Neonatal Congenital Diaphragmatic Hernia Research Articles

Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives.

In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25-30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24-48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.

Prenatal treatment with nitrite reduces pulmonary arteriolar remodeling in neonates with congenital diaphragmatic hernia

Therapeutic use of alternative nitric oxide (NO) sources, such as nitrite and nitrate may be a protective influence on pulmonary vasculature abnormalities. AimTo evaluate whether the maternal administration of nitrite prevents the morphological and molecular changes that affect the pulmonary arterioles of congenital diaphragmatic hernia (CDH) neonates. MethodsCEUA #88/2017. Sprague-Dawley neonate rats were divided into 6 groups: 1. control; 2. control + nitrite; 3. nitrofen exposed; 4. nitrofen exposed + nitrite; 5. CDH and 6. CDH + nitrite. The pregnant rats from nitrofen exposed and CDH groups were exposed to nitrofen on gestational day (GD) 9.5. The treatment with nitrite was made by gavage (15 mg/kg/day), on the last five gestational days. On GD 21.5 the fetuses were harvested. The following parameters were analyzed: lung and plasma nitrite concentration; media wall thickness (MWT) and endothelial NO synthase eNOS and inducible NO synthase iNOS immunohistochemistry of pulmonary arterioles. ResultsNitrite treatment increased the maternal plasma concentration of nitrite in control and nitrofen-exposed rats. All neonates exposed to nitrofen showed an increase of nitrofen in the lung and plasma. Nitrite treatment decreased the MWT of pulmonary arterioles of CDH neonates. Nitrite treatment increased eNOS marker and attenuated iNOS marker in neonates with CDH. ConclusionsNitrite maternal treatment rescued the morphometry and recovered eNOS expression of CDH pulmonary arterioles during CDH. Nitrite is a potential prenatally therapeutic approach to vascular alterations present in CDH neonates.

Diagnosis, management and long term cardiovascular outcomes of phenotypic profiles in pulmonary hypertension associated with congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm resulting in herniation of viscera into the chest. This condition is characterized by pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. PH is a key component of the pathophysiology of CDH in neonates and contributes to morbidity and mortality. Traditionally, PH associated with CDH (CDH-PH) is thought to be secondary to increased pulmonary arterial resistance and vasoreactivity resulting from pulmonary hypoplasia. Additionally, there is increasing recognition of associated left ventricular hypoplasia, dysfunction and elevated end diastolic pressure resulting in pulmonary venous hypertension in infants with CDH. Thus, hemodynamic management of these infants is complex and cautious use of pulmonary vasodilators such as inhaled nitric oxide (iNO) is warranted. We aim to provide an overview of different phenotypic profiles of CDH associated PH and potential management options based on current evidence and pathophysiology.

Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review.

To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare. 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156months, mean 38.7 ± 40.7). cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5days, prematurity ≤ 34weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3months of age up to at least 3-4years of age, and at least annually, should be recommended.

Extracorporeal membrane oxygenation for congenital diaphragmatic hernia: how to begin?

Extracorporeal membrane oxygenation (ECMO) is critical for congenital diaphragmatic hernia (CDH), who fails to achieve adequate oxygenation with conventional management. We aim to evaluate initial experiences with ECMO support in a tertiary women and children's medical center in mainland China. We retrospectively reviewed the establishment of ECMO for CDH Program in our center and analysis of five CDH neonates, who underwent repair during ECMO between December 2016 and December 2018. The first ECMO for CDH Program in our institution was established and managed by a multidisciplinary team since December 2016. An alert of ECMO was prenatally created for moderate-severe pulmonary hypoplasia. Of sixteen admissions prenatally diagnosed moderate-severe CDH, eight neonates (50%) required ECMO but five (31%) received eventually. Veno-arterial ECMO was established from 3 hours to 41 hours of age (median 20 h). All the five underwent CDH repair after a stabilization period on ECMO, which ranged from 12 h to 122 h (median 58 h). There were no clotting complications, related to coagulating during CDH repair. Bleeding was the most common complication. Cannula malposition was detected in one case. Their median ECMO duration was 437 (range 85-946) hours. Neonatal survival was 80% (4/5) and 3 survived (60%) in the first three months of life. Multidisciplinary teamwork, precise prenatal evaluation and skillful cannulation assist the successful beginning of ECMO for CDH. Our preliminary results would encourage other institutions, whose ECMO is not well-established.

