OBJECTIVE: Although many prognostic factors in neonates with congenital diaphragmatic hernia (CDH) have been described, no consensus thus far has been reached on which and how many factors are involved. The aim of this study is to analyze the association of multiple prenatal and postnatal factors with 1-month mortality of neonates with CDH and to construct a nomogram prediction model based on significant factors.
METHODS: A retrospective analysis of neonates with CDH at our center from 2013 to 2022 was conducted. The primary outcome was 1-month mortality. All study variables were obtained either prenatally or on the first day of life. Risk for 1-month mortality of CDH was quantified by odds ratio (OR) with 95% confidence interval (CI) in multivariable logistic regression models.
RESULTS: After graded multivariable adjustment, six factors were found to be independently and consistently associated with the significant risk of 1-month mortality in neonates with CDH, including gestational age of prenatal diagnosis (OR, 95% CI, P value: 0.845, 0.772 to 0.925, < 0.001), observed-to-expected lung-to-head ratio (0.907, 0.873 to 0.943, < 0.001), liver herniation (3.226, 1.361 to 7.648, 0.008), severity of pulmonary hypertension (6.170, 2.678 to 14.217, < 0.001), diameter of defect (1.560, 1.084 to 2.245, 0.017), and oxygen index (6.298, 3.383 to 11.724, < 0.001). Based on six significant factors identified, a nomogram model was constructed to predict the risk for 1-month mortality in neonates with CDH, and this model had decent prediction accuracy as reflected by the C-index of 94.42%.
CONCLUSIONS: Our findings provide evidence for the association of six preoperational and intraoperative factors with the risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO), has partly improved congenital diaphragmatic hernia (CDH) outcomes, yet the overall morbidity and mortality remain high. Existing prenatal indicators for CDH fetuses are operator-dependent, time-consuming, or less accurate, a new simple and accurate indicator to indicate adverse events in CDH patients is needed.
OBJECTIVE: To propose and assess the association of a new MRI parameter, the relative mediastinal displacement index (RMDI), with adverse events including in-hospital deaths or the need for ECMO in fetuses with isolated left CDH (iLCDH).
METHODS: Retrospective analysis.
METHODS: One hundred thirty-nine fetuses were included in the iLCDH group (24 with adverse events and 115 without) and 257 fetuses were included in the control group from two centers in Guangzhou.
UNASSIGNED: 3.0 T, T2WI-TRUFI; 1.5 T, T2WI-FIESTA.
RESULTS: Three operators independently measured the → DL $$ \\underset{\\mathrm{DL}}{\\to } $$ , → DR $$ \\underset{\\mathrm{DR}}{\\to } $$ , and DH on the axial images. The calculation formula of the RMDI was ( → DL $$ \\underset{\\mathrm{DL}}{\\to } $$  +  → DR $$ \\underset{\\mathrm{DR}}{\\to } $$ )/DH .
METHODS: The independent sample t test, Mann-Whitney U test, Chi-square test, Chi-square test continuity correction, Fisher\'s test, linear regression analysis, logistic regression analysis, intraclass correlation coefficient, receiver operating characteristic curve analysis, and Delong test. A P value <0.05 was considered statistically significant.
RESULTS: The RMDI did not change with gestational age in the iLCDH group (with [P = 0.189] and without [P = 0.567] adverse events) and the control group (P = 0.876). There were significant differences in RMDI between the iLCDH group (0.89 [0.65, 1.00]) and the control group (-0.23 [-0.34, -0.16]). In the iLCDH group, RMDI was the only indicator left for indicating adverse events, and the best cutoff value was 1.105. Moreover, there was a significant difference in diagnostic accuracy between the RMDI (AUC = 0.900) and MSA (AUC = 0.820), LHR (AUC = 0.753), o/e LHR (AUC = 0.709), and o/e TFLV (AUC = 0.728), respectively.
CONCLUSIONS: The RMDI is expected to be a simple and accurate tool for indicating adverse events in fetuses with iLCDH.
METHODS: 4 TECHNICAL EFFICACY: Stage 1.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a developmental defect that causes herniation of abdominal organs into the thoracic cavity with significant morbidity. Thoracoscopic repair of CDH is an increasingly prevalent yet controversial surgical technique, with limited long-term outcome data in the Asian region. The aim of this study was to compare open laparotomy versus thoracoscopic repair of CDH in paediatric patients in a major tertiary referral centre in Asia.
METHODS: We performed a retrospective analysis of neonatal patients who had open laparotomy or thoracoscopic repair for CDH in our institution between July 2002 and November 2021. Demographic data, perioperative parameters, recurrence rates and surgical complications were analysed.
RESULTS: 64 patients were identified, with 54 left sided CDH cases. 33 patients had a prenatal diagnosis and 35 patients received minimally invasive surgical repair. There was no significant difference between open and minimally invasive repair in recurrence rate (13 % vs 17 %, P = 0.713), time to recurrence (184 ± 449 days vs 81 ± 383 days, P = 0.502), or median length of ICU stay (11 ± 14 days vs 13 ± 15 days, P = 0.343), respectively. Gastrointestinal complications occurred in 7 % of neonates in the open group and none in the thoracoscopic group. Median follow-up time was 9.5 years.
CONCLUSIONS: This study is a large congenital diaphragmatic hernia series in Asia, with long term follow-up demonstrating no significant difference in recurrence rate, time to recurrence or median length of ICU stay between open and minimally invasive repair, suggesting thoracoscopic approach is a non-inferior surgical option with avoidance of gastrointestinal complications compared to open repair.
METHODS:
METHODS: Retrospective Cohort Study.

