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Classification of congenital diaphragmatic hernia (CDH) patients per the presence of a hernia sac (a), location of the liver (b), and accompanying syndromes (c)
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Objectives
We aim to determine factors that are associated with better outcomes of CDH patients.
Methods
A retrospective review was performed on all CDH patients admitted to our institution between 2003 and 2016. This study was performed at a single institution which has a fetal care center. Patients admitted with CDH with at least 1-year follow-u...
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... elimination. Results were compared to determine if the final model includes the same predictors with all methods. Exact logistic regres- sion was used when appropriate. Statistical associations were considered significant if the p value was < 0.05. Between 2003 and2016, 223 patients were admitted to our institution for CDH care and management (Fig. 1). Among these patients, 184 (82.5%) were formerly evaluated at our fetal center, while the rest were admitted postnatally. Over- all survival to discharge was 73.1% (163/223), while overall survival to date was 69.5% (155/223). Twenty-six (13.8%) patients had a hernia sac, 124 (59%) patients had liver herni- ated into the thorax, and 56 ...
Similar publications
Bochdalek hernias are the most common congenital diaphragmatic hernias, followed by Morgagni hernias. The failure of closure of the pleuroperitoneal membrane results in a posterolateral foramen, which can remain silent until adulthood. They remain a rare pathology with nearly a hundred cases published. Its clinical presentation is variable, making...
Citations
... Competition for space is established in the thoracic cavity, generating compression of the fetal lung tissue and egression of the essential intrapulmonary fluid that leads to the arrest of normal fetal lung development. The severity of CDH could be determined by non-invasive imaging techniques [2][3][4][5][6][7][8]. The rationale behind the importance of these measurements is that they can indirectly predict the degree of postnatal pulmonary hypertension and pulmonary hypoplasia. ...
... To increase the accuracy of determining the severity of the disease, pulmonary hypertension should be categorized throughout pregnancy. The current prognostic criteria are based on the association of prenatal and postnatal markers in retrospective patient data [8]. Since the growth and arborization of the lung are of primary significance during embryonic and fetal development, pulmonary vasculature is of particular importance. ...
Purpose
Impaired fetal lung vasculature determines the degree of pulmonary hypertension in the congenital diaphragmatic hernia (CDH). This study aims to demonstrate the morphometric measurements that differ in pulmonary vessels of fetuses with CDH.
Methods
Nitrofen-induced CDH Sprague–Dawley rat fetuses were scanned with microcomputed tomography. The analysis of the pulmonary vascular tree was performed with artificial intelligence.
Results
The number of segments in CDH was significantly lower than that in the control group on the left (U = 2.5, p = 0.004) and right (U = 0, p = 0.001) sides for order 1(O1), whereas there was a significant difference only on the right side for O2 and O3. The pooled element numbers in the control group obeyed Horton’s law (R² = 0.996 left and R² = 0.811 right lungs), while the CDH group broke it. Connectivity matrices showed that the average number of elements of O1 springing from elements of O1 on the left side and the number of elements of O1 springing from elements of O3 on the right side were significantly lower in CDH samples.
Conclusion
According to these findings, CDH not only reduced the amount of small order elements, but also destroyed the fractal structure of the pulmonary arterial trees.
... 20 This condition is implicated in higher rates of pulmonary hypoplasia and a more frequent need for patches in surgical repair. 21 Our data showed that intra-thoracic liver, regardless of laterality, had an impact on mortality. Other risk factors that deserve attention are the combination with other congenital anomalies and genetic syndromes. ...
Objective:
To describe post-natal risk factors associated with death in Newborns (NB) with Congenital Diaphragmatic Hernia (CDH) in a Brazilian reference center.
Methods:
In this retrospective cohort study, post-natal clinical factors of all NB diagnosed with CDH were reviewed in an 11-year period (2007‒2018). The primary outcome was death. Secondary outcomes included clinical features, prognostic indexes, type of mechanical ventilation, complications during hospitalization and surgical repair.
