Congenital Diaphragmatic Hernia Study Group Research Articles

Intraoperative surgical complications of open surgery for congenital diaphragmatic hernia: a multicenter, observational study in Japan.

This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients were obtained from the database of the Japanese CDH Study Group. Among 1,111 neonates with left or right CDH, 852 underwent open surgery (laparotomy or thoracotomy). Of these 852 neonates, 51 had the following surgical complications: organ injury (n = 48; 6% of open surgeries); circulatory failure caused by changes in the organ location (n = 2); and skin burns (n = 1). Injured organs included the spleen (n = 30; 62% of organ injuries), liver (n = 7), lungs (n = 4), intestine (n = 4), adrenal gland (n = 2), and thoracic wall (n = 2). Fourteen of the patients who experienced organ injury required a blood transfusion (2% of open surgeries). The adjusted odds ratio of splenic injury for patients with non-direct closure of the diaphragm was 2.2 (95% confidence interval, 1.1-4.9). Of the patients who underwent open surgery for CDH, 2% experienced organ injury that required a blood transfusion. Non-direct closure of the diaphragmatic defect was a risk factor for splenic injury.

Fetal biophysical predictors of pulmonary hypertension severity, management, and treatment in infants born with congenital diaphragmatic hernia

BackgroundPulmonary hypertension (PHTN) causes significant morbidity and mortality in patients with congenital diaphragmatic hernia (CDH). Currently, there is no routinely obtained prenatal prognostic marker to reliably predict postnatal CDH-associated PHTN severity. MethodsThe CDH Study Group (CDHSG) registry was queried for infants born from 2015 to 2021 with a graded (1–4) PHTN diagnosis. Fetal observed-to-expected lung volume to head circumference ratio (o/e LHR), percent predicted lung volume (PPLV), and total lung volume (TLV) were classified by severity. ResultsOf 4056 patients, 1047 and 785 infants had prenatal ultrasound or magnetic resonance imaging, respectively. Both moderate and severe o/e LHR were associated with increased odds of postnatal development of moderate (OR 2.913) and severe PHTN (OR 4.924). ConclusionsIn infants with CDH, prenatal predictor severity was associated with higher severity of PHTN and increased ECLS usage. Overall, patients with worse prenatal prognostic indicators were less likely to receive pulmonary vasodilator treatment.

Hydrops and congenital diaphragmatic hernia: reported incidence and postnatal outcomes. Analysis of the congenital diaphragmatic hernia study group registry

ObjectiveCongenital Diaphragmatic Hernia (CDH) associated with hydrops is rare. The aim of this study was to describe the incidence of this combination of anomalies and the postnatal outcomes from a large database for CDH.Study designData from the multicenter, multinational database on infants with prenatally diagnosed CDH (CDHSG Registry) born from 2015 to 2021 were analyzed.ResultsA total of 3985 patients were entered in the registry during the study period, 3156 were prenatally diagnosed and 88 were reported to have associated fluid in at least 1 compartment, representing 2.8% of all prenatally diagnosed CDH cases in the registry. The overall survival to discharge for CDH patients with hydrops was 43%. The hydropic CDH group had lower birth weight and gestational age at birth, and increased incidence of right-sided CDH (55%), and rate of non-repair (45%). However, the survival rate for hydropic infants with CDH undergoing surgical repair was 80%. Other associated anomalies were more common in hydropic CDH (50% vs 37%, p = 0.001).ConclusionHydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right-sided CDH. Survival rates are low, with higher rates of non-repair. However, decision-making regarding goals of care and an aggressive surgical approach in selected cases may result in survival rates comparable to non-hydropic cases.

Structured reporting in fetal magnetic resonance imaging with congenital diaphragmatic hernia.

We aim to provide a template structured report of fetal Magnetic Resonance Imaging in congenital diaphragmatic hernia (CDH) that was locally validated by the CDH study group in Mannheim. A selection of 50 fetal MRIs of patients with an isolated diaphragmatic hernia and associated radiology reports from five different senior radiologists from a single center resulted in a primary structured report, which was put into practice by using dedicated software. A questionnaire survey of the interdisciplinary CDH study group Mannheim was used to adapt the report to the clinical requirements. There was a huge variability in how deep the free text reports go into detail. The side of the hernia was named in 94% of cases. In 58%, both the lung volume and the total lung volume were reported. A comparison with the expected lung volume was reported in 66% of cases. Additional findings, such as herniated organs, were reported in 96% of cases. Overall satisfaction with the newly established structured report was high within the CDH study group with a mean of 4.7. The use of the structured report of this study can optimize the interdisciplinary dialog, the standardization of report content, increase report completeness and improve quality.

