Incidence Of Congenital Diaphragmatic Hernia Research Articles

Survival and Predictors of Mortality of Congenital Diaphragmatic Hernia in Newborns at a Tertiary Care Hospital in Saudi Arabia.

Background Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered an emergency that needs urgent intervention to prevent further complications or death. Our study aimed to estimate survival and evaluate predictors of mortality in newborns with CDH using available prediction tools in the literature. Methods This retrospective cohort study included neonates with CDH in King Abdulaziz Medical City (KAMC), Jeddah, from 2000 to 2021. Prevalence, demographics, and clinical characteristics were compared between surviving and deceased infants. C-statistics wereused to measure the area under the curve for the prenatal and postnatal predictor tools, and a p-value of <0.05 was considered significant. Results Between 2000 and 2021, 45 neonates with CDH were included (six per 10,000 inborn live births). The mortality rate was 51.1%. The differences in demographics were not significant among surviving and deceased patients. One prenatal predictor tool, the lung-to-head ratio, was found to be significant; in addition, three postnatal predictor tools of mortality, SNAP-II, CDHSG-probability survival, and Brindle Score, had the highest concordance (C) statistics of 0.8, 0.79, and 0.8, respectively. Conclusion Although the incidence of CDH was found to be higher in our study compared to global statistics, our mortality rates correspond with international figures. The most significant differences between predictors and prediction models of mortality were lung-to-head ratio prenatally, SNAP-II, CDHSG-probability survival, and Brindle Score postnatally. Further multicentered studies are recommended with a larger sample size.

Intra-Amniotic Sildenafil and Rosiglitazone Late in Gestation Ameliorate the Pulmonary Hypertension Phenotype in Congenital Diaphragmatic Hernia

BackgroundPulmonary hypertension remains difficult to manage in congenital diaphragmatic hernia (CDH). Prenatal therapy may ameliorate postnatal pulmonary hypertension. We hypothesized that intra-amniotic (IA) injection of either sildenafil, a phosphodiesterase 5 inhibitor, or rosiglitazone, a PPAR-γ agonist, or both late in gestation would decrease the detrimental pulmonary vascular remodeling seen in CDH and improve peripheral pulmonary blood flow. MethodsPregnant rats were gavaged with nitrogen on embryonic day (E) 9.5 to induce fetal CDH. Sildenafil and/or rosiglitazone were administered to each fetus via an intra-amniotic injection after laparotomy on the pregnant dam at E19.5, and fetuses delivered at E21.5. Efficacy measures were gross necropsy, histology, peripheral blood flow assessment using intra-cardiac injection of a vascular tracer after delivery, and protein expression analysis. ResultsIntra-amniotic injections did not affect fetal survival, the incidence of CDH, or lung weight-to-body weight ratio in CDH fetuses. IA sildenafil injection decreased pulmonary vascular muscularization, and rosiglitazone produced an increase in peripheral pulmonary blood flow distribution. The combination of sildenafil and rosiglitazone decreased pulmonary artery smooth muscle cell proliferation. These intra-amniotic treatments did not show any negative effects in either CDH fetuses or control fetuses. ConclusionIA injection of sildenafil and rosiglitazone late in gestation ameliorates the pulmonary hypertensive phenotype of CDH and may have utility in clinical translation. Level of EvidenceNot applicable.

Congenital diaphragmatic hernia: a case series in tertiary care centre

Congenital diaphragmatic hernia (CDH) is the presence of a defect in the diaphragm that allows the herniation of the abdominal viscera into the thorax. The mortality is the result of persistent pulmonary hypertension and respiratory insufficiency due to abnormal pulmonary vessels and hypoplastic lungs. The incidence of congenital diaphragmatic hernia is 1 in 2500 births. The etiology of congenital diaphragmatic hernia remains unclear. It is thought to be multifactorial with genetic, environmental, and/or nutritional factors playing a role. CDH can be an isolated anomaly or associated with anomalies in other organ systems. The most frequent aneuploidies associated with CDH include trisomy 18, trisomy 13, trisomy 21, and less frequently Turner syndrome (45, X). Exposure to teratogenic agents like mycophenolate mofetil, allopurinol, and lithium during pregnancy is reported to be associated with CDH. Recently, studies indicate disturbances in the retinoid-signaling pathway as a potential cause of CDH. This case series aims to pull importance of a viable protocol for antenatal evaluation of CDH, and meanwhile to underline the importance of a good interdisciplinary antenatal evaluation of these cases for the fetal outcome.