Results:
After applying the exclusion criteria, the authors analyzed 137 charts. Overall mortality was 59% (81/137), and the highest rates were observed for low-birth-weight NB (87%), syndromic phenotype (92%), and those with major malformations (100%). Prognostic indexes such as Apgar, SNAPPE-II and 24hOI (best oxygenation index in 24 hours) were all associated with poor evolution. In a multivariate analysis, only birth weight and 24hOI were statistically significant risk factors for mortality, with a reduction in mortality risk of 17.1% (OR = 0.829, 95% IC 0.72‒0.955, p = 0.009) for each additional 100g at birth and an increase by 26.5% (OR = 1.265, 95% IC 1.113‒1.436, p = 0.0003) for each unitary increase at the 24hOI.
Conclusion:
Prognostic indexes are an important tool for predicting outcomes and improving resource allocation. Post-natal risk factors may be more suitable for settings where antenatal diagnosis is not universal. Classical risk factors, such as prematurity, low birth weight, higher need for supportive care, and poorer prognostic indexes were associated with mortality in our CDH population.
... La forma más extrema de HDC es la ausencia (agenesia) diafragmática, y es la que peor pronóstico tiene (1). Cabe destacar que la presencia de un saco herniario, compuesto por peritoneo parietal y pleura, se ha asociado a un mejor pronóstico al contar con un mayor volumen pulmonar debido a una menor compresión y menor incidencia de herniación hepática (6). Posterior al nacimiento, estos pacientes presentan dificultad respiratoria en las primeras horas de vida. ...
La hernia diafragmática congénita (HDC) es una malformación infrecuente del desarrollo del diafragma por un defecto en el cierre de los canales pericardioperitoneales en el desarrollo embriológico. Se caracteriza por la herniación de los contenidos abdominales en el tórax, comprometiendo el desarrollo pulmonar. Tiene una prevalencia anual estimada de 1 a 4 casos en 10,000 nacidos vivos. La mayoría de los casos son esporádicos y multifactoriales, incluyendo factores ambientales y genéticos, o se presenta en asociación a otras malformaciones congénitas. Ambos alteran la diferenciación de las células mesenquimales en el proceso de formación del diafragma. La forma más frecuente es la hernia de Bochdalek (más del 95% de los casos), descrita como un defecto posterolateral que predomina en el lado izquierdo. Las consecuencias más relevantes son la hipoplasia pulmonar e hipertensión pulmonar persistente, responsables de la morbimortalidad asociadas a la HDC. Actualmente, el abordaje de esta patología se basa en el diagnóstico oportuno y la identificación de los fetos gravemente afectados para permitir planificar el parto o las nuevas terapias in útero, las cuales siguen en constantes estudios. Este artículo expone una revisión actualizada del diagnóstico y tratamiento pre y postnatal de la HCD.
... The incidence of sac in neonates with CDH was 22% in our series. Aydin et al. [2] found in their series that the presence of the hernia sac was associated with better outcomes. Bouchghoul et al. [14] found that the presence of the hernia sac was associated with better outcomes, especially survival at 6 months. ...
... However, this did not have any influence on the mortality as 75% of the children were discharged even with significant anomalies (malrotation, chylous ascites and generalised hypotonia). Aydin et al. [2] had similar results in their series. Six (10%) neonates operated for left CDH in our series had liver herniation and was associated with 100% mortality but it was not statistically significant. ...
Introduction:
Despite all the advances, the mortality rate of congenital diaphragmatic hernia (CDH) ranges from 30% to 60% for isolated CDH and as high as 89% when they are associated with additional structural or chromosomal anomalies. Hence, a study was conducted to evaluate the factors contributing to the mortality of neonates treated for CDH or the eventration of diaphragm.
