Treatment Of Congenital Diaphragmatic Hernia Research Articles

COMPARATIVE EVALUATION OF SURGICAL OUTCOMES IN NEONATES WITH CONGENITAL DIAPHRAGMATIC HERNIA TREATED BY LAPAROTOMY OR THORACOTOMY

The choice of surgical approach often depends on the experience of the surgeon and the specific clinical situation. The lack of consensus makes it difficult to compare outcomes between centers and hinders the establishment of a standard for high-risk CDH patients. Objective: to describe the structure and comparison of reoperation rates in children with congenital diaphragmatic hernia (CDH) based on surgical approach, to identify the major risk factors for reoperation, and to provide pathophysiologic rationale for the optimal surgical approach. Materials and Methods: This single-center prospective cohort study compares the outcomes of surgical correction of CDH by laparotomy (n=53) and thoracotomy (n=41). The study included a neonatal group of children with high-risk left-sided CDH who underwent surgery at Ohmatdyt Hospital between 2000 and 2024. Follow-up was at least one year. Clinical data were analyzed statistically. The mean (M) and standard deviation (SD), and the median (Me) and interquartile range (IQR) for metric data were calculated. Comparisons were made using the independent samples t-test with calculation of critical value (t) and statistical significance (p). For nominal data, the 2 criterion was used. The calculation of mortality risk included the assessment of absolute risks, their ratio (RR) with the determination of the error and 95% confidence interval. The analysis was performed with MS Excel and IBM SPSS Statistic 19. The study was conducted in accordance with the tenets of the Declaration of Helsinki. The study protocol was approved by the Local Ethics Committee (LEC) of all institutions mentioned in the paper. Informed consent for the study was obtained from the parents (or guardians) of the children. Results: The overall rate of surgical complications was higher in the laparotomy group (39.6% vs. 29.3%, p = 0.39), including the rate of reoperations (30.1% vs. 19.5%, p = 0.32) during the observation period. Complications such as adhesive and strangulation bowel obstruction and ventral hernia formation were more common in the laparotomy group. Recurrence and surgical treatment of GERD were almost equal (7.3% vs. 1.9%, p = 0.196). Early postoperative mortality was higher in the laparotomy group (32% vs. 17%, p=0.09). Conclusions: Postnatal treatment of CDH by laparotomy is associated with a higher incidence of surgical complications such as bowel obstruction and ventral hernia formation. Mortality was also more common in the laparotomy group. The use of thoracotomy as a surgical approach allows a pathogenetically justified correction of CDH by applying the developed concept of thoracalization of the abdominal cavity. This creates conditions for reducing the incidence of complications and improving the results of CDH repair.

Pleural Effusion and Chylothorax in Congenital Diaphragmatic Hernia-Risk Factors, Management and Outcome.

Background: Pleural effusion and chylothorax are common complications in the treatment of congenital diaphragmatic hernia (CDH). We set out to identify risk factors for chylothorax development in patients with CDH and to investigate the association of pleural effusion and chylothorax with neonatal morbidity and mortality. Methods: In this retrospective cohort study, we included 396 neonates with CDH treated at our institution between January 2013 and June 2019. Preoperative and postoperative chest radiographs and clinical data were evaluated and correlated with morbidity, complications and mortality. Results: Laboratory-confirmed chylothorax occurred in 58 (18.6%) of all CDH cases. Pleural effusion was frequently observed as a postoperative complication but also occurred as a pre-existing condition. Neonates with large defects of size C and D, patch repair, the need for presurgical and/or postsurgical ECMO support, pulmonary hypertension, liver-up phenomenon and lower relative fetal lung volume were associated with higher occurrences of chylothorax. After stepwise logistic regression, larger CDH defects (p < 0.0001) and the need for postsurgical ECMO (p = 0.0158) remained significant risk factors for CTX to occur (AUC 0.71). The same potential risk factors were used to assess their association with both presurgical and postsurgical pleural effusion. After stepwise logistic regression, only the need for presurgical ECMO remained significantly associated with presurgical PE (p < 0.01, AUC 0.65) and patch repair as the therapeutic intervention remained significantly associated with the occurrence of postsurgical PE (p < 0.0001, AUC 0.80). Patients with CTX had longer durations of both MV (p < 0.0001) and subsequent ventilatory assistance with spontaneous breathing (p = 0.0004), increased total lengths of hospitalization (p < 0.0001), increased durations of ECMO (p < 0.01) and increased incidences of CLD (p < 0.0001) compared to patients without CTX. No significant difference could be found for survival in both groups (p = 0.12). Conclusions: Our data suggest that the incidence of chylothorax is associated with large diaphragmatic defects, the need for postsurgical ECMO and the development of chronic lung disease, but not with survival.