Epidemiology of congenital diaphragmatic hernia, esophageal atresia, and gastroschisis in South Brazil

Background: Birth defects remain a major contributor to infant mortality and lifelong disabilities. The epidemiology of congenital abnormalities varies around the world and little information is available from Latin America. Methods: This is an epidemiological, descriptive, cross-sectional study with data from the Department of Informatics of the Unified Health System (DATASUS) on Congenital diaphragmatic hernia (CDH), esophageal atresia (EA) and gastroschisis (GS) in South Brazil from 2009 to 2019. Results: The incidence of CDH is 0.93 cases, while EA is 0.47 and GS is 2.87, all per 10,000 live births. There is an association between all the malformations and premature birth, cesarean delivery, low birth weight, and low Apgar scores. Both EA and GS are associated with maternal age, EA with older, and GS with younger mothers. While EA is associated with multiple pregnancies, GS is associated with fewer years of maternal formal education, single parenting, and a lower number of prenatal consultations. CDH is associated with the male sex and black ethnicity. Conclusion: This large population-based study estimates the prevalence and demographic factors associated with CDH, EA, and GS, and extends the limited descriptive epidemiologic information available.

Epidemiology and One-Year Follow-Up of Neonates with CDH-Data from Health Insurance Claims in Germany.

Congenital diaphragmatic hernia (CDH) is a major congenital malformation with high mortality. Outcome data on larger unselected patient groups in Germany are unavailable as there is no registry for CDH. Therefore, routine data from the largest German health insurance fund were analyzed for the years 2009–2013. Main outcome measures were incidence, survival and length of hospital stay. Follow-up was 12 months. 285 patients were included. The incidence of CDH was 2.73 per 10,000 live births. Overall mortality was 30.2%. A total of 72.1% of the fatalities occurred before surgery. Highest mortality (64%) was noted in patients who were admitted to specialized care later as the first day of life. Patients receiving surgical repair had a better prognosis (mortality: 10.8%). A total of 67 patients (23.5%) were treated with ECMO with a mortality of 41.8%. The median cumulative hospital stay among one-year survivors was 40 days and differed between ECMO- and non-ECMO-treated patients (91 vs. 32.5 days, p < 0.001). This is the largest German cohort study of CDH patients with a one-year follow-up. The ECMO subgroup showed a higher mortality. Another important finding is that delayed treatment in specialized care increases mortality. Prospective clinical registries are needed to elucidate the treatment outcomes in detail.

Diagnostic challenges in late presenting diaphragmatic hernia

Background: The incidence of congenital diaphragmatic hernia (CDH) has been reported to be 1 in 2000-5000 newborns. The late presentation can occur in 5-30% of all cases and it can be harder to diagnose, as symptoms are usually vague and non-specific. Methods: We performed a retrospective review of chart files of all patients admitted to our hospital with the diagnosis of congenital diaphragmatic hernia between 2010 and 2021. Result: Of the ninety-two cases of CDH treated in our hospital between 2010 and 2021, twenty-six (28%) were late presenting. In the late-presenting CDH group, we found a slight female preponderance, with fifteen female patients (58%) and eleven male patients. A posterolateral left defect in the diaphragm was noted in fifteen cases (58%), a right defect was noted in just one case (4%) and ten patients (38%) were diagnosed with an anterior diaphragmatic hernia. The age at diagnosis varied from 2 months to 14 years. Associated anomalies were noted in just four cases. In all cases, a thoracoabdominal radiograph was performed and ten patients also had a CT scan. Primary repair was performed in 25 of the 26 patients. The mortality rate in our study population was 11%, we reported 3 cases with unfavorable evolution. Conclusion: CDH presenting beyond the neonatal period is a usual finding and it represents a true diagnostic challenge.