Development of a 3D-printed neonatal congenital diaphragmatic hernia model and standardisation of intra-operative measurement

IntroductionThree-dimensional (3D) printing is frequently used for surgical simulation and training, however, no widely available model exists for neonatal congenital diaphragmatic hernia (CDH). The aim of this study was to develop a 3D-printed model of CDH and test interobserver variability in the simulated model for obtaining measurements of the diaphragmatic defect and ipsilateral diaphragm.MethodsA term fetal MRI (3.5 kg) of thorax, diaphragm and defect (15 mm × 5 mm) were delineated and segmented after parental consent to produce 3D-printed models. Consultant and trainee paediatric surgeons were invited to measure the posterior-lateral diaphragmatic defect and ipsilateral diaphragm. Mean measurement error was calculated (millimetres). Data are presented as median (range) and number/total (%).ResultsAn abdominal and thoracoscopic model were produced and tested by 52 participants (20 consultants and 32 trainees). Diaphragmatic defect via laparotomy measured 15 (10–20) mm (AP) × 16 (10–25) mm (ML) and thoracoscopically 14 (11–19) mm (AP) × 15 (11–22) mm (ML). Mean error per measurement was 4 (1–17) mm via laparotomy vs. 3 (0.5–9.5) mm thoracoscopically. Mean error was similar between consultants and trainees via laparotomy (4.3 vs. 3.9 mm, p = 0.70) and thoracoscopically (3 vs. 3 mm, p = 0.79). Error did not correlate with experience as operating surgeon via laparotomy (β = 13.0 [95% CI − 55.9 to 82.0], p = 0.71) or thoracoscopically (β = 1.4[95% CI − 6.4 to 9.2], p = 0.73.ConclusionsWe have designed and built simulation models for CDH repair via laparotomy and thoracoscopically. Operators can reliably measure the diaphragmatic defect and ipsilateral diaphragm, regardless of surgical experience and operative approach.

Spontaneous breathing in selected neonates with very mild congenital diaphragmatic hernia.

Current treatment guidelines recommend immediate postnatal intubation in all neonates with congenital diaphragmatic hernia (CDH). This study aimed to investigate the feasibility and outcomes of a spontaneous breathing approach (SBA) versus immediate intubation in neonates with prenatally diagnosed very mild CDH. A retrospective study was conducted comparing neonates with very mild CDH (left-sided, liver-down, observed-to-expected lung-to-head ratio ≥45%) undergoing SBA and matched controls receiving standard treatment. Data on early echocardiographic findings, respiratory support, length of hospital stay, and clinical outcomes were analyzed. Of 151 CDH neonates, eight underwent SBA, while 31 received standard treatment. SBA was successful in six of eight patients. SBA patients had shorter length of stay (14 vs. 30 days, p = .005), mechanical ventilation (3.5 vs. 8.7 days, p = .011), and oxygen supplementation (3.2 vs. 9.3 days, p = .013) compared to matched controls. Echocardiographic evidence of pulmonary hypertension and cardiac dysfunction were significantly lower in SBA neonates after admission but similar before surgical repair. The SBA group tolerated enteral feeding earlier (day of life 7 vs. 16, p = .019). SBA appears feasible and beneficial for prenatally diagnosed very mild CDH. It was associated with a shortened hospital stay supportive therapies. However, larger trials are needed to confirm these findings and determine optimal respiratory support.

Molecular insights using spatial transcriptomics of the distal lung in congenital diaphragmatic hernia.