Fetal surgery using fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: a single-center experience.

To provide a comprehensive report of the experience gained in the prenatal treatment of congenital diaphragmatic hernia (CDH) using fetoscopic endoluminal tracheal occlusion (FETO) following its implementation at a newly established specialized fetal medicine center. Mothers of fetuses with severe CDH were offered prenatal treatment by FETO. Between 2018 and 2021, 16 cases of severe CDH underwent FETO. The median gestational age (GA) at balloon insertion was 28.4 weeks (IQR 27.8-28.6). The median GA at delivery was 37 weeks (IQR 34.4-37.8). The survival rate was 8/16 cases (50%). None of the survivors required home oxygen therapy at 6 months of age. Comparison between the survivors and deceased showed that survivors had balloon insertion 1 week earlier (27.8 vs. 28.4 weeks, p = 0.007), a higher amniotic fluid level change between pre- to post-FETO (3.4 vs 1.3, p = 0.024), a higher O/E LHR change between pre- to post-FETO (50.8 vs. 37.5, p = 0.047), and a GA at delivery that was 2 weeks later (37.6 vs. 35.4 weeks, p = 0.032). The survival rate at 6 months of age in cases of severe CDH treated with FETO in our center was 50%. Our new fetal medicine center matches the performance of other leading international centers.

Cardiopulmonary Morbidity in Adults Born With Congenital Diaphragmatic Hernia.

Studies concerning cardiopulmonary outcomes of adults born with congenital diaphragmatic hernia (CDH) are sparse. Moreover, they don't include participants who have been treated with extracorporeal membrane oxygenation (ECMO) during the neonatal period. This study evaluated the cardiopulmonary morbidities in young adults born with CDH. We assessed 68 participants between the ages of 18 and 30 years. The assessment included auxology assessment, lung function tests, pulmonary imaging, cardiopulmonary exercise testing, and echocardiography. Lung function parameters in the overall group were significantly worse than normal values. Mean (SD) scores postbronchodilator forced expiratory volume in 1 second were -2.91 (1.38) in the ECMO-treated and -1.20 (1.53) in the non-ECMO-treated participants. Chest computed tomography scans showed mild to moderate abnormal lung structure in all ECMO-treated participants, and to a lesser extent in non-ECMO treated participants. A recurrent diaphragmatic defect was observed in 77% of the ECMO-treated group and in 43% of the non-ECMO-treated group. Except for 2 cases with acute symptoms, no clinical problems were noted in cases of recurrence. Cardiopulmonary exercise testing revealed mean (SD) percentage predicted peak oxygen consumption per kilogram of 73 (14)% and 88 (16)% in ECMO-treated and non-ECMO-treated participants, respectively. The mean (SD) workload was normal in the non-ECMO-treated group (111 [25]% predicted); in the ECMO-treated group, it was 89 (23)%. Cardiac evaluation at rest revealed no signs of pulmonary hypertension. In young adults who survived treatment of CDH, significant pulmonary morbidity, reduced exercise capacity, and frequent hernia recurrence should be anticipated. Lifelong follow-up care, with the emphasis on prevention of further decline, is to be recommended.