Association of congenital diaphragmatic hernia with extrapulmonary sequestration: A case report

Pulmonary sequestration (PS) is a non-functioning lesion of lung tissue which lacks communication with the tracheobronchial tree. The incidence of congenital diaphragmatic hernia (CDH) is about 1 in 2500 newborns. While the association of PS with CDH is as high as 30%–40%, the prognosis of their coexistence is unknown. Awareness of the association between pulmonary sequestration with CDH and recognition of the sequestration at the time of the surgery for CDH repair, will prevent the need of a later second operation for a mediastinal mass.

Incidence of congenital diaphragmatic hernia in Olmsted County, Minnesota: a population-based study

Objective: The rate of congenital diaphragmatic hernia (CDH) varies, but most reports estimate the incidence to be less than three per 10,000 births. Our objective was to document the incidence of CDH in a geographically well-defined population using available resources for highly accurate incident case ascertainment.Methods: We ascertained CDH cases in Olmsted County, Minnesota, United States of America, from 1981 through 2014 using resources of the Rochester Epidemiology Project. Overall and sex-specific incidence rates were calculated, and hospital survival was assessed.Results: A total of 26 incident CDH cases were identified; the overall incidence of 3.6 (95% CI, 2.2–5.0) per 10,000 did not differ significantly over the 34-year study period (p = .28). The estimated incidence was 4.3 (95% CI, 2.2–6.5) for male infants and 2.9 (95% CI, 1.1–4.6) for female infants. The percentage of cases diagnosed prenatally was 33% from 1981 through 2000 and 50% from 2001 through 2014. The rate of survival to discharge in the two periods was 50% and 88%.Conclusion: The incidence of CDH in Olmsted County exceeds the majority of published rates, which most likely can be attributed to our comprehensive case ascertainment.

Seasonality, Epidemiology and Outcome of Congenital Diaphragmatic Hernia in South West of Iran.

Introduction :The pathogenesis of congenital diaphragmatic hernia (CDH) is not clear. Risk factors including environmental factors have been implicated in the pathogenesis of few congenital anomalies. We aimed to assess the effect of season on the incidence of CDH and mortality rate in the southwest of Iran.Material and Methods :In this retrospective study, the records of 60 patients with CDH who were admitted at Neonatal Intensive Care Unit (NICU) of Imam Khomeini Hospital of Ahvaz, Iran were evaluated.Results :Assuming that all the neonates born with CDH in the region reach this hospital, overall CDH prevalence rate was 1.09 per 10 000 total births. Conceptions in spring and summer in this region had statistically significantly higher incidence of CDH. Survival rate in the series was 41.6%.Conclusion :Seasonal variation has impact on the incidence of CDH. Mortality rate in neonates with CDH is still very high.

Role of Ultrasound-Based Prenatal Prediction of Pulmonary Function in Congenital Diaphragmatic Hernia: Does It Have Prognostic Significance Postnatally?

The incidence of congenital diaphragmatic hernia (CDH) in India is 1 in 1000. About 60% of these are isolated, and the survival prognosis in them depends upon the quantum of contralateral functional lung. Out of the various pulmonary and extrapulmonary sonological predictors, observed to expected lung-head ratio (O/E LHR) is an efficient gestation-independent predictor of pulmonary function. This study was carried out to see the correlation of this prenatal predictor with the postnatal outcome depending on the pulmonary function. This study was carried out at Apollo Center of Fetal Medicine, New Delhi, from January 2009 to December 2015. A total of 14 fetuses with isolated left-sided CDH were included. The contralateral lung area was measured in 2D transverse view of the thorax at the level of four-chamber view of the heart by tracing method. The obtained value (square mm) was then divided by the expected mean lung area at that gestation and multiplied with 100 to express O/E LHR as percentage. These were then classified as severe (O/E LHR <25%), moderate (25-45%) or mild (>45%) varieties of CDH. The parents to be were counselled for termination or continuation of pregnancy based on severity of CDH and total lung area. The patients were followed up for obstetrical and neonatal outcome till the time of first postoperative visit (diaphragmatic repair). The survival correlation in mild cases was 100% (n=5 out of 5) and 50% in moderate cases (n=2 out of 4), and both severe cases were terminated. There was a significant difference (p<0.01) in the survival rate in the mild versus severe cases. The prenatal predictor for postnatal pulmonary function correlates well with the neonatal outcome and hence is an important tool in prenatal counseling and triaging those who require termination of pregnancy versus expectant management. An obstetrician who is a first point of contact to the pregnant women can understand this and use it for counseling and differentiating the patients who need termination with regard to CDH.