BACKGROUND: The prevalence of congenital diaphragmatic hernia (CDH) varies across countries, with limited information available on its epidemiology in China. Our study aimed to investigate the prevalence, time trends, and perinatal outcomes of CDH in China, as well as its associated malformations and potential associations with maternal and infant characteristics.
METHODS: This study included all birth and CDH cases from the Chinese Birth Defects Monitoring Network between 2007 and 2019, with CDH cases classified as either isolated or associated. We employed the joinpoint regression model to calculate the trends of prevalence and the annual percent change, with Poisson regression used for adjusted prevalence rate ratios. A P value ≤ 0.05 was considered statistically significant.
RESULTS: A total of 4397 CDH cases were identified among 24,158,029 births in the study period, yielding prevalence rates of 1.82, 1.13 and 0.69 per 10,000 for overall, isolated, and associated CDH, respectively. The prevalence of each type of CDH increased over time. The prevalence of overall CDH varied significantly by infant sex (male vs. female, 1.91/10,000 vs. 1.63/10,000), maternal residence (urban vs. rural, 2.13/10,000 vs. 1.45/10,000), maternal age (< 20 years, 1.31/10,000; 20-24 years, 1.63/10,000; 25-29 years, 1.80/10,000; 30-34 years, 1.87/10,000; ≥ 35 years, 2.22/10,000), and geographic region (central, 1.64/10,000; east, 2.45/10,000; west, 1.37/10,000). Cardiovascular anomalies were the most common malformations associated with CDH. Infants with associated CDH had a higher risk of premature birth and perinatal death than those with isolated CDH.
CONCLUSIONS: The increasing prevalence and high perinatal mortality rate of CDH highlight the need for further etiological, epidemiological, and clinical studies among the Chinese population. Video Abstract.

UNASSIGNED: To evaluate whether the patent ductus arteriosus (PDA) can serve as a predictive factor for inpatient outcomes in congenital diaphragmatic hernia (CDH) patients.
UNASSIGNED: A retrospective cohort study was conducted on 59 CDH patients at the Capital Institute of Pediatrics from January 2020 to August 2022. Echocardiography was performed at least three times: within 2-3 h after birth, pre-operatively, and post-operatively of CDH surgery. Based on the direction of the PDA shunt in the first echocardiogram, patients were classified into three groups: left-to-right shunting or closed PDA (L-R), bi-directional shunting, and right-to-left shunting (R-L).
UNASSIGNED: The mortality rate was 15.3% (9/59), with all non-survivors having R-L shunting and group mortality of 39.1% (9/23). The direction of the PDA shunt was significantly associated with the duration of ventilation and length of hospital stay (p < 0.05). Decreased PDA diameter or pre-operative shunting direction change towards L-R or bi-directional shunting were associated with higher survival rates, while increased PDA diameter or continuous R-L shunting were associated with higher mortality rates. Pre-operative PDA shunt direction, PDA size after birth and before surgery, gestational age of diagnosis, and shortening fraction before surgery were significantly correlated with patient outcomes. The direction of the preoperative PDA shunt was the most relevant factor among these relationships (p = 0.009, OR 20.6, CI 2.2∼196.1).
UNASSIGNED: Our findings highlight the importance of monitoring changes in PDA shunt directionality and diameter in the early stage after birth, as these parameters may serve as valuable predictors of patient outcomes.

