asthma-related physician visit. Among the CDH patients, we did not find a clinical characteristic associated with increased inhaled bronchodilator or steroid prescriptions at any age. A history of CDH is associated with higher rates of inhaled bronchodilator prescriptions, inhaled steroid prescriptions, and asthma-related physician visits from 0 to 5 years of age compared with age-matched controls. However, this difference resolves by 5 to 10 years of age.

Long-term feeding issue and its impact on the daily life of congenital diaphragmatic hernia survivors: results of the first patient-led survey

BackgroundCDH UK is a registered charity governed by a volunteer committee and providing informal support to patients, families and healthcare workers affected directly or indirectly with congenital diaphragmatic hernia (CDH) internationally. This is the first patient-led survey undertaken by CDH UK aiming for highlighting the feeding problems and their impact on the daily life of CDH survivors.MethodsAnswers from CDH survivors were collected through an online questionnaire (SurveyMonkey®) undertaken by CDH UK. The questionnaire contained questions about their feeding problems and support they were receiving for it.Main resultsOverall, 151 patients answered some parts of the survey and 102 patients completed the questionnaire. Overall, 116 (76.8%) responders reported suffering from any type of feeding issue. Gastric acid reflux (GER) and growth retardation were the commonest symptoms experienced by 97 (91.5%) and 72 (62.2%) responders, respectively. Only 18 (17.0%) responders have received any written information on feeding or details of patient/parent support. Eighty (75.5%) responders are satisfied with the level of support they are receiving, but 78 (76.4%) answered that the whole experience associated with the disease has been very or extremely stressful.ConclusionsCDH survivors frequently have various issues with feeding, which may not be adequately supported or discussed clinically. It is desirable to assist the patients to reliable resources of long-term support, including multidisciplinary team (MDT) approach.

Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia

To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic. This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1second (FEV1pp), percent predicted forced vital capacity (FVCpp), and FEV1/FVC over time. Of 268 patients with CDH who survived to discharge, 119 had at least 1 pulmonary function test study. The FEV1pp (P<.001), FVCpp (P=.017), and FEV1/FVC (P=.001) decreased with age. Compared with defect size A/B, those with defect size C/D had lower FEV1pp by an average of 11.5% (95% CI, 2.9%-20.1%; P=.010). A history of oxygen use at initial hospital discharge also correlated with decreased FEV1pp by an average of 8.0% (95% CI, 1.2%-15.0%; P=.023). In a select cohort of CDH survivors, average pulmonary function declines with age relative to expected population normative values. Those with severe CDH represent a population at risk for worsening pulmonary function test measurements who may benefit from recognition and monitoring for complications.

Longitudinal Analysis of Ventilation Perfusion Mismatch in Congenital Diaphragmatic Hernia Survivors

To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH). This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model. During the study period, 212 patients had at least oneV/Q study. The average ipsilateral V/Q of the cohort increased over time (P<.01), an effect driven by progressive reduction in relative perfusion (P=.012). A higher V/Q ratio was correlated with lower body mass index percentile (P<.001) and higher probability of poor functional status (New York Heart Association class III or IV) (P=.045). In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.

Does Timing and Duration of Inhaled Nitric Oxide before Extracorporeal Membrane Oxygenation in Congenital Diaphragmatic Hernia Survivors Predict Respiratory Morbidity at Discharge?

Does Timing and Duration of Inhaled Nitric Oxide before Extracorporeal Membrane Oxygenation in Congenital Diaphragmatic Hernia Survivors Predict Respiratory Morbidity at Discharge?

Prenatal Imaging Features and Postnatal Factors Associated with Gastrointestinal Morbidity in Congenital Diaphragmatic Hernia