Hernia diafragmática congénita: relación entre el tamaño del defecto y evolución. Experiencia en un centro de referencia

IntroductionCongenital diaphragmatic hernia (CDH) remains a therapeutic challenge. The surgical classification recommended by the Congenital Diaphragmatic Hernia study group (CDHSG), based on the size of the defect, is used for staging in reference centres. Larger defects are associated with poorer outcomes. Our aim was to describe and compare the morbidity at hospital discharge of newborns who underwent surgical correction of CDH at the Juan P. Garrahan, according to the surgical staging of the defect proposed by the CDHSG. Material and methodsThe study included patients with CDH admitted to the Juan P. Garrahan Hospital between 2012 and 2020, and we analysed the distribution, morbidity and mortality associated with the size of the defect. We carried out a descriptive analysis, calculating measures of central tendency and dispersion, and bivariate and multivariate analyses. ResultsA total of 230 patients with CDH were admitted and 158 underwent surgery. We found that defect sizes C and D sizes were associated with an increased risk of chronic pulmonary disease (CPD) (OR, 5.3; 95% CI, 2.2-13.4; P<.0000), need of extracorporeal membrane oxygenation (OR 3.9; 95% CI, 1.3-12.8; P<.005) and chylothorax (OR, 2.1; 95% CI, 0.8- 6.4; P<.10]. The multivariate analysis revealed that a large defect size (C-D) was independently and significantly associated with CPD (OR 4.19; 95% CI, 1.76-9.95). ConclusionStaging the defect according to de CDHSG classification during surgery allows the application of uniform management criteria and the prediction of patient outcomes and complications during the hospital stay.

Assessment of pulmonary artery size at birth as a prognostic factor in congenital diaphragmatic hernia: results of a multicenter study in Japan.

To assess the reliability of pulmonary artery (PA) parameters as a prognostic marker in neonates with isolated left-sided congenital diaphragmatic hernia (IL-CDH). A retrospective cohort study conducted by the Japanese CDH Study Group (JCDHSG). 323 IL-CDH patients registered with the JCDHSG were included. 272 patients survived to 90 days of age. Right PA (RPA) and left PA (LPA) diameters and pulmonary artery index (PAIndex) at birth were significantly larger in survivors. The cutoff values of RPA and LPA diameters and PAIndex for survival up to 90 days were 3.2 mm, 2.8 mm and 83.7, respectively, and logistic regression analysis showed that these were significantly related to survival. Multiple logistic regression analysis showed that both the PA parameters and liver herniation were significantly related to survival. The three PA parameters at birth can predict clinical outcomes and are considered as independent risk factors of liver herniation.

Pulmonary outcomes of congenital diaphragmatic hernia patients based on defect size (CDH Study Group Stage).

Congenital diaphragmatic hernia (CDH) is associated with significant pulmonary morbidity. Previous investigation has shown that postnatal inpatient morbidity is linked to diaphragmatic defect size. The objective of this study was to evaluate long-term pulmonary outcomes by CDH study group defect size. A retrospective analysis was conducted for CDH patients (n=133) managed in a neonatal intensive care unit (NICU) at a single children's hospital within an adult hospital system and subsequently followed up at a comprehensive multidisciplinary CDH clinic (n=102) from January 2012 to April 2022. CDH patients were stratified according to Congenital Diaphragmatic Hernia Study Group (CDHSG) Stage, and then categorized as low-risk (LR), defect size A and B, or high-risk (HR), defect size C and D. Inpatient data, including the presence of pulmonary hypertension, extracorporeal life support (ECLS) utilization, and mechanical ventilation days, were collected. Post-discharge data including the prevalence of asthma, pulmonary hypertension, emergency department visits, the total number of hospitalizations, and average rehospitalization days were collected. Frequentist analysis was used. The outcomes for 133 NICU patients were analyzed (HR: n=54, LR: n=79). During NICU stay, the prevalence of pulmonary hypertension [HR: 16/54 (30%) vs. LR: 9/79 (12%), P=0.009], ECLS utilization [HR: 19/54 (35%) vs. LR: 4/79 (5%), P<0.001], and the average number of mechanical ventilation days [HR: 17 days (IQR: 12-27) vs. LR: 5 days (IQR: 2-9), P<0.001] were significantly higher in the HR CDH group. Post NICU discharge, the prevalence of asthma [HR: 20/54 (37%), vs. LR: 17/79 (22%), P=0.050)] and the total days of rehospitalization [HR: 9 (IQR: 2-27) vs. LR: 4 (IQR: 1-8), P=0.035] were significantly higher in HR group. Of the patients seen in the comprehensive multidisciplinary CDH clinic, obstructive lung disease measured by impulse oscillometry was increased in the HR CDH population compared to the reference group [median R5Hz was 12.95 kPa/(L/s) in CDH vs. 9.8 kPa/(L/s) (P=0.010)]. HR CDHSG Stage is associated with worse inpatient and long-term pulmonary outcomes.