Epidemiology and Outcomes of Congenital Diaphragmatic Hernia in Croatia: A Population-Based Study.

Outcomes of neonates with congenital diaphragmatic hernia (CDH) are variable; reports are frequently limited to the experience of single tertiary care centres-a possible source of bias. Population-based studies decrease survivor bias and provide additional insight into this high-mortality condition. The objective of this study was to examine the incidence and outcomes of CDH in Croatia. All cases of CDH in Croatia from 2001 through 2013 were ascertained from public health records. Overall and sex- and region-specific incidence rates were calculated, and characteristics associated with 1-year survival were assessed. We identified 145 cases of CDH during the study period, for an incidence of 2.67 per 10000 total births. The incidence did not differ by calendar year (P=0.38) or geographic region (P=0.67). There was a slightly higher incidence among males (rate ratio, 1.37, 95% CI 0.99, 1.91). The 1-year survival rate was 33.1% for the entire cohort and 47.9% for liveborns who received any treatment at an intensive care unit. From multivariable analysis, survival was decreased in neonates with left CDH, liver up (odds ratio 0.1, 95% CI, 0.03, 0.4) and increased when treated in a centre with higher case volume (odds ratio 12.8, 95% CI, 2.2, 72.1). The incidence of CDH in Croatia is within the range of previous reports. Survival was substantially higher in neonates treated in a centre with higher case volume, which suggests that centralisation of medical care for CDH may be warranted in Croatia.

Congenital Diaphragmatic Hernia with Delayed Presentation.

Congenital diaphragmatic hernia (CDH) is caused due to abnormal formation of the muscular parts of diaphragm. The incidence of CDH in common births ranges from 1/25000 to 1/30000. Pulmonary hypoplasia and pulmonary hypertension are factors that associate with the increase of mortality and morbidity due to CDH. We presented a 68-year-old Iranian woman with abdominal pain and tenderness in right upper quadrant who was diagnosed as having CDH. The disease was detected using chest X-ray and chest and abdomen sonography and confirmed with chest and abdomen CT scan with and without oral contrast. A defect was revealed in posterolateral right diaphragm with omentum and transverse colon herniated through it. Right posterolateral thoracotomy was performed to cure the disease. CT and CXR were the two useful methods in diagnosis of CDH in this patient, although CDH detection prior to surgery is too challenging because of rare cases and different types of CDH. In order to improve clinical cares in adult CDH patients, investigating more cases and long term follow-up are recommended.

Right sided congenital diaphragmatic hernia: A rare case report

A diaphragmatic hernia is defined as a communication between abdominal and thoracic cavity with or without abdominal contents in the thorax. The true incidence of Congenital diaphragmatic hernia is 1 in 5000 live births while right side diaphragmatic hernia (15%) is rare comparing to left side diaphragmatic hernia (85%) because liver plugs the opening. Congenital diaphragmatic hernia typically refers to Bochdalek form, other forms are rarer. Despite advances in neonatal intensive care, congenital diaphragmatic hernia is associated with high mortality and morbidity. The posterolateral right congenital DH is a rare diaphragmatic defect. Females are twice affected than that of males. The symptoms are non characteristic and patients with this disease maybe without symptoms for a long period. The main tool for diagnosis of congenital DH is radiography. Surgical correction is required.

Use of high-frequency oscillatory ventilation with inhaled nitric oxide in congenital diaphragmatic hernia, omphalocele, and patent ductus arteriosus.

Use of high-frequency oscillatory ventilation with inhaled nitric oxide in congenital diaphragmatic hernia, omphalocele, and patent ductus arteriosus.

