Prenatally-counseled congenital diaphragmatic hernia parents exhibit positive well-being before and after surgical repair (original) (raw)
Related papers
Long-term functional impact of congenital diaphragmatic hernia repair on children
Journal of Pediatric Surgery, 2007
Congenital diaphragmatic hernia (CDH) is a malformation requiring neonatal surgical repair with in-hospital survival rates above 90%. We examined the long-term functional impact of CDH repair on a cross-sectional cohort of survivors.A cohort of 53 CDH families participated in this study. Functional impact was evaluated with parent report of the Functional Status IIR and the Child Health Ratings Inventories General Health Module. Parents also provided a clinical severity score, the child's medical history, and family demographic information. The primary outcome was the effect of medical morbidity on the Functional Status IIR total score.Congenital diaphragmatic hernia survivors had a median age of 8 years; 50% were in third grade or above. Sixty-six percent had major medical issues at hospital discharge, whereas 48% had current clinical problems. Functional Status IIR total score was strongly correlated with child's clinical severity (r = −0.65; P < .0001) and was lower among children with ongoing medical morbidity, denoting worse functioning (P = .01). Child Health Ratings Inventories General Health Module scores followed a similar pattern.A subset of long-term CDH survivors continues to have ongoing clinical problems a median of 8 years after surgery, translating to lower functional status. Affected children and their families may benefit from prospective identification and ongoing interventions.
Health and Quality of Life Outcomes, 2018
Background: Patients 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. Methods: Forty-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. Results: Compared 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 parentreported 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. Conclusion: Patients 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.
Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes
Journal of Pediatric Surgery, 2019
Purpose: Effective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with post-natal outcomes at two tertiary pediatric referral centers. Methods: Prenatal lung-head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24-32 weeks gestational age. Post-natal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted. Results: Eighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values >45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair. Conclusions: This audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review.
Journal for Specialists in Pediatric Nursing
Purpose: Last decennia remarkable advances have been made in decreasing the mortality rate of children with congenital diaphragmatic hernia (CDH), resulting in a relatively growing patient group with long-term complications and complex care needs. These consequences have a huge impact on the quality of life of both children and their families. To provide practical recommendations for improving the quality of care for this patient group, the present study sought to obtain insights into the experiences and needs of parents with a child with CDH. Design and Methods: A qualitative study was conducted on the experiences and needs of parents with a child with CDH living in the Netherlands. Data was obtained by means of a discourse analyses of 17 weblogs written by parents and three online focus groups with 8-12 parents per group (n = 29). The data was analysed thematically and structured by using the model of Lawoko (2007) on parental satisfaction with care. Results: Although parents were generally satisfied with the delivered care, they frequently encountered challenges in managing insecurities throughout the care process. Besides the unpredictable disease progress, insecurities were exacerbated by: (a) limited specialized knowledge of long-term consequences, (b) logistical problems, and (c) nontransparent communication. Providing security through, for instance, a clear care plan and by engaging parents in the decision-making process helped them feel more in control. Practice Implications: This study showed that parents' main challenge was to manage insecurities. Creating securities by providing a care plan and involving parents in the decision-making process helped parents to feel more in control. To improve quality of care for children with CDH, future measures should, therefore, focus on reducing insecurities by managing expectations, improving transparency and stimulating engagement.
Journal of Pediatric Surgery, 2014
Purpose: Pulmonary support (PS) on day-of-life-30 (DOL-30) has been shown to be the strongest predictor of subsequent morbidity and in-patient mortality in congenital diaphragmatic hernia (CDH). We hypothesized that PS on DOL-30 can also predict long-term outcomes in CDH survivors. Methods: We analyzed records of 201 CDH survivors followed by a single multidisciplinary clinic (1995)(1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010). Follow-up was 83 and 70% at 1 and 5 years respectively. PS was defined as: (1) invasive support (n = 44), (2) noninvasive support (n = 54), or (3) room air (n = 103). Logistic regression was used to estimate the adjusted association of PS on DOL-30 with outcomes at 1 and 5-years. Results: Use of PS on DOL-30 was significantly associated with pulmonary and developmental morbidities at 1 and 5-years. Even after adjusting for defect-size and presence of ventilation/perfusion mismatch, greater PS on DOL-30 was associated with a significantly increased odds of requiring supplemental oxygen and developmental referral at 1-year, and asthma and developmental referral at 5-years. Conclusion: CDH survivors continue to have significant long-term pulmonary and developmental morbidities. PS on DOL-30 is a strong independent predictor of morbidity at 1 and 5-years and may be used as a simple prognostic tool to identify high-risk infants.
Antenatal predictors of outcome in prenatally diagnosed congenital diaphragmatic hernia (CDH)
Journal of pediatric surgery, 2016
Pulmonary hypoplasia is the main cause of mortality in isolated congenital diaphragmatic hernia (CDH) and its prediction is paramount when counseling parents. We sought to identify antenatal parameters that predicted neonatal mortality in CDH. Search was conducted in MEDLINE, EMBASE, Cochrane Database of Systematic reviews, PubMed, Scopus, and Web of Science on the ability of lung-to-head ratio (LHR), observed-to-expected LHR (o/e LHR), total fetal lung volume (TFLV), o/e TFLV, percentage predicted lung volume (PPLV) and degree of liver herniation to predict neonatal morbidity and mortality in fetuses with CDH. Primary outcome was perinatal survival and secondary was the use of extracorporeal membrane oxygenation (ECMO). Until April 2016, 1067 articles were found, of which 22 were included in our meta-analysis. This showed that the odds of survival with LHR <1.0 and liver herniation on ultrasound were 0.14 (CI 0.10-0.27) and 0.21 (CI 0.13-0.35) respectively. Mean LHR, o/e LHR, ab...
Pediatric research, 2018
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 multidiscip...
Pediatric Surgery International, 2010
Purpose Fetal stabilization (FS) is a way to decrease stresses to the fetus during the perinatal period to control persistent pulmonary hypertension in neonates (PPHN). Although FS in congenital diaphragmatic hernia (CDH) patients has been reported, the effect of FS has not been evaluated sufficiently. The present study retrospectively evaluated influences of FS on the postnatal status of CDH patients. Methods Twenty-three cases of prenatally diagnosed CDH which were treated after birth in our institution from April 1998 to March 2010 were reviewed. From April 1998 to May 2007, FS was performed by administration of fentanyl and midazolam to the mother before subsequent cesarean section (FS group, n = 10). Beginning from June 2007, FS was discontinued (non-FS, n = 13). Results At the first postnatal estimation, flow pattern of the ductus arteriosus had no difference between two groups. The oxygenation index of the FS group was significantly higher than that in the non-FS group (p = 0.045). Only the non-FS group had correlations between estimated standardized lung volume (% lung volume) and alveolar-arterial oxygen tension difference (p = 0.022), and between % lung volume and the oxygenation index (p = 0.0037). Conclusion During the period immediately after birth, FS had no obvious therapeutic effect on PPHN, and had a negative impact on respiratory status.