A novel congenital diaphragmatic hernia prediction model for Chinese subjects: A multicenter cohort investigation

Purpose of the studyBrindle et al. (2014) and the Congenital Diaphragmatic Hernia Study Group constructed a simplified clinical prediction rule (Brindle score) to stratify infants with congenital diaphragmatic hernia based on disease severity. We aimed to develop a predictive model applicable to Chinese patients with congenital diaphragmatic hernia and externally validate whether the Brindle score is applicable to the Chinese population. Patients and the methodsMultiple imputations supplemented the missing data. A least absolute shrinkage and selection operator regression was used to screen the factors influencing adverse outcomes. Internal validation was performed by bootstrap resampling. The C-index, area under the receiver operating characteristic curve, and the Hosmer-Lemeshow test evaluated the predictive power. ResultsA nomogram named “CCDH score” (Chinese Congenital Diaphragmatic Hernia score), including pulmonary hypertension, low 5-min Apgar score (<7), chromosomal anomaly, major cardiac anomalies (MCAs), observed-to-expected lung-to-head ratio, and the percentage of liver herniation, was constructed. The CCDH score revealed good calibration and discriminative abilities, with a C-index of 0.941. In the training and external validation cohorts, the area under the receiver operating characteristic curve of the Brindle score were 0.820 and 0.881, respectively. The Brindle score has fair predictive power in the Chinese population, but the newly established CCDH score seems more suitable for Chinese patients with congenital diaphragmatic hernia. ConclusionThe CCDH score is the first predictive model constructed based on the characteristics of the Chinese population and can accurately predict the survival outcomes of patients with congenital diaphragmatic hernia.

Sex-Specific Differences in Congenital Diaphragmatic Hernia Mortality

To compare disease severity and mortality differences between female and male patients with congenital diaphragmatic hernia (CDH). We queried the CDH Study Group (CDHSG) database for CDH neonates managed between 2007 and 2018. Female and males were compared in statistical analyses using t tests, χ² tests, and Cox regression, as appropriate (P≤.05). There were 7288 CDH patients, of which 3048 (41.8%) were female. Females weighed less on average at birth than males (2.84kg vs 2.97kg, P<.001) despite comparable gestational age. Females had similar rates of extracorporeal life support (ECLS) utilization (27.8% vs 27.3%, P=.65). Although both cohorts had equivalent defect size and rates of patch repair, female patients had increased rates of intrathoracic liver herniation (49.2% vs 45.9%, P=.01) and pulmonary hypertension (PH) (86.6% vs 81.1%, P<.001). Females had lower survival rates at 30-days (77.3% vs 80.1%, P=.003) and overall lower survival to discharge (70.2% vs 74.2%, P<.001). Subgroup analysis revealed that increased mortality was significant among those who underwent repair but were never supported on ECLS (P=.005). On Cox regression analysis, female sex was independently associated with mortality (adjusted hazard ratio 1.32, P=.02). After controlling for the established prenatal and postnatal predictors of mortality, female sex remains independently associated with a higher risk of mortality in CDH. Further study into the underlying causes for sex-specific disparities in CDH outcomes is warranted.