Background: To perform a comprehensive assessment of postnatal gastrointestinal (GI) morbidity and determine the prenatal imaging features and postnatal factors associated with its development in patients with congenital diaphragmatic hernia (CDH). Materials and Methods: A retrospective review was conducted of all infants evaluated for CDH at a quaternary fetal center from February 2004 to May 2017. Prenatal imaging features and postnatal variables were analyzed. GI morbidity was the primary outcome. The Mann-Whitney U test, the Kruskal-Wallis test with Dunnett’s T3 post hoc analysis and logistic regression, and the χ² test were performed when appropriate. Results: We evaluated 256 infants; 191 (75%) underwent CDH repair and had at least 6 months of follow-up. Of this cohort, 60% had gastroesophageal reflux disease (GERD), 13% had gastroparesis, 32% received a gastrostomy tube (G-tube), and 17% needed a fundoplication. Large defect, patch repair, extracorporeal membrane oxygenation (ECMO), and prolonged use of mechanical ventilation were significantly associated with having GERD, gastroparesis, G-tube placement, and fundoplication (p < 0.05). Fetuses with stomach grades 3 and 4 were most likely to have GERD, a G-tube, and a long-term need for supplemental nutrition than fetuses with stomach grades 1 and 2 (p < 0.05). Conclusion: Survivors of CDH with large defects, prolonged use of mechanical ventilation, or that have received ECMO may be at an increased risk for having GERD, gastroparesis, and major GI surgery. Marked stomach displacement on prenatal imaging is significantly associated with GI morbidity in left-sided CDH.

Hospital readmission and long-term morbidities of neonates with congenital diaphragmatic hernia treated in children’s hospitals

Purpose: 1) To describe the rates of readmission and patient characteristics of CDH neonates who were readmitted after their index hospital visit and 2) To examine the risk of admission for morbidities among CDH neonates. Background: Congenital diaphragmatic hernia (CDH) survivors may suffer long-term morbidities, such as respiratory, neurological, and gastrointestinal diseases. Few have studied the risk of readmission for those morbidities among CDH survivors after initial hospital discharge at a national level. Methods: Data were obtained from the Pediatric Healthcare Information System (PHIS) database from January 1st, 2004 …

Long-term medical and psychosocial outcomes in congenital diaphragmatic hernia survivors

ObjectiveSurvival rates for congenital diaphragmatic hernia (CDH) are increasing. The long-term outcomes of CDH survivors were compared with a healthy control group to assess the morbidity for guidance of antenatal...

MIF inhibition enhances pulmonary angiogenesis and lung development in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a complex birth anomaly with significant mortality and morbidity. Lung hypoplasia and persistent pulmonary hypertension (PPHN) limit survival in CDH. Macrophage migration inhibitory factor (MIF), a key regulator of innate immunity, is involved in hypoxia-induced vascular remodeling and PPHN. We hypothesized that antenatal inhibition of MIF in CDH fetuses, would reduce vascular remodeling, and improve angiogenesis and lung development. Pregnant rats were randomized into three groups: Control, nitrofen, and nitrofen + ISO-92. Lung volumes of pups were measured by CT scanning. Right ventricular systolic pressure (RVSP) and vascular wall thickness (VWT) were measured together with MIF concentration, angiogenesis markers, lung morphometry, and histology. Prenatal treatment with ISO-92, an MIF inhibitor, improved normalization of static lung volume, lung volume-to-body weight ratio, decreased alveolar septal thickness, RVSP and VWT and improved radial alveolar count as compared to the non-treated group. Expression of MIF was unaffected by ISO-92; however, ISO-92 increased p-eNOS and VEGF activities and reduced arginase 1, 2 and Sflt-1. Prenatal inhibition of MIF activity in CDH rat model improves angiogenesis and lung development. This selective intervention may be a future therapeutic strategy to reduce the morbidity and mortality of this devastating condition.

Application of a postnatal prediction model of survival in CDH in the era of fetal therapy

Background: The disease severity in patients with a congenital diaphragmatic hernia (CDH) is highly variable. To compare patient outcomes, set up clinical trials and come to severity-based treatment guidelines, a performant prediction tool early in neonatal life is needed.Objective: The primary purpose of this study was to validate the CDH study group (SG) prediction model for survival in neonates with CDH, including patients who had fetal therapy. Secondary, we aimed to assess its predictive value for early morbidity.Methods: This is a retrospective single-center study at the University Hospitals Leuven on all infants with a diagnosis of CDH live-born between April 2002 and December 2016. The prediction model of the CDHSG was applied to evaluate its performance in determining mortality risk. Besides, we examined its predictive value for early morbidity parameters, including duration of ventilation, respiratory support on day 30, time to full enteral feeding and length of hospital stay.Results: The CDHSG prediction model predicted survival well, with an area under the curve of 0.796 (CI: 0.720–0.871). It had poor value in predicting infants who needed respiratory support on day 30 (area under the curve (AUC) 0.606; CI: 0.493–0.719), and correlated poorly with duration of ventilation, time to full enteral feeding and length of hospital stay.Conclusion: The CDHSG prediction model was in our hands also a useful tool in predicting mortality in neonates with CDH in the fetal treatment era. Correlation with early morbidity was poor.RationaleObjectives: (1) Validation of the CDHSG prediction model for survival in a cohort of neonates with CDH, in whom fetal endoscopic tracheal occlusion was applied according to the severity of lung hypoplasia. (2) Evaluation of performance of the model in the prediction of early morbidity.Main results: (1) Confirmation of the predictive value of the model for survival in neonates with CDH in the era of fetal therapy. (2) No correlation of the model with early morbidity parameters.

Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia.

The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors. A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance. Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group. Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood.

Health-related quality of life, educational and family outcomes in survivors of congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) survivors experience increased risk of medical and neurodevelopmental challenges. This study describes the health-related quality of life (HRQOL), special education utilization and the family impact among neonatal CDH survivors. A single-center prospective cohort of CDH survivors born between 1995 and 2006 was followed. Parents completed the PedsQL HRQOL index and a Family Impact survey to assess the need for medical equipment, home health services, and special education and quantify the burden placed on the family by their child's medical needs. Parents of 32 survivors participated at a mean survivor age of 8 ± 4years. Many survivors utilized medical equipment (62%), home health services (18%) and special education (28%). CDH survivor HRQOL (79 ± 17) did not differ significantly from that of healthy children (83 ± 15, p = 0.12). HRQOL was diminished among survivors who required special education (67 ± 8 vs 82 ± 3; p = 0.04) and those reporting increased Family Impact score (p = 0.001). Many CDH survivors continue to require home medical equipment and home health services at school age. Most survivors have normal parent-reported HRQOL; however, the need for special education and higher family impact of neonatal CDH correlates with decreased HRQOL.

Fetal brain morphometry on prenatal magnetic resonance imaging in congenital diaphragmatic hernia

Many infants with congenital diaphragmatic hernia (CDH) show brain abnormality on postnatal brain MRI related to severity of CDH, degree of lung hypoplasia, intrathoracic liver, right diaphragmatic hernia and large diaphragmatic defect. It is not known whether these factors affect brain growth in utero in CDH. To assess prenatal brain morphometry and abnormalities on fetal MR in congenital diaphragmatic hernia. We retrospectively reviewed 109 fetal MRIs in 63 fetuses with CDH from 2009 to 2014 (27 died before discharge, 36 survived to discharge). We compared brain injury and gestational-age-corrected z-scores of brain measurements between survivors and non-survivors. We assessed correlations between brain abnormalities and CDH severity. Enlarged extraaxial space was the most common abnormality, frequently seen on fetal MRI at >28weeks of gestation, similar in survivors versus non-survivors. Anteroposterior cerebellar vermis dimension at >28weeks of gestation was smaller in non-survivors compared to survivors (P=.02) and positively correlated with observed/expected total fetal lung volume (P=.01). Transverse cerebellar diameter at >28weeks of gestation was also positively correlated with observed/expected total fetal lung volume (P=.04). We did not identify maturational delay, abnormal parenchymal signal or intracranial hemorrhage on fetal MRI. Enlarged extraaxial spaces in the third trimester was the most common abnormality on fetal MRI in congenital diaphragmatic hernia. Cerebellar dimensions on fetal MRI are associated with CDH severity. There was no major brain parenchymal injury on fetal MRI, even in the third trimester, in CDH survivors and non-survivors.

Proteomic profiling of tracheal fluid in an ovine model of congenital diaphragmatic hernia and fetal tracheal occlusion.