Maternal and Neonatal Outcomes After Ex-Utero Intrapartum Treatment for Congenital Diaphragmatic Hernia: A Case Series.

Despite advances in neonatal intensive care, fetuses with congenital diaphragmatic hernia (CDH) remain to have a poor prognosis. Exclusive postnatal treatment is inadequate in patients with moderate CDH (observed than expected lung-to-head ratio [O/E LHR] 26-45%) and can lead to respiratory failure at birth, requiring extracorporeal membrane oxygenation in 75% of cases. An ex-utero intrapartum treatment (EXIT) procedure may be beneficial in these cases, improving the fetal-neonatal transition. We review all pregnancies with fetal isolated left CDH with moderate O/E LHR delivered by EXIT in our center from January 2007 to December 2022. Maternal and neonatal variables were analyzed. As primary outcomes, we included neonatal survival and mortality rates, surgical and infectious complications, uterine scar dehiscence, and blood loss during EXIT. As secondary outcomes, we studied recurrences of the diaphragmatic defect, long-term evolution, subsequent pregnancies, and mode of delivery. A total of 14 patients were delivered by the EXIT procedure, with a neonatal survival rate of 85.7%. All these children had optimal physical and neurocognitive development and no pulmonary morbidity. We found no major complications and 7.1% of minor maternal complications. There were no cases of surgical wound infection or endometritis. The median decrease in hemoglobin during the EXIT procedure was 1.9 mg/dL, and only one case required postoperative transfusion. Two out of the 14 women became pregnant again, and both pregnancies were uneventful. In our series, the EXIT procedure allows for adequate airway management associated with a high neonatal survival rate in patients with moderate O/E LHR CDH, with a low rate of neonatal and maternal complications.

Placental fetal vascular malperfusion in congenital diaphragmatic hernia.

The success of in-utero or intrapartum treatment for congenital diaphragmatic hernia (CDH) can be impacted by poor placental function; however, this relationship has not yet been studied. To analyze placental histomorphology in CDH, the frequencies of 24 independent clinical and 48 placental phenotypes were compared. Slides from 103 CDH placentas (group 1) and 133 clinical umbilical cord (UC) compromise/anatomical UC abnormality placentas without CDH (group 2) were subjected to hematoxylin/eosin staining and CD34 immunostaining and then examined. CD34 immunostaining was performed to identify clustered distal villi with endothelial fragmentation of recent fetal vascular malperfusion (FVM). Cesarean delivery and ex utero intrapartum treatment were more common in group 1, but group 2 showed a higher frequency of statistically significant increases in other clinical phenotypes. The frequencies of large vessels and distal villous FVMs (clustered endothelial fragmentation by CD34 immunostaining, stromal vascular karyorrhexis, avascular, or mineralized villi) did not differ between the groups, but low-grade distal villous FVMs were statistically significantly more common in group 1 than in group 2, while high-grade distal villous FVMs were significantly more common in group 2 than group 1. Large vessel and distal villous FVMs were manyfold more common in both the CDH and UC compromise groups than in the general population. However, CDH placentas were more likely to show low-grade distal villous FVMs and less likely to show high-grade distal villous FVMs in UC compromise placentas. FVM of CDH may therefore be caused by a similar pathomechanism as that of UC compromise, resulting in impaired placental fetal blood outflow.

Successful extracorporeal membrane oxygenation for postoperative cardiopulmonary failure in newborns with congenital diaphragmatic hernia: case reports and literature reviews.

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. 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. ECMO is feasible for the treatment of postoperative cardiopulmonary failure in newborns with CDH.

PBPK-based dose finding for sildenafil in pregnant women for antenatal treatment of congenital diaphragmatic hernia.