Materials and methods:
A retrospective study was conducted in the department of paediatric surgery at a tertiary centre. The neonates admitted with a diagnosis of CDH or eventration requiring surgery, between March 2013 and March 2021, were included in the study.
Results:
A total of 123 neonates were included in the study. The variables, earlier median age at presentation (1 [1-23] vs. 3 [1-28]; P < 0.001; Mann-Whitney U-test), preterm birth (10/79 vs. 0/44; P = 0.01; Fischer's exact test), inborn (68/79 vs. 27/44; P = 0.002; Chi-square test), weight ≤2 kg (18/79 vs. 1/44; P = 0.003; Chi-square test), central cyanosis at presentation (21/79 vs. 1/44; P < 0.001; Chi-square test), antenatal detection (47/79 vs. 14/44; P = 0.003; Chi-square test) and earlier mean age at surgery (3.66 ± 1.47 vs. 7.66 ± 6.88; P < 0.001; Independent sample t-test) were associated with increased mortality. On multinominal logistic regression analysis, the factors preterm (odd's Ratio [OR] =4.735; P = 0.03), weight ≤2 kg (OR = 5.081; P = 0.02), central cyanosis at presentation (OR = 6.969; P = 0.008) and antenatal detection (OR = 7.471; P = 0.006) were found to be independently associated with increased mortality in CDH/eventration.
Conclusion:
The factors: prematurity, weight <2 kg, cyanosis at presentation and antenatal diagnosis were independently associated with increased mortality in neonates with CDH/eventration requiring surgery.
... The selection process is presented in the ow diagram ( (24.0%) in Asia, two studies (4.0%) in North America, and two studies (4.0%) in South America. Of these articles, ve studies included data on LHR < 1 [14,43,[47][48][49], six studies on o/e-LHR < 25% [22,[50][51][52][53][54], four studies on o/e-TFLV < 25% [44,45,55,56], ve studies on L/T ratio < 0.08 [26, 38, 51, 57, 58], 27 studies on liver-up [13,14,18,19,22,25,26,44,45,48,49,[59][60][61][62][63][64][65][66][67][68][69][70][71][72][73][74], seven studies on intrathoracic stomach herniation [19,33,44,47,54,61,75] (Table 1). Table 1 The results of the summary of sensitivity, speci city, DOR, PLR, NLR, and AUC of antenatal parameter. ...
Purpose
This study aimed to evaluate prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia (CDH).
Methods
A systematic literature search was performed to identify relevant observational studies that evaluated the ability of lung-to-head ratio (LHR), observed-to-expected LHR (o/e-LHR), observed-to-expected total fetal lung volume (o/e-TFLV), lung-to-thorax transverse area ratio (L/T ratio), intrathoracic herniation of the liver and the stomach, and side of diaphragmatic hernia, using a threshold for the prediction of mortality in fetuses with CDH. Study quality was assessed using the QUADAS-2 tool. Hierarchical summary receiver operating characteristic curves were constructed.
Results
A total of 50 articles were included in this meta-analysis. The QUADAS-2 tool identified a high risk of bias in more than one domain scored in all parameters. Among those parameters, the diagnostic odds ratio of mortality with o/e-LHR < 25%, o/e-TFLV < 25%, and L/T ratio < 0.08 were 11.98 (95% confidence interval (CI), 4.65–30.89), 11.14 (95%CI, 5.19–23.89), and 10.28 (95%CI, 3.38–31.31), respectively. The predictive values for mortality were similar between the presence of liver herniation and retrocardiac fetal stomach position.
Conclusions
This systematic review suggests that o/e-LHR, o/e-TFLV, and L/T ratio are equally good predictors of neonatal mortality in fetuses with isolated CDH.
... Factors such as prenatal diagnosis, lung to head ratio (LHR) and observed to expected LHR, need for and duration of extracorporeal membrane oxygenation (ECMO), prematurity, side of hernia, low birth weight, and size of the defect have all been found to correlate with outcomes [3][4][5][6][7][8][9][10]. Additionally, liver location appears to correlate significantly with degree of fetal lung development and function [11][12][13]. ...