Abnormal pulmonary vascular development and function in congenital diaphragmatic hernia (CDH) is a significant factor leading to pulmonary hypertension. The lung is a very heterogenous organ and has marked cellular diversity that is differentially responsive to injury and therapeutic agents. Spatial transcriptomics provides the unmatched capability of discerning the differences in the transcriptional signature of these distinct cell subpopulations in the lung with regional specificity. We hypothesized that the distal lung parenchyma (selected as a region of interest) would show a distinct transcriptomic profile in the CDH lung compared with control (normal lung). We subjected lung sections obtained from male and female CDH and control neonates to spatial transcriptomics using the Nanostring GeoMx platform. Spatial transcriptomic analysis of the human CDH and control lung revealed key differences in the gene expression signature. Increased expression of alveolar epithelial-related genes (SFTPA1 and SFTPC) and angiogenesis-related genes (EPAS1 and FHL1) was seen in control lungs compared with CDH lungs. Response to vitamin A was enriched in the control lungs as opposed to abnormality of the coagulation cascade and TNF-alpha signaling via NF-kappa B in the CDH lung parenchyma. In male patients with CDH, higher expression of COL1A1 (ECM remodeling) and CD163 was seen. Increased type 2 alveolar epithelial cells (AT-2) and arterial and lung capillary endothelial cells were seen in control lung samples compared with CDH lung samples. To the best of our knowledge, this is the first use of spatial transcriptomics in patients with CDH that identifies the contribution of different lung cellular subpopulations in CDH pathophysiology and highlights sex-specific differences.NEW & NOTEWORTHY This is the first use of spatial transcriptomics in patients with congenital diaphragmatic hernia (CDH) that identifies the contribution of different lung cellular subpopulations in CDH pathophysiology and highlights sex-specific differences.

P‐TS‐35 | Group O Red Blood Cell Utilization in Group A recipients with Neonatal Congenital Diaphragmatic Hernia Surgical Repair: A Patient Blood Management Opportunity

P‐TS‐35 | Group O Red Blood Cell Utilization in Group A recipients with Neonatal Congenital Diaphragmatic Hernia Surgical Repair: A Patient Blood Management Opportunity

Hemolysis during Venovenous Extracorporeal Membrane Oxygenation in Neonates with Congenital Diaphragmatic Hernia: A Prospective Observational Study

To investigate the incidence of hemolysis and its association with outcome in neonates with congenital diaphragmatic hernia (CDH) requiring venovenous extracorporeal membrane oxygenation (ECMO) treatment using a Medos Deltastream circuit with a DP3 pump, a hilite 800 LT oxygenator system, and a ¼' tubing. Plasma free hemoglobin (PFH) was prospectively measured once daily during ECMO using spectrophotometric testing. Patients (n=62) were allocated into two groups according to presence or absence of hemolysis. Hemolysis was defined as PFH ≥ 50mg/dL on at least 2 consecutive days during ECMO treatment. Hemolysis was classified as either moderate with a maximum PFH of 50-100mg/dL or severe with a maximum PFH >100mg/dL. Hemolysis was detected in 14 patients (22.6%). Mortality was 100% in neonates with hemolysis compared with 31.1% in neonates without hemolysis (P<.001). In 21.4% hemolysis was moderate and in 78.6% severe. Using multivariable analysis, hemolysis (hazard ratio: 6.8; 95%CI: 1.86-24.86) and suprasystemic pulmonary hypertension (PH) (hazard ratio: 3.07; 95%CI: 1.01-9.32) were independently associated with mortality. Hemolysis occurred significantly more often using 8 French (Fr) cannulae than 13 Fr cannulae (43% vs 17%; P=.039). Cutoff for relative ECMO flow to predict hemolysis were 115ml/kg/ minute for patients with 8 Fr cannulae (Area under the curve [AUC] 0.786, P=.042) and 100ml/kg/ minute for patients with 13 Fr cannulae (AUC 0.840, P<.001). Hemolysis in CDH neonates receiving venovenous ECMO is independently associated with mortality.

Enteric tube position on preoperative radiographs predicts clinical outcomes in neonatal congenital diaphragmatic hernia with and without prenatal diagnosis.

Congenital Diaphragmatic Hernia (CDH) is diagnosed prenatally in ~60% of cases. Prenatal measures typically guide management and prognostication. Simple postnatal prognosticators are needed when prenatal diagnosis is lacking. We hypothesized that preoperative orogastric tube (OGT) tip position relative to the contralateral diaphragm correlates with defect severity, resource utilization, and clinical outcomes regardless of diagnostic status. 150 neonates with left-posterolateral CDH were analyzed. Impact of intrathoracic and intraabdominal preoperative tip position on clinical outcomes was compared. Ninety-nine neonates were prenatally diagnosed. Overall, intrathoracic position significantly correlated with larger diaphragmatic defects, advanced postnatal pulmonary support requirements (HFOV, pulmonary vasodilators, and ECMO), operative complexity, longer hospitalization, and poorer survival to discharge. These observations persisted when analyzing only cases lacking prenatal diagnosis. Preoperative OGT tip position predicts defect severity, resource utilization, and outcomes in CDH. This observation enhances postnatal prognostication and care planning for neonates without a prenatal diagnosis.