Sildenafil is a potent vasodilator and phosphodiesterase type five inhibitor, commercially known as Revatio® and approved for the treatment of pulmonary arterial hypertension. Maternal administration of sildenafil during pregnancy is being evaluated for antenatal treatment of several conditions, including the prevention of pulmonary hypertension in fetuses with congenital diaphragmatic hernia. However, determination of a safe and effective maternal dose to achieve adequate fetal exposure to sildenafil remains challenging, as pregnancy almost always is an exclusion criterion in clinical studies. Physiologically-based pharmacokinetic (PBPK) modelling offers an attractive approach for dose finding in this specific population. The aim of this study is to exploit physiologically-based pharmacokinetic modelling to predict the required maternal dose to achieve therapeutic fetal exposure for the treatment congenital diaphragmatic hernia. A full-PBPK model was developed for sildenafil and N-desmethyl-sildenafil using the Simcyp simulator V21 platform, and verified in adult reference individuals, as well as in pregnant women, taking into account maternal and fetal physiology, along with factors known to determine hepatic disposition of sildenafil. Clinical pharmacokinetic data in mother and fetus were previously obtained in the RIDSTRESS study and were used for model verification purposes. Subsequent simulations were performed relying either on measured values for fetal fraction unbound (fu = 0.108) or on values predicted by the simulator (fu = 0.044). Adequate doses were predicted according to the efficacy target of 15ng/mL (or 38ng/mL) and safety target of 166ng/mL (or 409ng/mL), assuming measured (or predicted) fu values, respectively. Considering simulated median profiles for average steady state sildenafil concentrations, dosing regimens of 130mg/day or 150mg/day (administered as t.i.d.), were within the therapeutic window, assuming either measured or predicted fu values, respectively. For safety reasons, dosing should be initiated at 130mg/day, under therapeutic drug monitoring. Additional experimental measurements should be performed to confirm accurate fetal (and maternal) values for fu. Additional characterization of pharmacodynamics in this specific population is required and may lead to further optimization of the dosing regimen.

Mind the gap: identifying opportunities for fetal intervention for congenital diaphragmatic hernia in Ireland

Mind the gap: identifying opportunities for fetal intervention for congenital diaphragmatic hernia in Ireland

Surgical treatment of congenital diaphragmatic hernia in a single institution

BackgroundThis study aimed to evaluate the effectiveness of video-assisted thoracic surgery for the treatment of congenital diaphragmatic hernia (CDH) in a larger series compared with conventional open surgery. Additionally, we summarized the experience of thoracoscopic surgery in the treatment of CDH in infants.MethodsWe retrospectively analysed the clinical data of 109 children with CDH who underwent surgical treatment at the Department of Cardiothoracic Surgery of Children’s Hospital of Chongqing Medical University from January 2011 to January 2021. According to the surgical method, the children were divided into an open group (62 cases) and a thoracoscopy group (47 cases).Patients who underwent surgical correction had the diaphragmatic defect size graded (A–D) using a standardized system. We compared the operation time, intraoperative blood loss, postoperative mechanical ventilation time, postoperative hospital stay, postoperative CCU admission time and other surgical indicators as well as the recurrence rate, mortality rate and complication rate of the two groups of children.ResultsThe index data on the operation time, intraoperative blood loss, postoperative mechanical ventilation time, postoperative hospital stay and postoperative CCU admission time were better in the thoracoscopy group than in the open group. The difference between the two groups was statistically significant (P < 0.05). We compared the number of incision infections, lung infections, atelectasis, pleural effusion, and chylothorax between the two groups. There were more children in the open group than in the thoracoscopy group. The overall incidence of postoperative complications in the open group (51.61%) was higher than that in the thoracoscopy group (44.68%).The recurrence rate of the thoracoscopy group (8.51%) was higher than that of the open group (3.23%). In the open group, 7 patients died of respiratory distress after surgery, and no patients died in the thoracoscopy group.ConclusionsThoracoscopic surgery and open surgery can effectively treat CDH. Compared with conventional open surgery, thoracoscopy has the advantages of shorter operation time, less trauma, faster recovery and fewer complications. We believe that thoracoscopic surgery for type A/B diaphragmatic defect has certain advantages, but there is a risk of recurrence.