Association Between Early Prostacyclin Therapy and Extracorporeal Life Support Use in Patients With Congenital Diaphragmatic Hernia

Prostacyclin (PGI2) is a therapeutic option to treat congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension in neonates. Its use may decrease the need for extracorporeal life support (ECLS). To evaluate the association of early PGI2 therapy with ECLS use and outcomes among patients with CDH. This was a cohort study from the CDH Study Group (CDHSG) registry of patients born from January 2007 to December 2019. Patients were from 88 different tertiary pediatric referral centers worldwide that contributed data to the CDHSG. Patients were included in the study if they were admitted within the first week of life. Propensity score matching was performed using estimated gestational age, birth weight, transfer status, 1-minute and 5-minute Apgar scores, highest and lowest partial pressure of arterial carbon dioxide in the first 24 hours of life, and degree of pulmonary hypertension as covariates to generate a matched cohort of exposed and unexposed patients. Data were analyzed from January 2021 to December 2022. Early PGI2 therapy was defined as initiation of PGI2 within the first week of life. Patients who received ECLS were included in the early PGI2 group if PGI2 was started prior to ECLS. The primary outcome of the study was the proportion of patients receiving ECLS in the exposed and unexposed groups. Of 6227 patients who met inclusion criteria (mean [SD] gestational age, 37.4 [2.36] weeks; 2618 [42%] female), 206 (3.3%) received early PGI2 therapy. ECLS was used in 46 of 206 patients who received PGI2 (22.2%) and 1682 of 6021 who did not (27.9%). After propensity score matching, there were 147 patients in the treatment and control groups. Thirty-four patients who received PGI2 (23.3%) and 63 who did not (42.9%) received ECLS. Those who received PGI2 were less likely to receive ECLS (adjusted odds ratio, 0.39; 95% CI, 0.22-0.68) and had shorter mean (SD) duration of ECLS (8.6 [3.73] days vs 12.6 [6.61] days; P < .001), although there was no significant difference in in-hospital mortality. In this study, there was decreased use of ECLS and decreased ECLS duration among patients with CDH who started PGI2 therapy during the first week of life. These results identify a potential advantage of early prostacyclin therapy in this population.

Intraoperative Transpyloric Tube Insertion for Congenital Diaphragmatic Hernia: Analysis of Japanese Study Group Data

Congenital diaphragmatic hernia (CDH) is a severe congenital disease. Some CDH infants suffer from gastro-esophageal reflux disease (GERD), even after surgical correction of gastric position. A transpyloric tube (TPT) is inserted into CDH patients under direct observation intraoperatively in some hospitals in Japan to establish early enteral feeding. This strategy avoids gastric expansion to maintain a better respiratory condition. However, it is unclear whether the strategy has a secure effect for patient prognosis. This study aimed to evaluate the effectiveness of intraoperative TPT insertion on enteral feeding and postoperative weight gain. The Japanese CDH Study Group database was used to identify infants with CDH born between 2011 and 2016, who were then divided into two groups: the TPT group and gastric tube (GT) group. In the TPT group, infants underwent intraoperative TPT insertion; postoperative insertion/extraction of TPT was irrelevant to the analysis. Weight growth velocity (WGV) was calculated using the exponential model. Subgroup analysis was performed using Kitano's gastric position classification. We analyzed 204 infants, of which 99 and 105 were in the TPT and GT groups, respectively. Enteral nutrition (EN) in the TPT and GT groups was 52±39 and 44±41kcal/kg/day (p=0.17) at age 14 days (EN14), respectively, and 83±40 and 78±45kcal/kg/day (p=0.46) at age 21 days (EN21), respectively. WGV30 (WGV from day 0 to day 30) in the TPT and GT groups was 2.3±3.0 and 2.8±3.8g/kg/day (p=0.30), respectively, and WGV60 (WGV from day 0 to day 60) was 5.1±2.3 and 6.0±2.5g/kg/day (p=0.03), respectively. In infants with Kitano's Grade 2+3, EN14 in the TPT and GT groups was 38±35 and 29±35kcal/kg/day (p=0.24), respectively, EN21 was 73±40 and 58±45kcal/kg/day (p=0.13), respectively, WGV30 was 2.3±3.2 and 2.0±4.3g/kg/day (p=0.76), respectively, and WGV60 was 4.6±2.3 and 5.2±2.3g/kg/day (p=0.30), respectively. Intraoperative TPT insertion did not improve nutritional intake and WGV30. WGV60 in TPT was less than that in GT. In Grade 2+3 subgroup analysis, TPT also had no advantage. We could not recommend routine TPT insertion at surgery. III.