The Association between the First Cry and Clinical Outcomes in CDH Neonates: A Retrospective Study.

Congenital diaphragmatic hernia (CDH) is a life-threatening condition characterized by the herniation of abdominal organs into the thorax, resulting in hypoplastic lungs and pulmonary hypertension. The impact of the first cry, a crucial event for lung transition during birth, on CDH patients remains unclear. This study investigated the impact of the first cry during birth on CDH patient survival, along with other prognosis factors. A multi-institutional retrospective study assessed CDH patient characteristics and survival rates by analyzing factors including the first cry, disease severity, birth weight, Apgar scores, oxygenation index (OI) and surgical closure. Among the CDH patients in the study, a positive first cry was linked to 100% survival, regardless of disease severity (p < 0.001). Notably, the presence of a positive first cry did not significantly affect survival rates in patients with worse prognostic factors, such as low birth weight (<2500 g), high CDH severity, low Apgar scores (1 min ≤ 4), high best OI within 24 h after birth (≥8), or those who underwent patch closure. Furthermore, no significant association was found between the first cry and the use of inhaled nitric oxide (iNO) or extracorporeal membrane oxygenation (ECMO). In conclusion, this study suggests that the first cry may not have a negative impact on the prognosis of CDH patients and could potentially have a positive effect.

Dynamics of pulmonary hypertension severity in the first 48 h in neonates with prenatally diagnosed congenital diaphragmatic hernia.

Pulmonary hypertension (PH) is one of the major contributing factors to the high morbidity and mortality in neonates with congenital diaphragmatic hernia (CDH). The severity and duration of postnatal PH are an established risk factor for patient outcome; however, the early postnatal dynamics of PH have not been investigated. This study aims to describe the early course of PH in CDH infants, and its relation to established prognostic markers and outcome measures. We performed a monocentric retrospective review of neonates with prenatally diagnosed CDH, who received three standardized echocardiographic examinations at 2-6 h, 24, and 48 h of life. The degree of PH was graded as one of three categories: mild/no, moderate, or severe PH. The characteristics of the three groups and their course of PH over 48 h were compared using univariate and correlational analyses. Of 165 eligible CDH cases, initial PH classification was mild/no in 28%, moderate in 35%, and severe PH in 37%. The course of PH varied markedly based on the initial staging. No patient with initial no/mild PH developed severe PH, required extracorporeal membrane oxygenation (ECMO)-therapy, or died. Of cases with initial severe PH, 63% had persistent PH at 48 h, 69% required ECMO, and 54% died. Risk factors for any PH included younger gestational age, intrathoracic liver herniation, prenatal fetoscopic endoluminal tracheal occlusion (FETO)-intervention, lower lung to head ratio (LHR), and total fetal lung volume (TFLV). Patients with moderate and severe PH showed similar characteristics, except liver position at 24- (p = 0.042) and 48 h (p = 0.001), mortality (p = 0.001), and ECMO-rate (p = 0.035). To our knowledge, this is the first study to systematically assess the dynamics of PH in the first postnatal 48 h at three defined time points. CDH infants with initial moderate and severe PH have a high variation in postnatal PH severity over the first 48 h of life. Patients with mild/no PH have less change in PH severity, and an excellent prognosis. Patients with severe PH at any point have a significantly higher risk for ECMO and mortality. Assessing PH within 2-6 h should be a primary goal in the care for CDH neonates.

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.

Neonatal outcomes of congenital diaphragmatic hernia in full term versus early term deliveries: A systematic review and meta-analysis.

This systematic review and meta-analysis aimed to review the optimal timing of delivery at term for neonates with prenatally diagnosed congenital diaphragmatic hernia (CDH). We reviewed the literature up to December 19, 2022 using MEDLINE and the Cochrane Library databases. The inclusion criteria were original articles, comparative studies of CDH neonates delivered at an early term (37-38weeks of gestation) and at full term (39weeks of gestation or later), and comparative studies investigating outcomes of CDH neonates. Six studies met the inclusion criteria, including 985 neonates delivered at an early term and 629 delivered at full term. The cumulative rate of survival to discharge showed no significant difference between CDH neonates delivered at an early term (395/515; 76.7%) or at full term (345/467; 73.9%) (risk ratio [RR] 1.01; 95% confidence interval [CI], 0.89-1.16; p=0.85). Furthermore, the number of neonates requiring oxygen therapy at discharge was not significantly different between CDH neonates delivered at an early term (32/370; 8.6%) and at full term (14/154; 9.1%) (RR, 0.99; 95% CI, 0.36-2.70; p=0.99). Therefore, the optimal timing of delivery at term for neonates with CDH remains unclear.