Background:
Congenital diaphragmatic hernia (CDH) accounts for 8% of all major congenital anomalies. Neonates who are small for gestational age (SGA) generally have a poorer prognosis. We sought to identify risk factors and variables associated with outcomes in neonates with CDH who are SGA in comparison to neonates who are appropriate for gestational age (AGA).
Methods:
We used the multicenter Diaphragmatic Hernia Research & Exploration Advancing Molecular Science (DHREAMS) study to include neonates enrolled from 2005 to 2019. Chi-squared or Fisher's exact tests were used to compare categorical variables and t tests or Wilcoxon rank sum for continuous variables. Cox model analyzed time to event outcomes and logistic regression analyzed binary outcomes.
Results:
589 neonates were examined. Ninety were SGA (15.3%). SGA patients were more likely to be female (p = 0.003), have a left sided CDH (p = 0.05), have additional congenital anomalies and be diagnosed with a genetic syndrome (p < 0.001). On initial single-variable analysis, SGA correlated with higher frequency of death prior to discharge (p < 0.001) and supplemental oxygen requirement at 28 days (p = 0.005). Twice as many SGA patients died before repair (12.2% vs 6.4%, p = 0.04). Using unadjusted Cox model, the risk of death prior to discharge among SGA patients was 1.57 times the risk for AGA patients (p = 0.029). There was no correlation between SGA and need for ECMO, pulmonary hypertensive medication at discharge or oxygen at discharge. After adjusting for confounding variables, SGA no longer correlated with mortality prior to discharge or incidence of unrepaired defects but remained significant for oxygen requirement at 28 days (p = 0.03).
Conclusion:
Infants with CDH who are SGA have worse survival and poorer lung function than AGA infants. However, the outcome of SGA neonates is impacted by other factors including gestational age, genetic syndromes, and particularly congenital anomalies that contribute heavily to their poorer prognosis.
... 1,7 Despite the pulmonary condition and associated anomalies, characteristics of the hernia such as liver herniation, presence of hernia sac, and size and diameter of the defect seem to be related to the post-operative outcome. 8,9 The aim of this study was to evaluate the effect of surgical and morphologic factors such as the presence of hernia sac and defect size on the Original Article Abstract Introduction: Congenital diaphragmatic hernia (CDH) is a congenital malformation with challenging management. The aim of this study was to evaluate the effect of the presence of hernia sac and defect size on the prognosis of CDH in Tabriz children's hospital. ...
... The reported incidence of hernia sac is approximately 20% in different studies ranging from 13% to 26% of CDH patients. 9,13,15 In our study, in the presence of hernia sac, there was not any observed death which was statistically higher than patients without sac (P = 0.033). Several studies corroborated hernia sac as a positive prognostic factor in the outcome of patients with CDH. ...
... They reported that the presence of a hernia sac in CDH was associated with a better outcome, especially survival at 6 months. 9,14,15 However, Levesque studied 71 patients with CDH and showed that mortality did not differ in presence or absence of hernia sac; nevertheless, the presence of a hernia was associated with decreased inhaled nitric oxide, vasoactive medication, and ventilator use. 8 The exact impact of hernia sac in CDH is unknown. ...
Background: The aim of this study was to evaluate the effect of the presence of hernia sac and defect size on the prognosis of CDH in our pediatric hospital.
Methods: In this prospective study all the infants with the diagnosis of CDH that were admitted in the Tabriz Pediatric Hospital since 2016- 2019 were enrolled. Laparatomy was performed when the patients were stabilized. Presence or absence of hernia sac was evaluated and the largest diameter on diaphragmatic defect was measured before the repair. Postoperative outcome including instability, duration of mechanical ventilation, mortality rate or hospital discharge were obtained and analyzed.