Risk-stratified Results among Congenital Diaphragmatic Hernia Patients in Two Large Extracorporeal Membrane Oxygenation Centers in South America

IntroductionMortality related to CDH is high, but with great variability among centers. There are few studies on patients with this condition born in South America which show poor outcomes. The goal of this study is to present the outcome of CDH in several high-volume quaternary centers in South America, ascertain the factors associated with lower mortality in our population, and compare our outcomes to those of the CDH Study Group (CDHSG). MethodsThe data from two South American centers were retrospectively analyzed and compared with contemporary data from other CDHSG participating centers. Patient demographic and clinical characteristics were also evaluated and compared. ResultsBetween 2013 and 2018, the two South American centers saw 335 patients with CDH with an overall survival rate of 73.1%. Survival for the high, intermediate, and low-risk groups as determined by the Brindle score was 50%, 70%, and 87%, respectively. In our cohort the strongest predictors of mortality were ECMO use and early PaCO2. There were no significant differences in mortality between the two South American centers and the other CDHSG centers when adjusted by risk score, however, the South American centers had higher use of ECMO in the intermediate-risk group. DiscussionQuaternary South American centers had similar outcomes to CDHSG centers worldwide. The availability and coordination of centralized dedicated care allow more efficient use of scarce technical and professional resources in patients with CDH. Level of evidenceIII.

Early nitric oxide is not associated with improved outcomes in congenital diaphragmatic hernia.

Inhaled nitric oxide (iNO) is widely used for the management of infants with congenital diaphragmatic hernia (CDH); however, evidence of benefit is limited. This is a multicenter cohort study using data from the Congenital Diaphragmatic Hernia Study Group between 2015 and 2020. The impact of early iNO use in the first 3 days of life prior to ECLS use on mortality or ECLS use was explored using multivariate logistic regression models and subgroup analyses. Of the 1777 infants, 863 (48.6%) infants received early iNO treatment. Infants receiving iNO had lower birth weight, larger defect size, more severe pulmonary hypertension, and abnormal ventricular size and function. After controlling for these factors, early iNO use was associated with increased mortality (aOR 2.06, 95% CI 1.05-4.03, P = 0.03) and increased ECLS use (aOR 3.44, 95% CI 2.11-5.60, P < 0.001). Subgroup analyses after stratification by echocardiographic characteristics and defect size revealed no subgroup with a reduction in mortality or ECLS use. Use of iNO in the first 3 days of life prior to ECLS was not associated with a reduction in mortality or ECLS use in either the regression models or the subgroup analyses. The widespread use of iNO in this vulnerable population requires reconsideration. Evidence to support widespread use of iNO for infants with congenital diaphragmatic hernia is limited. The use of iNO in the first 3 days of life was associated with significantly increased mortality and ECLS use. Stratification by echocardiographic characteristics and defect size did not reveal a subgroup that benefited from iNO. Even the subset of patients with R-to-L shunts at both ductal and atrial levels, a surrogate for elevated pulmonary arterial pressures in the absence of significantly decreased LV compliance, did not benefit from early iNO use. Early iNO therapy was of no benefit in the management of acute pulmonary hypertension in infants with congenital diaphragmatic hernia, supporting reconsideration of its use in this population.

Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients. Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality. Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3kg vs. 2.9kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χ2 p < 0.001). CDH + IAAA were less likely to undergo diaphragm repair (72 vs. 87%, p < 0.001), and overall mortality was higher for CDH + IAAA infants (58 vs. 24%, p < 0.001). When controlling for defect size, birth weight, and Apgar, IAAA were significantly associated with mortality (OR 3.3, 95% CI 2.2-5.0; p < 0.01) but not associated with ECLS (OR 0.98, 95% CI 0.65-1.50; p = 0.90). Only17% (n = 28) of CDH + IAAA patients underwent aortic intervention. IAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, givenfew CDH + IAAA patients undergo aortic intervention.

Association of timing of congenital diaphragmatic hernia repair with survival and morbidity for patients not requiring extra-corporeal life support.