Attempt to shift from traditional to minimal invasive treatment of congenital diaphragmatic hernia at a low volume center

Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal morbidity and mortality. During the last 10-years, the progress on minimal invasive surgical techniques, has stimulated many Pediatric Surgical centers to switch from traditional open surgery to thoracoscopic or laparoscopic repair. We report our experience to convert treatment of CDH from traditional to minimal invasive. We have reviewed the charts of 10 neonates with diagnosed CDH over the period 2016–2021. On all of them, we collected pre, intra and post-operative information. Interestingly, none of neonates had prenatal diagnosis of CDH but this didn't impact on morbidity or mortality. None of them was extremely preterm or had major cardiac anomalies. Thoracoscopic or laparoscopic approach was attempted in 3 cases but only in one patient was successful. In the other 2 neonates we had to switch to open surgery because of anatomical conditions. In our opinion, the learning curve needed to achieve minimal invasive treatment of CDH is associated with a high conversion rate often, as a consequence of anatomical conditions.

5p deletion with congenital diaphragmatic hernia: a case report

Background5p deletion syndrome is known as cri-du-chat syndrome, but there are no reports on congenital diaphragmatic hernia complications associated with it.Case presentationA 28-year-old primigravida Japanese woman was referred for 5 mm of nuchal translucency. Fetal growth restriction was found at 20 weeks, and a left-sided congenital diaphragmatic hernia was diagnosed at 24 weeks. The karyotype of the fetus was diagnosed as 46, XX, del(5)(p14) and referred to our hospital. At 36 + 6 weeks, a 1524 g female infant was delivered after premature membrane rupture, with Apgar scores of 4 and 6 at 1 and 5 minutes, respectively. The baby was intubated immediately with sedation and muscle relaxation, after birth for initial treatment for congenital diaphragmatic hernia. The peripheral blood karyotype was consistent with the prenatal result. The infant was discharged alive, without any respiratory support, after the defect of the diaphragm was repaired.ConclusionThe results of this study may be helpful for antenatal genetic counseling.

Center Volume and Cost-Effectiveness in the Treatment of Congenital Diaphragmatic Hernia.

The care of patients with congenital diaphragmatic hernia (CDH) is expensive, yet little is known about variability in cost-of-care for these patients. The purpose of this study was to examine the cost-effectiveness of CDH treatment, comparing high- versus low-volume centers. This is a retrospective study of neonatal patients with CDH at U.S. hospitals using data from the Pediatric Health Information System database (2015-2018). Centers were considered high-volume if they had ≥10 patients with CDH for≥2y. Cost-effectiveness analysis was performed with cost per survivor as the outcome measure, and probabilistic sensitivity analysis was performed. A total of 1687 patients were included in the study. Overall mortality was 24.4%. Patients at high-volume centers had a longer mean length of stay (64d versus 48d for low-volume centers, P=0.0001) and higher extracorporeal life support (ECLS) rates (32% versus 24%, P=0.002). Risk-adjusted mortality did not differ between high- and low-volume centers (23.9% versus 25.9%, P=0.39), except when ECLS was involved (42% versus 56%, P=0.011). Costs were significantly higher at high-volume centers ($395,291±508,351 versus $255,074±308,802, P<0.0001). Survival status, ECLS use, operative repair, length of stay, high-volume status, and gestational age were identified as independent drivers of cost. On cost-effectiveness analysis, low-volume hospitals were more cost-effective in 95% of simulations. High-volume centers have substantially higher costs without an associated survival benefit and are less cost-effective than low-volume centers. Standardization of care is necessary to minimize the delivery of low-value care.