Congenital diaphragmatic hernia (CDH) occurs in ~1:2,000 pregnancies and is associated with substantial morbidity and mortality. Fetal tracheal occlusion (TO) is an emerging therapy that improves lung growth and reduces mortality, although substantial respiratory compromise persists in survivors. In this study, we used tracheal fluid in a fetal sheep model of CDH with TO for proteomic analysis with subsequent validation of findings in sheep lung tissue. We found that the proteomic profiles of CDH tracheal fluid was most similar to control lung and CDH/TO lung most similar to TO lung. Among 118 proteins altered in CDH, only 11 were reciprocally regulated in CDH/TO. The most significantly altered pathways and processes were cell proliferation, phosphatidylinositol 3-kinase/AKT/mammalian target of rapamycin signaling, inflammation, and microtubule dynamics. CDH suppressed and TO promoted cell proliferation and AKT-related signaling cascades. By Western blot analysis and immunohistochemistry, epithelial PCNA and phosphorylated AKT were decreased in CDH and increased in TO and CDH/TO lungs. The Wnt target Axin2 was decreased threefold in CDH lung compared with control without a significant increase in CDH/TO lung. Cilia-related pathways were among the most dysregulated with CDH lung having a nearly twofold increase in acetylated α-tubulin and a relative increase in the number of ciliated cells. While TO improves lung growth and patient survival in CDH, the procedure substantially alters many processes important in lung development and cell differentiation. Further elucidation of these changes will be critical to improving lung health in infants with CDH treated with TO.

Predicting Survival of Congenital Diaphragmatic Hernia on the First Day of Life.

This study aimed to determine perinatal risk factors for 30-day mortality of congenital diaphragmatic hernia (CDH) patients and develop a prognostic index to predict 30-day mortality of CDH patients. Identifying risk factors that can prognosticate outcome is critical to obtain the best management practices for patients. A retrospective study was performed for patients who were diagnosed with CDH from November 2000 to August 2016. A total of 10 prenatal risk factors and 14 postnatal risk factors were analyzed. All postnatal variables were measured within 24h after birth. A total of 95 CDH patients were enrolled in this study, including 61 males and 34 females with mean gestational age of 38.86 ± 1.51weeks. The overall 30-day survival rate was 63.2%. Multivariate analysis revealed that five factors (polyhydramnios, gestational age at diagnosis <25weeks, observed-to-expected lung-to-head ratio ≤45, best oxygenation index in 24h >11, and severity of tricuspid regurgitation ≥ mild) were independent predictors of 30-day mortality of CDH. Using these five factors, a perinatal prognostic index for 30-day mortality was developed. Four predictive models (poor, bad, good, and excellent) of the perinatal prognostic index were constructed, and external validation was performed. Awareness of risk factors is very important for predicting prognosis and managing patients. Five independent perinatal risk factors were identified in this study. A perinatal prognostic index was developed for 30-day mortality for patients with CDH. This index may be used to help manage CDH patients.

Working Memory Training Following Neonatal Critical Illness: A Randomized Controlled Trial.

To test the immediate and long-term effectiveness of Cogmed Working Memory Training following extracorporeal membrane oxygenation and/or congenital diaphragmatic hernia. A nationwide randomized controlled trial assessing neuropsychologic outcome immediately and 1 year post Cogmed Working Memory Training, conducted between October 2014 and June 2017. Researchers involved in the follow-up assessments were blinded to group allocation. Erasmus MC-Sophia Children's Hospital, Rotterdam, and Radboud University Medical Center, Nijmegen, the Netherlands. Eligible participants were neonatal extracorporeal membrane oxygenation and/or congenital diaphragmatic hernia survivors (8-12 yr) with an intelligence quotient greater than or equal to 80 and a z score less than or equal to -1.5 on at least one (working) memory test at first assessment. Cogmed Working Memory Training, comprising 25 45-minute training sessions for 5 consecutive weeks at home. Participants were randomized to Cogmed Working Memory Training (n = 19) or no intervention (n = 24) (two dropped out after T0). Verbal working memory (estimated coefficient = 0.87; p = 0.002) and visuospatial working memory (estimated coefficient=0.96, p = 0.003) significantly improved at T1 post Cogmed Working Memory Training but was similar between groups at T2 (verbal, p = 0.902; visuospatial, p = 0.416). Improvements were found at T2 on long-term visuospatial memory following Cogmed Working Memory Training (estimated coefficient = 0.95; p = 0.003). Greater improvements in this domain at T2 following Cogmed Working Memory Training were associated with better self-rated school functioning (r = 0.541; p = 0.031) and parent-rated attention (r = 0.672; p = 0.006). Working memory improvements after Cogmed Working Memory Training disappeared 1 year post training in neonatal extracorporeal membrane oxygenation and/or congenital diaphragmatic hernia survivors. Gains in visuospatial memory persisted 1 year post intervention. Cogmed Working Memory Training may be beneficial for survivors with visuospatial memory deficits.