Preliminary study on the predictive value of the vasoactive-inotropic score for the prognosis of neonatal congenital diaphragmatic hernia

BackgroundNo study has reported on the relationship between the vasoactive-inotropic score (VIS) and the prognosis of neonates with a severe congenital diaphragmatic hernia (CDH). This study aimed to identify potential risk factors for mortality in patients with CDH. We calculated the VIS based on the vasoactive drugs used during the perioperative period to investigate the relationship between VIS and infant prognosis.MethodsWe retrospectively analyzed the clinical data of 75 neonates with CDH who were treated at our center between January 2016 and October 2021. We calculated the maximum and mean VIS during the first 24 h of hospitalization (hosVIS [24max] and hosVIS [24mean], respectively) and after surgery (postVIS [24max] and postVIS [24mean], respectively). The relationship between the VIS and the prognosis of neonates with CDH was analyzed using a receiver operating characteristic (ROC) curve, t-test, chi-square test, rank-sum test, and logistic regression analysis.ResultsIn total, 75 participants with CDH were included in the study. The chance of survival was 80%. Our results showed that hosVIS (24max) was an accurate predictor of prognosis (area under the ROC curve = 0.925, p = 0.007). The calculated optimal critical value of hosVIS (24max) for predicting a poor prognosis was 17 (J = 0.75). Multivariate analysis revealed that hosVIS (24max) was an independent risk factor for death in neonates with CDH.ConclusionIn neonates with CDH, a higher VIS, especially hosVIS (24max), suggests worsened cardiac function, a more severe condition, and a higher risk of death. The rising VIS score in infants prompts physicians to implement more aggressive treatment to improve cardiovascular function.

Ductus arteriosus flow predicts outcome in neonates with congecsion.

To investigate whether the pattern of flow through the ductus arteriosus (DA) is associated with the need for extracorporeal membrane oxygenation support (ECMO) or death in neonates with congenital diaphragmatic hernia (CDH). Retrospective observational study. German level III Neonatal Intensive Care Unit. One hundred and nine CDH neonates were born between March 2009 and May 2021. DA flow pattern was assessed in echocardiograms obtained within 24 h of life by measuring flow time and velocity time integral (VTI) for both left-to-right (LR) and right-to-left (RL) components of the ductal shunt. A VTI ratio (VTILR/VTIRL) < 1.0 and an RL relative flow time (flow timeRL/(Flow timeLR+Flow timeRL)) >33% were defined as markers of abnormal flow patterns. The primary outcome was the need for ECMO. The secondary outcome was death. Seventy-two patients (51.8%) had a VTI ratio <1.0, 73 (52.5%) an RL relative flow time >33%. Fifty-nine patients (42.4%) had an alteration of both values. Need for ECMO was present in 37.4% (n = 52), while 19.4% (n = 27) died. A VTI ratio <1.0 had the highest diagnostic accuracy for the need for ECMO, (sensitivity 82.7%, specificity 66.7%, negative predictive value [NPV] 86.6%, and positive predictive value [PPV] 59.7%) as well as for death (sensitivity 77.8%, specificity 54.5%, NPV 91.0%, and PPV 29.2%). Patients with VTI ratio <1.0 were 4.7 times more likely to need ECMO and 3.3 times more likely to die. VTI ratio values correlated significantly with pulmonary hypertension (PH) severity (r = -0.516, p < 0.001). A VTI ratio <1.0 is a valuable threshold to identify high-risk CDH neonates. For improved risk stratification, other parameters-for example, left ventricular cardiac dysfunction-should be combined with DA flow assessment.

Assessment of Antenatal and Postnatal Prognostic Indicators in the Outcome of Neonatal Congenital Diaphragmatic Hernia: A Prospective Observational Study.