Основні чинники ризику повторних операцій у дітей з вродженими діафрагмальними грижами

Congenital diaphragmatic hernia (CDH) occurs with a frequency of 3.5 per 10,000 live births and occurs due to a fusion disorder of the closure of the pleuroperitoneal fold and transverse septum during 8-12 weeks of gestation. In an isolated variant of this pathology, mortality is quite high and there are a number of controversial issues. Even in the best specialized prenatal intensive care centers, the mortality rate reaches 30%. The use of new technologies in the treatment of CDH has increased the survival rate of patients, however, against this background, there is an increase in surgical problems associated with CDH. Purpose - to describe the structure and incidence of reoperations in children with CDH, depending on the access, identification of the main risk factors for reoperations, pathogenetic justification of optimal surgical access. Materials and methods. A retrospective cohort study of surgical correction of CDH in 104 infants who were operated on the basis of the NCH «OKHMATDYT» during 2000-2020 was conducted. To homogenize the group and to maximally exclude selection bias in order to identify risk factors, we include a group of patients with left-sided CDH in the study. Correction of the left-sided CDH was performed through the laparotomic approach in 51 patients (61%). Thoracotomy for left-sided EDH was used in 33 (39%) patients. Results. In the study group of patients, 14 (16.7%) reoperations were performed in this group at different times of the long-term period. The indications for reoperations were: adhesive intestinal obstruction - 3 (21.5%), inc. strangulated intestinal obstruction with bowel necrosis - 2 (14%), obstruction caused by malrotation - 1 (7%), gastroesophageal reflux - 4 (29.5%), recurrent hernia - 2 (14%), pectus excavatum - 1 (7%), spleen torsion - 1 (7%). The number of reoperations in the study group during a certain observation period was slightly higher in the thoracotomy group (18% versus 14%, p=0.80). More than half of repeated interventions were associated with acute intestinal obstruction, more often after laparotomy (35.7 versus 7%; p=0.16). In this group, 5 reoperations were performed, the cause of which was intestinal obstruction, in contrast to the thoracic group, where one patient was operated for malrotation. Reoperations for recurrent diaphragmatic hernia occurred only in the thoracotomy group in one patient with agenesis of the left diaphragm dome. Early postoperative mortality was found slightly higher in the laparotomy group (27.4% versus 18.1%; p=0.167). Conclusions. The optimal method of surgical treatment of large defects and agenesis of the dome is surgical correction of the diaphragm through a thoracotomy approach using a synthetic patch and thoracalization of the abdominal cavity. The abdominal approach has a high risk of reoperations, which is associated with the development of the adhesive process and the likelihood of the formation of ventral hernias due to viscero-abdominal imbalance. The indications for the use of thoracic access and patches for plasty of the diaphragm defect should be expanded regardless of the side of the lesion and the size of the defect. The main factors determining the risk of recurrent CDH are the size of the hernial defect and the method of diaphragm correction. The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local ethics committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: congenital diaphragmatic hernia, reoperations, intestinal obstruction, gastroesophageal reflux, recurrence, malrotation, thoracatomy, laparotomy.

Quality of life in adolescents who underwent congenital diaphragmatic hernia surgical repair.

The advances made in the surgical and postnatal treatment of congenital diaphragmatic hernia (CDH) have considerably improved patient survival, but morbidity remains significant. The objective of this study was to analyze the effect these sequels have on the health-related quality of life (HRQL) of adolescents and young adults who have survived CDH, and to compare it with that of the general population. A transversal descriptive study of patients diagnosed with CDH in our institution from 1997 to 2004 was carried out. Survival, location, hernia size, herniated organs, need for extracorporeal membrane oxygenation, and mechanical ventilation time were analyzed. In addition, a comparative study of the current HRQL of survivors was conducted using the SF-36 survey (36-Item Health Survey Short Form), which assessed physical function, physical role, body pain, general health, vitality, social function, emotional role, and mental health. Data of 24 healthy adolescents was used as a control group. Of the 29 survivors (70.7%), 21 were successfully contacted, and 16 responded to the survey. They all claimed their overall quality of life was good or very good. The group of adolescents who underwent CDH surgical repair had better results in the vitality (p= 0.001) and mental health (p<0.05) areas, but the overall HRQL score and the remaining health areas were similar. No significant differences were found regarding diaphragmatic size or need for ECMO. According to adolescent survivors who underwent CDH surgical repair, their quality of life is similar to that of other individuals of their age. Our results are encouraging and may prove useful for future parents of CDH patients.