While physiologic stabilization followed by repair has become the accepted paradigm for management of congenital diaphragmatic hernia (CDH), few studies have examined the effect of incremental changes in operative timing on patient outcomes. We hypothesized that later repair would be associated with higher morbidity and mortality. Data were queried from the CDH Study Group (CDHSG) from 2007-2020. Patients with chromosomal or cardiac abnormalities and those who were never repaired or required pre-repair extra-corporeal life support (ECLS) were excluded. Time to repair was analyzed both as a continuous variable and by splitting the cohort into top/bottom percentiles. The primary outcome of interest was in-hospital mortality. Secondary outcomes included need for and duration of post-repair ventilatory and nutritional support. A total of 4,104 CDH infants were included. Median time to repair was 4 days (IQR 2-6). On multivariable analysis, high-risk (CDHSG stage C/D) defects and lower birthweight predicted later repair. Overall, in-hospital mortality was 6%. On univariate analysis, there was no difference in the number of days to repair between survivors and non-survivors. On risk-adjusted analysis, single-day changes in day of repair were not associated with increased mortality. Later repair was associated with longer time to reach full oral feeds, increased post-repair ventilator days, and increased need for tube feeds and supplementary oxygen at discharge. For infants with isolated CDH not requiring pre-operative ECLS, there is no difference in mortality based on timing of repair, but single-day delays in repair are associated with increased post-repair duration of ventilatory and nutritional support.

A predictive scoring system for small diaphragmatic defects in infants with congenital diaphragmatic hernia.

To develop a predictive score for small diaphragmatic defects in infants with congenital diaphragmatic hernia (CDH) for determining thoracoscopic surgery indication. The Japanese CDH Study Group cohort was randomly divided into derivation (n = 397) and validation (n = 396) datasets. Using logistic regression, a prediction model and weighted scoring system for small diaphragmatic defects were created from derivation dataset and validated with validation dataset. Six weighted variables were selected: no hydramnios, 1 point; 1min Apgar score of 5-10, 1 point; apex type of the lung (left lung is detected radiographically in apex area), 1 point; oxygenation index < 8, 1 point; abdominal nasogastric tube (tip of the nasogastric tube is detected radiographically in the abdominal area), 2 points; no right-to-left flow of ductus arteriosus, 1 point. In validation dataset, rates of small diaphragmatic defects for Possible (0-3 points), Probable (4-5 points), and Definite (6-7 points) groups were 36%, 81%, and 94%, respectively (p < 0.001). Additionally, sensitivity, specificity, positive predictive value, and C statistics were 0.78, 0.79, 0.88, 0.76, and 0.45, 0.94, 0.94, 0.70 for Probable and Definite groups, respectively. Our scoring system effectively predicted small diaphragmatic defects in infants with CDH.

Development of mortality prediction models for infants with isolated,left-sided congenital diaphragmatic hernia before and after birth.

Mortality prediction of congenital diaphragmatic hernia (CDH)is essential for developing treatment strategies, including fetal therapy. Several researchers have reported prognostic factors for this rare but life-threatening condition; however, the optimal combination of prognostic factors remains to be elucidated. This study aimed to develop the most discriminative prenatal and postnatal models to predict the mortality of infants with an isolated left-sided CDH. This multi-institutional retrospective cohort study included infants with CDH born at 15 tertiary hospitals of the Japanese CDH Study Group between 2011 and 2016. We developed multivariable logistic models with every possible combination of predictors and identified models with the highest cross-validated area under the receiver operating characteristic curve (AUC) for prenatal and postnatal predictions. Among 302 eligible infants, 44 died before discharge. The prenatal mortality prediction model was based on the observed/expected lung area to head circumference ratio (O/E LHR), liver herniation, and stomach herniation (AUC, 0.830). The postnatal mortality prediction model was based on O/E LHR, liver herniation, and the lowest oxygenation index (AUC, 0.944). Our models can facilitate the prenatal and postnatal mortality prediction of infants with isolated left-sided CDH.

Impact of Repeat Extracorporeal Life Support on Mortality and Short-term In-hospital Morbidities in Neonates With Congenital Diaphragmatic Hernia.