Results: Fifteen cases were diagnosed during the first 24 hours of life and Sixteen cases experienced hypoxia in the first hour after birth. Left sided CDH was detected in 26 patients (86.66%), 3 patients (10%) had right sided defect and in one case diaphragmatic defect was bilateral. Hernia sac was present in the 8 cases. Mortality rate was 30% (9 cases) in postoperative period. Mean diameter of defect size in survived group was lower than nonsurvived group (4.12±0.61cm vs. 4.61±0.48; P=0.09). Postoperative death, occurred in 9 of patients without hernia sac but none of cases with hernia sac expired (P= 0.035).
Conlusion: In our study the presence of hernia sac was related to lower postoperative mortality rate. Defect size in survived group was also lower. We suggested presence of hernia sac and lower diameter of defect size acting as good prognostic factors in outcome of patients with CDH.
... Despite the fact that antenatal diagnosis via ultrasonography (USG), assessment of prognostic variables, prenatal counseling, and therapeutic modalities to the fetus can be used to predict disease outcomes, CDH death and morbidity rates remain high. For the management of the disease and its complications, as well as the management of concomitant malformations, CDH newborns must be hospitalized for an extended amount of time, with long-term follow-up [10,11]. ...
... Advancements in surgical interventions, neonatal care, and treatment strategies have improved CDH management in the last decades. Although these advancements improve patient survival, certain factors, such as the presence of liver herniation and associated defects, reduce the chances of survival [10]. In a previous study by our team, we showed that as more liver mass herniated into the thoracic cavity, ECMO need was increased, and survival of the patients was decreased; moreover, this herniation decreased the prenatal lung measurements as well [10]. ...
... Although these advancements improve patient survival, certain factors, such as the presence of liver herniation and associated defects, reduce the chances of survival [10]. In a previous study by our team, we showed that as more liver mass herniated into the thoracic cavity, ECMO need was increased, and survival of the patients was decreased; moreover, this herniation decreased the prenatal lung measurements as well [10]. Another study investigated the liver position and lung-tohead ratio (LHR) to predict the requirement for ECMO cannulation and survival in isolated left CDH; its results showed that fetuses with liver up and liver down had 45 percent and 93 percent overall survival rates, respectively [17]. ...
Current literature for congenital diaphragmatic hernia (CDH) focuses on the comparison of the overall mortality in CDH patients. Only a few studies concentrate on analyzing the unstable patients who could not achieve surgical repair, as well as those who could but did not survive after. Hence, this study aimed to analyze the effects of various parameters on the timing of death. A retrospective analysis was performed by using the data of all CDH patients from 2003 to 2016 at a single tertiary center. Patients who were diagnosed with left-sided CDH and expired were included in the study regardless of the cause. Of the 66 expired patients, 5 were excluded due to right-sided CDH. The study population constituted a total of 61 patients, of which 31 patients expired prior to CDH repair, and 30 patients expired at different times after CDH repair. Multinomial regression analysis identified that the ECMO need (B = 20.257, p = 0.000, OR: 62.756, 95% CI 10.600-371.384) and O/E LHR (B = 20.376, p = 0.000, OR: 70.663, 95% CI 48.716-102.415) values were the independent predictors that influenced mortality in this cohort. Prenatal pulmonary measurements are the major predictors determining the severity of the disease in patients with CDH.
... Congenital diaphragmatic hernia (CDH), which occurs in one in 2000 infants, is still a challenging phenomenon. [1][2][3][4][5][6] It arrests the development of the lung and is characterized by pulmonary hypoplasia and pulmonary hypertension of the newborn. 7 To improve fetal lung growth and reverse the adverse effects of the disease, one of the emerging surgical methods is fetal tracheal occlusion (TO). ...