OBJECTIVE: To develop a mediastinal shift angle (MSA) measurement method applicable to right-sided congenital diaphragmatic hernia (RCDH) in fetal MRI and to validate the predictive value of MSA in RCDH.
METHODS: Twenty-seven fetuses with isolated RCDH and 53 controls were included in our study. MSA was measured on MRI axial image at the level of four-chamber view of the fetal heart. The angle between the sagittal midline landmark line and the left boundary landmark line touching tangentially the lateral wall of the left ventricle was used to quantify MSA for RCDH. Appropriate statistical analyses were performed to determine whether MSA can be regarded as a valid predictive tool for postnatal outcomes. Furthermore, predictive performance of MSA was compared with that of lung area to head circumference ratio (LHR), observed/expected LHR (O/E LHR), total fetal lung volume (TFLV), and observed/expected TFLV (O/E TFLV).
RESULTS: MSA was significantly higher in the RCDH group than in the control group. MSA, LHR, O/E LHR, TFLV, and O/E TFLV were all correlated with postnatal survival, pulmonary hypertension (PH), and extracorporeal membrane oxygenation (ECMO) therapy (p < 0.05). Value of the AUC demonstrated good predictive performance of MSA for postnatal survival (0.901, 95%CI: (0.781-1.000)), PH (0.828, 95%CI: (0.661-0.994)), and ECMO therapy (0.813, 95%CI: (0.645-0.980)), which was similar to O/E TFLV but slightly better than TFLV, O/E LHR, and LHR.
CONCLUSIONS: We developed a measurement method of MSA for RCDH for the first time and demonstrated that MSA could be used to predict postnatal survival, PH, and ECMO therapy in RCDH.
CONCLUSIONS: Newly developed MRI assessment method of fetal MSA in RCDH offers a simple and effective risk stratification tool for patients with RCDH.
CONCLUSIONS: • We developed a measurement method of mediastinal shift angle for right-sided congenital diaphragmatic hernia for the first time and demonstrated its feasibility and reproducibility. • Mediastinal shift angle can predict more prognostic information other than survival in right-sided congenital diaphragmatic hernia with good performance. • Mediastinal shift angle can be used as a simple and effective risk stratification tool in right-sided congenital diaphragmatic hernia to improve planning of postnatal management.

UNASSIGNED: Brindle 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.
UNASSIGNED: Multiple 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.
UNASSIGNED: A 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.
UNASSIGNED: The 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.

UNASSIGNED: Congenital diaphragmatic hernia (CDH) is a structural defect caused by inadequate fusion of the pleuroperitoneal membrane that forms the diaphragm, allowing peritoneal viscera to protrude into the pleural cavity. Up to 30% of newborns with CDH require extracorporeal membrane oxygenation (ECMO) support. As with all interventions, the risks and benefits of ECMO must be carefully considered in these patients. Cardiopulmonary function has been shown to worsen rather than improve after surgical CDH repair. Even after a detailed perioperative assessment, sudden cardiopulmonary failure after surgery is dangerous and requires timely and effective treatments.
UNASSIGNED: Three cases of cardiopulmonary failure after surgical CDH treatment in newborns have been reported. ECMO support was needed for these three patients and was successfully discontinued. We report our treatment experience.
UNASSIGNED: ECMO is feasible for the treatment of postoperative cardiopulmonary failure in newborns with CDH.

Congenital diaphragmatic hernia (CDH) is associated with high mortality rates and significant pulmonary morbidities. The objective of this study was to delineate the histopathological features observed in necropsies of CDH patients and correlate these with their clinical manifestations.
We retrospectively reviewed the postmortem findings and corresponding clinical characteristics in eight CDH cases from 2017 to July 2022.
The median survival time was 46 (8-624) hours. Autopsy reports showed that diffuse alveolar damage (congestion and hemorrhage) and hyaline membrane formation were the primary pathological lung changes observed. Notably, despite significant reduction in lung volume, the lung development appeared normal in 50% of the cases, while lobulated deformities were present in three (37.5%) cases. All patients displayed a large patent ductus arteriosus (PDA) and a patent foramen ovale, resulting in increased right ventricle (RV) volume, and myocardial fibers appeared slightly congested and swollen. The pulmonary vessels indicated thickening of the arterial media and adventitia. Lung hypoplasia and diffuse lung damage resulted in impaired gas exchange, while PDA and pulmonary hypertension led to RV failure, subsequent organ dysfunction and ultimately death.
Patients with CDH typically succumb to cardiopulmonary failure, a condition driven by a complex interplay of pathophysiological factors. This complexity accounts for the unpredictable response to currently available vasodilators and ventilation therapies.

A fetus was found to have a right diaphragmatic hernia during a prenatal ultrasonography examination late in the second trimester. A \"green channel\" with multi department dynamic monitoring was instituted, at 40 + 4 weeks, with the infant under general anesthesia, hernia repair was later successfully performed. After the operation, the infant\'s vital signs were stable and their condition remained good during follow-up.