Despite advances in neonatal intensive care, surgical methods, and anesthesia, congenital diaphragmatic hernia (CDH) is still associated with significant mortality. Predicting which babies will have poorer outcomes is essential to identify the high-risk babies and to give targeted care and accurate prognosis to the parents, especially in a resource crunch set-up. The aim of this study is to evaluate the antenatal and postnatal prognostic factors in neonatal CDH that can be used to predict the outcome. This was a prospective observational study in a tertiary care center. Neonates presented with CDH within 28 days of life were included in the study. Bilateral disease, recurrent diseases, and babies operated outside were excluded from the study. The data were collected prospectively, and babies were followed until discharge or death. Data were expressed in mean with standard deviation or median with range based on normality. All the data were analyzed using the SPSS software version 25. Thirty babies with neonatal CDH were studied. There were three right-sided cases. The male-to-female ratio was 2.3:1, and 93% of babies were antenatally diagnosed. Seventeen out of the 30 babies underwent surgery. Nine (52.9%) underwent laparotomy, and 8 (47%) underwent thoracoscopic repair. Overall mortality was 53.3%, and operative mortality was 17.6%. Demographic characteristics were comparable between expired versus survived babies. The significant predictors of outcome identified were - Persistent pulmonary hypertension (PPHN), mesh repair, high-frequency oscillatory ventilation (HFOV), use of inotropes, 5-min APGAR, ventilator index (VI), and HCO3 levels. We conclude that the prognostic indicators associated with poor prognosis are low 5-min APGAR, high VI, low HCO3 levels in venous blood gas analysis, mesh repair, HFOV, inotropes usage, and PPHN. None of the antenatal factors studied showed any statistical significance. Further prospective studies with a larger sample size are recommended to confirm the findings.

A new method of ventilation inhomogeneity assessment based on a simulation study using clinical data on congenital diaphragmatic hernia cases

Congenital Diaphragmatic Hernia (CDH) is a diaphragm defect associated with lung hypoplasia and ventilation inhomogeneity (VI). The affected neonates are usually born with respiratory failure and require mechanical ventilation after birth. However, significant interindividual VI differences make ventilation difficult. So far, there are no clinical methods of VI assessment that could be applied to optimize ventilation at the bedside. A new VI index is a ratio of time constants T1/T2 of gas flows in both lungs. Pressure-controlled ventilation simulations were conducted using an infant hybrid (numerical-physical) respiratory simulator connected to a ventilator. The parameters of the respiratory system model and ventilator settings were based on retrospective clinical data taken from three neonates (2, 2.6, 3.6 kg) treated in the Paediatric Teaching Clinical Hospital of the Medical University of Warsaw. We searched for relationships between respiratory system impedance (Z) and ventilation parameters: work of breathing (WOB), peak inspiratory pressure (PIP), and mean airway pressure (MAP). The study showed the increased VI described by the T1/T2 index value highly correlated with elevated Z, WOB, PIP and MAP (0.8–0.9, the Spearman correlation coefficients were significant at P < 0.001). It indicates that the T1/T2 index may help to improve the ventilation therapy of CDH neonates.

Thoracoscopic surgery for congenital diaphragmatic hernia in neonates: Should it be the first choice?

Congenital diaphragmatic hernia (CDH) is an uncommon but potentially life-threatening surgical condition in neonates. Surgery can be performed by either open or thoracoscopic techniques. In this study, we compared the clinical efficacy, safety, and effectiveness of thoracoscopic and open CDH repair. A retrospective review of neonates with CDH who underwent operations at our hospital from 2013 to 2021 was performed. The various perioperative parameters were compared between neonates undergoing thoracoscopic and open surgery. There were 50 neonates in this study (37 in the thoracoscopic group and 13 in the open group). Thoracoscopic surgery was associated with significantly shorter hospital stay (13.32 vs. 18.77 days, p < 0.001); shorter duration of postoperative mechanical ventilation (3.70 vs. 5.98 days, p < 0.001); early feeding (4.34 vs. 7.46 days, p < 0.001); and shorter time to reach optimal feeding (8.21 vs. 13.38 days, p < 0.001). There was one postoperative death in the open group and no death in the thoracoscopic group. The median follow-up time of the two groups was 23.8 months (20.5 months in open group and 25.0 months in thoracoscopic group). Thoracoscopic surgery was associated with lower recurrence rates, but the difference was not statistically significant (2.7% vs. 7.7%, p = 0.456). Thoracoscopy CDH repair, a safe and effective surgical technique for neonates, has better cosmesis, faster postoperative recovery, and a lower recurrence rate than other procedures. It can be considered the first choice for CDH treatment for neonates among experienced surgeons.