Background
There is a certain need for reversible, cheap, and reproducible animal models for understanding the impact of tracheal occlusion (TO) in the congenital diaphragmatic hernia and pathophysiology. We aimed to present an easy, reversible, and minimally invasive murine TO model with optimized time points for introduction and removal of TO.
Methods
Time-mated C57BL/6 mice underwent laparotomy at embryonic day 16.5 (E16.5) with transuterine TO performed on two fetuses in each uterine horn. In the TO group, the fetuses were harvested at E18.5 without suture removal; the suture was released at E17.5 in the TO-R group, and all fetuses were harvested at E18.5. The lungs of the fetuses were compared by morphometric and histologic analysis.
Results
Successful TO was confirmed in 34 of 37 fetuses. Twenty-nine of them survived to E18.5 (90.6%), six of the fetuses had a spontaneous vaginal delivery. Fetal weights were comparable, but there was significant difference in lung weights and lung-to-body weight ratios (0.020 ± 0.006 [control] versus 0.026 ± 0.002 [TO] versus 0.023 ± 0.005 [TO-R]; P = 0.013). DNA/protein and DNA/lung weight ratios were elevated, whereas protein/lung weight ratio was lower in TO compared with the control group.
Conclusions
Reversal of fetal transuterine TO at E17.5, which was put at E16.5 in mice, is feasible with comparable outcomes to other current animal models with certain advantages and potential to translate the studies to the human.
... Interestingly, we observed that CDH cases with hernia sac were more often associated with liver herniation though the difference(s) were not statistically significant vs infants with no hernia sac. Intrathoracic liver herniation is considered to be a strong prognostic marker in foetal CDH and associated with poor outcome(s) [7,10,21]. In our current study, we found that more than half of the CDH cases we analysed with a hernia sac had reportedly liver herniation and yet a very high survival rate of almost 93%. ...
... Requirement for ECMO was reported in three studies we analysed [12,20,21] with one study clearly showing statistically significant difference in outcome [12]. The combined data from all three studies established that ECMO was less often required in infants with a hernia sac. ...
... The combined data from all three studies established that ECMO was less often required in infants with a hernia sac. Although we were unable from the current publications to fully analyse data on hospital LOS and number of days ventilated, the emerging evidence suggests that the presence of CDH hernia sac is associated with shorter LOS [12,20,21], and also fewer hospital days requiring ventilation [12,16]. These findings are most likely reflective of more favourable physiology with better lung growth preserved in infants (see earlier) also with reduced rates of pulmonary hypertension. ...
Early reports have suggested survival benefits associated with a hernia sac in congenital diaphragmatic hernia (CDH). However, these studies have included only small subsets of patients. This systematic review aimed to evaluate differences in outcomes of CDH newborns with and without a hernia sac. PubMed and Embase databases were searched using relevant key terms. Papers were independently reviewed by two authors with final selection approved by the senior author. Original search retrieved 537 papers; the final review included 8 studies (n = 837 patients). There were 168 CDH patients (20%) with a hernia sac with an overall survival of 93% vs 73% for CDH newborns without a sac (p < 0.001). Twenty-three percent of patients with a CDH sac required diaphragm patch repair vs 44% patients without a sac (p < 0.001). Pulmonary hypertension was manifested in 44% of CDH babies with a hernia sac vs 64% without a sac (p < 0.001). Three studies compared ECMO requirement: 15% with a hernia sac and 34% without sac, p < 0.001.Conclusion: This study shows significant survival benefits in newborns associated with presence of a CDH sac. This may be likely related to these infants having more favourable physiology with less severe pulmonary hypertension and/or smaller anatomical defects requiring primary closure only.
What is Known:
• Early reports have suggested survival benefits associated with a hernia sac in CDH.
• Previous studies have included only a small number of patients.
What is New:
• A systematic review of published studies clearly shows that CDH newborns with a hernia sac have better overall survival outcomes and less severe pulmonary hypertension.
• ECMO utilization and patch repair were also less often required in newborns with a hernia sac.