Ретроспективный анализ результатов диагностики и лечения врожденной диафрагмальной грыжи плода, по данным перинатального консилиума

Study Objective: Analysis of the detection of congenital diaphragmatic hernia (CDH) in the fetus at the antenatal stage and perinatal outcomes of newborns with CDH in the Russian Federation in a sample of women who applied to the perinatal consultation of the National Medical Research Center for Obstetrics, Gynecology and Perinatology named after academician V.I. Kulakov. Study Design: Retrospective study. Materials and Methods. The study included 235 cases of CDH in fetuses and newborns were selected by the federal perinatal consultation of the National Medical Research Center for Obstetrics, Gynecology and Perinatology named after academician V.I. Kulakov. The following were retrospectively analyzed: medical and social characteristics of pregnant women, results of ultrasound and MRI examination of fetuses, delivery features and perinatal outcomes. Study Results. The number of women with fetal CDH in the perinatal consultation of the Center continues to grow every year. In 3/4 of the treated patients, fetal CDH was detected in the 2nd trimester of pregnancy, which exceeds the European level of detection. But against this background, an extremely low level of invasive prenatal diagnosis was noted. Detection using ultrasound and MRI of liver infection in the chest, including a group with a prognosis of high neonatal survival of 2.2–4 times. The perinatal mortality rate with CDH in the Centers detection is 43.4%. The most common method of delivery is the natural birth. The optimal term of delivery with the least increase in postnatal loss should be considered the period of 38–39 weeks of pregnancy. Forecast of survival of newborns. At the same time, the Apgar score does not have statistical indicators of differences in surviving and deceased newborns, regardless of the method of delivery, which is associated with a violation of child development. Conclusion. The level of detection of CDH and perinatal mortality of newborns is similar to the data of foreign perinatal centers that provide assistance to mothers and their newborns. Increasing the survival rate of newborns with CDH is possible with the development and implementation of an integrated system for diagnosing the severity of CDH, as this will open up the possibility of a personalized approach to the treatment of CDH. Keywords: congenital diaphragmatic hernia, perinatal mortality, perinatal consultation, pulmonary hypoplasia, cardiac compression.

Antithrombin III infusion improves anticoagulation in congenital diaphragmatic hernia patients on extracorporeal membrane oxygenation.

Achieving effective anticoagulation during neonatal extracorporeal membrane oxygenation (ECMO) without increasing the risk of hemorrhage remains challenging. The use of antithrombin III (AT-III) for this purpose has been examined, but studies have been limited to intermittent bolus dosing. We aimed to evaluate the efficacy and safety of an institutionally developed AT-III continuous infusion protocol in neonates receiving ECMO for the treatment of congenital diaphragmatic hernia (CDH). In this single center, retrospective study, all neonates with a CDH who received ECMO support during the study period were included. Data on anticoagulation labs and therapy, life-threatening bleeding, and circuit changes were analyzed. Eleven patients were divided into two groups: patients with AT-III continuous infusion (n = 5) and without (n = 6). There were no differences in the gestational age (p = 0.29), sex (p = 1.00), ECMO duration (p = 0.59), or initial AT-III levels (p = 0.76) between groups. Patients in the AT-III infusion group had on average 18.5% higher AT-III levels (p < 0.0001). Patients receiving continuous AT-III infusions spent a significantly higher percentage of ECMO time within the therapeutic range, measured using anti-Factor Xa levels (64.9±4.2% vs. 29.1±8.57%, p = 0.008), and required fewer changes to the heparin infusion rate (6.48±0.88 vs 2.38±0.36 changes/day changes/day, p = 0.005). Multivariate analysis revealed continuous infusion of AT-III did not increase the rate of intracranial or surgical bleeding (p = 0.27). AT-III as a continuous infusion in CDH neonates on ECMO provides a decreased need to modify heparin infusion and more consistent therapeutic anticoagulation without increasing the risk of life-threatening bleeding.