To evaluate the impact of repeat extracorporeal life support (ECLS) on survival and in-hospital outcomes in the congenital diaphragmatic hernia (CDH) neonates. Despite the widespread use of ECLS, investigations on multiple ECLS courses for CDH neonates are limited. This is a retrospective cohort study of all ECLS-eligible CDH neonates enrolled in the Congenital Diaphragmatic Hernia Study Group registry between 1995 and 2019. CDH infants with estimated gestational age at birth <32 weeks and a birth weight <1.8kg and/or with major cardiac or chromosomal anomalies were excluded. The primary outcomes were survival and morbidities during the index hospitalization. Of 10,089 ECLS-eligible CDH infants, 3025 (30%) received 1 ECLS course, and 160 (1.6%) received multiple courses. The overall survival rate for patients who underwent no ECLS, 1 ECLS course, and multicourse ECLS were 86.9±0.8%, 53.8±1.8%, and 43.1±7.7%, respectively. Overall ECLS survival rate is increased by 5.1±4.6% ( P =0.03) for CDH neonates treated at centers that conduct repeat ECLS compared with those that do not offer repeat ECLS. This suggests that there would be an overall survival benefit from increased use of multiple ECLS courses. Infants who did not receive ECLS support had the lowest morbidity risk, while survivors of multicourse ECLS had the highest rates of morbidities during the index hospitalization. Although survival is lower for repeat ECLS, the use of multiple ECLS courses has the potential to increase overall survival for CDH neonates. Increased use of repeat ECLS might be associated with improved survival. The potential survival advantage of repeat ECLS must be balanced against the increased risk of morbidities during the index hospitalization.

Comparison of CDHSG model and PCO2 in predicting mortality risk in patients with congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is one of the illnesses with high mortality and morbidity rates. The study aims to compare the Congenital Diaphragmatic Hernia Study Group (CDHSG) model and PCO2 in determining the mortality risk of CDH in the early postnatal period in neonates. The data of 35 patients who were treated CDH were analyzed retrospectively. The sex, gestational age, birth weight, delivery method, presence of chromosomal anomaly, congenital cardiac and other anomalies, pulmonary hypertension, the 5-min Apgar score, PCO2 values of blood gas in the first 24 h, mode of ventilation were recorded. According to the CDHSG model, the mortality risk of CDH was divided into three categories: as low, moderate, high risk. Based on the blood gases in the first 24 h after delivery, the CDH mortality risk was considered in two categories as low and high. Based on the CDHSG model, the risk of CDH mortality was low in 11.4%, moderate in 20%, and high in 68.6%. Mortality rates were 0%, 42.8%, and 83.3%, respectively. Based on the PaCO2 , the risk of CDH mortality was low in 37.1% of patients and high in 62.8%. The mortality rate was 86.3% in high-severity patients and 30.7% in low-risk patients. No significant difference was found between the area under the curve values of the CDHSG model and PCO2 . Especially in developing countries, in cases where opportunities are limited, the severity of the disease, the need for more aggressive treatment, and the need for higher-level intensive care can be determined with the easily accessible and low-cost blood gas PCO2 at the bedside.

Congenital Hepatopulmonary Fusion.

Congenital hepatopulmonary fusion (HPF) is a rare anomaly characterized by a fusion between the liver and lung parenchyma. HPF cases have been scarcely reported in the literature. An extensive search of publications was performed in the PubMed and Google Scholar databases without a time limit. In total, 34 clinical case reports were found in the literature, and a study by the Congenital Diaphragmatic Hernia (CDH) Study Group reported data on 10 patients with HPF. Of these 44 infants, 20 were male, 20 were female, and four were reported without gender specification. Nineteen (43.2%) patients required intubation on the first day of life. Six (13.6%) patients had their clinical presentation during the first year of life, and four (9%) clinically presented with HPF between 2.5 and 11 years of age. In these patients, cough, asthma-like symptoms, dyspnea, hemoptysis, right-side chest pain, respiratory infections, and pneumonia were the relevant clinical signs. Right-lung vascular anomalies were present in 18 (40.9%) patients. A complete liver and lung separation was successful in 17 (38.6%) patients. The overall survival was 56.8%. Congenital HPF has no gender predominance. In most cases, it behaves similar to a right CDH in need of resuscitation and intubation after birth. The majority of the cases are discovered during the surgical procedure for CDH. The best surgical approach has not been established and depends on the degree of fusion and vascular anomalies. An advanced imaging assessment is necessary before a surgical approach is attempted. The prognosis is ominous.