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

Aims & Objectives: Children treated with neonatal extracorporeal membrane oxygenation (ECMO) and/or with congenital diaphragmatic hernia (CDH) are at risk of long-term neurodevelopmental deficits. We assessed the effects of Cogmed Working-Memory Training (CWMT) in school-age survivors of neonatal ECMO and/or CDH. Methods A nationwide, single-blind randomized controlled trial assessing white matter microstructure and neuropsychological outcome before(T0) and immediately(T1) after CWMT. CWMT, an adaptive computerized training program, comprised 25 sessions of 45 minutes for five consecutive weeks at home. Participants were neonatal ECMO and/or CDH survivors (8–12 years) with an IQ≥80 and a z-score≤-1.5 on at least one (working)memory test at T0. Diffusion Tensor Imaging was used to asses white matter microstructure. Results Of 34 participants, 27 children with useable data at both T0 and T1 were randomly assigned to CWMT(n=12) or no-training(n=15)(Figure 1). Training-induced changes were found in global white matter microstructure at T1 (estimated coefficient=.007, p=.015) and the left superior longitudinal fasciculus (estimated coefficient=.009, p=.033). Changes in the superior longitudinal fasciculus were significantly associated with the improvements in verbal working-memory in the CWMT group at T1, F(1,13)=7.13, p=.026, partial eta square=.44. No other associations between training-induced changes in white matter microstructure and neuropsychological improvement were found (p>.05). Conclusions Our findings demonstrate that neurobiological plasticity exists in survivors of neonatal ECMO and/or CDH and that, despite significant brain alterations found in these children, their neurodevelopmental outcome is changeable. Given the high risk of long-term neuropsychological deficits and subsequent school problems following neonatal critical illness, these results are of great significance.

Defining outcomes following congenital diaphragmatic hernia using standardised clinical assessment and management plan (SCAMP) methodology within the CDH EURO consortium.

Treatment modalities for neonates born with congenital diaphragmatic hernia (CDH) have greatly improved in recent times with a concomitant increase in survival. In 2008, CDH EURO consortium, a collaboration of a large volume of CDH centers in Western Europe, was established with a goal to standardize management and facilitate multicenter research. However, limited knowledge on long-term outcomes restricts the identification of optimal care pathways for CDH survivors in adolescence and adulthood. This review aimed to evaluate the current practice of long-term follow-up within the CDH EURO consortium centers, and to review the literature on long-term outcomes published from 2000 onward. Apart from having disease-specific morbidities, children with CDH are at risk for impaired neurodevelopmental problems and failure of educational attainments which may affect participation in society and the quality of life in later years. Thus, there is every reason to offer them long-term multidisciplinary follow-up programs. We discuss a proposed collaborative project using standardized clinical assessment and management plan (SCAMP) methodology to obtain uniform and standardized follow-up of CDH patients at an international level.

Health-related quality of life in children and adolescents with congenital diaphragmatic hernia: a cross-sectional study

BackgroundPatients with congenital diaphragmatic hernia (CDH) have a high residual morbidity rate. We compared self-reported health-related quality of life (HRQoL) between patients with CDH and healthy children.MethodsForty-five patients with CDH who were born from January 1, 1990, through February 15, 2015, were matched to healthy, age-matched control participants at a 1:2 ratio. The health records of the study participants were reviewed to determine comorbid conditions, and HRQoL was assessed by both the participants and their parents with the Pediatric Quality of Life Inventory (PedsQL). The HRQoL scores of the patients with CDH and the control participants were compared by using analysis of variance to adjust for age group and sex. Among patients with CDH, analysis of variance was used to compare HRQoL scores across groups defined according to their characteristics at initial hospitalization, postdischarge events, and comorbid conditions.ResultsCompared with control participants, patients with CDH had lower mean PedsQL scores, as reported by the parent and child, for the physical and psychosocial domains (P < 0.001). Risk factors associated with lower parent-reported HRQoL included bronchopulmonary dysplasia, longer initial hospitalization, severe cognitive impairment, and orthopedic symptoms; among patients with CDH, low HRQoL was associated with chronic respiratory issues.ConclusionPatients with CDH had lower HRQoL compared with healthy participants. Parent-reported HRQoL tended to be higher than child-reported HRQoL. Results were also inconsistent for the risk factors associated with HRQoL obtained by using child- and parent-reported scores. Therefore, when interpreting HRQoL in CDH survivors, a proxy report should not be considered a substitute for a child’s self-report.