Managementul obstetrical și perinatal actual al herniei diafragmatice congenitale

Congenital diaphragmatic hernia is a severe condition with an etiology and pathogenesis still been developed; also, the involvement of genetic and environmental factors in the occurrence of this malformation is under study. Morbi-mortality in these patients is high, and quality of life is diminished. Under these circumstances, careful follow-up of the pregnancy and multidisciplinary management after birth is needed to diagnose the malformation as early as possible and adapt the therapeutic plan to the particularities of each case. In conclusion, despite several research data in the literature concerning this topic, the management of newborn babies with congenital diaphragmatic hernia remains a severe challenge. The treatment of congenital diaphragmatic hernia remains the prerogative of overqualified teams.

Clinical guidelines for the treatment of congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm in which abdominal organs herniate through the defect into the thoracic cavity. The main pathophysiology is respiratory distress and persistent pulmonary hypertension because of pulmonary hypoplasia caused by compression of the elevated organs. Recent progress in prenatal diagnosis and postnatal care has led to an increase in the survival rate of patients with CDH. However, some survivors experience mid- and long-term disabilities and complications requiring treatment and follow-up. In recent years, the establishment of clinical practice guidelines has been promoted in various medical fields to offer optimal medical care, with the goal of improvement of the disease' outcomes, thereby reducing medical costs, etc. Thus, to provide adequate medical care through standardization of treatment and elimination of disparities in clinical management, and to improve the survival rate and mid- and long-term prognosis of patients with CDH, we present here the clinical practice guidelines for postnatal management of CDH. These are based on the principles of evidence-based medicine using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. The recommendations are based on evidence and were determined after considering the balance among benefits and harm, patient and society preferences, and medical resources available for postnatal CDH treatment.

ECMO in Neonates with Congenital Diaphragmatic Hernia: The Experience of a Portuguese ECMO Referral Center

The use of extracorporeal membrane oxygenation (ECMO) is considered by many authors as one of the most important technological advances in the care of newborns with congenital diaphragmatic hernia. The main objective of this study was to report the experience of a Portuguese ECMO center in the treatment of congenital diaphragmatic hernia. Descriptive retrospective study of newborns with congenital diaphragmatic hernia requiring ECMO support in a Pediatric Intensive Care Unit from January 2012 to December 2019. Data collection using the Extracorporeal Life Support Organization registration and unit data base. Fourteen newborns were included, all with left congenital diaphragmatic hernia, in a total of 15 venoarterial ECMO cycles. The median gestational age was 38 weeks and the median birth weight was 2.950 kg. Surgical repair was performed before entry into ECMO in six, during in seven and after in one newborn. The average age at placement was 3.3 days and the median cycle duration was 16 days. Prior to ECMO, all newborns had severe hypoxemia and acidosis despite optimized ventilatory support, with nitric oxide and inotropic therapy. After 24 hours on ECMO, there was correction of acidosis, improvement of oxygenation and hemodynamic stability. All cycles presented mechanical complications, the most frequent being the presence of clots in the circuit. The most frequent physiological complications were hemorrhagic and embolic (three newborns suffered an ischemic stroke during the cycle). Five newborns (35.7%) died, all associated with complications (two strokes, two massive bleedings and one accidental decannulation). Chronic lung disease, poor weight gain and psychomotor developmental delay were the most frequent long-term morbidities. Despite technological advances in respiratory care and improved safety of the ECMO technique, the management of these newborns is complex and there are still several open questions, including the appropriate selection of patients, the best approach and time for surgical correction, and the treatment of pulmonary hypertension in the presence of persistent fetal shunts. Survival rate was higher than reported in 2017 Extracorporeal Life Support Organization report (64% versus 50%). Mechanical and hemorrhagic complications were very frequent.