Incidence, predictors and outcomes of congenital diaphragmatic hernia: a population-based study of 32 million births in the United States

Objectives: To evaluate the incidence, risk factors and neonatal outcomes associated with a congenital diaphragmatic hernia (CDH).Study design: We conducted a population-based cohort study using the CDC’s Linked Birth-Infant Death and Fetal Death data files on all births and foetal deaths in USA between 1995 and 2002. We estimated the yearly incidence of CDH and measured its adjusted effect on various outcomes using unconditional logistic regression analysis.Results: About 32 145 448 births during the 8-year study period met the study’s inclusion criteria. The incidence of CDH was 1.93/10 000 births. Risk factors for the development of CDH included foetal male gender [OR 1.12, 95% CI: 1.06, 1.17], maternal age beyond 40 [OR 1.51, 95% CI: 1.26, 1.80], Caucasian ethnicity [OR 1.15, 95% CI: 1.10, 1.21], smoking [OR 1.34, 95% CI: 1.22, 1.46] and alcohol use during pregnancy [OR 1.37, 95% CI: 1.05, 1.79]. As compared to foetuses with no CDH, foetuses with CDH were at an increased risk of preterm birth [OR 2.90, 95% CI: 2.72, 3.11], intrauterine growth restriction [OR 3.84, 95% CI: 3.51, 4.18], stillbirth [OR 9.65, 95% CI: 8.20, 11.37] and overall infant death [OR: 94.80, 95% CI: 88.78, 101.23]. The 1-year mortality was 45.89%.Conclusion: Congenital diaphragmatic hernia is strongly associated with an increased risk of adverse pregnancy, foetal and neonatal outcomes. These findings may be helpful in counselling pregnancies affected by CDH, and may aid in the understanding of the burden of this condition at the public health level.

Epidemiology and outcome of congenital diaphragmatic hernia in a tertiary care university hospital: 10 Years′ Experience

Background: Congenital diaphragmatic hernia (CDH) is a major birth defect, which continues to be associated with significant rate of morbidity and mortality. CDH-local studies are limited. The objective of this article is to determine the epidemiology and demographics of CDH; and if the introduction of nitric oxide has influenced the outcome. Materials and Methods: A retrospective 10-year medical records review of neonates with CDH admitted to the neonatal intensive care unit (NICU) of a tertiary care hospital. Results: A total of 29 infants had been admitted to the NICU with CDH between January 2001 and December 2010. The mean gestational age was 38.58 ΁ 2.6 weeks and the mean birth weight was 2821 ΁ 682 g. The incidence of CDH among the inborn infants was 1/250 live births (P 0.05. Conclusion: The incidence of CDH was found much higher than the reported cases in literature; and although the mortality rate has improved after the introduction of iNO, the difference was not statistically significant. Both interesting findings necessitate further studies.

613 Congenital Diaphragmatic Hernia Surveillance in Ireland

IntroductionThe incidence of Congenital Diaphragmatic Hernia (CDH) varies from 1 in 2,000 to 15,000 births per annum. Due to the legislation prohibiting termination of any pregnancy in the Republic of...

Antenatal Sildenafil Treatment Attenuates Pulmonary Hypertension in Experimental Congenital Diaphragmatic Hernia

Lung hypoplasia and persistent pulmonary hypertension of the newborn limit survival in congenital diaphragmatic hernia (CDH). Unlike other diseases resulting in persistent pulmonary hypertension of the newborn, infants with CDH are refractory to inhaled nitric oxide (NO). Nitric oxide mediates pulmonary vasodilatation at birth in part via cyclic GMP production. Phosphodiesterase type 5 (PDE5) limits the effects of NO by inactivation of cyclic GMP. Because of the limited success in postnatal management of CDH, we hypothesized that antenatal PDE5 inhibition would attenuate pulmonary artery remodeling in experimental nitrofen-induced CDH. Nitrofen administered at embryonic day 9.5 to pregnant rats resulted in a 60% incidence of CDH in the offspring and recapitulated features seen in human CDH, including structural abnormalities (lung hypoplasia, decreased pulmonary vascular density, pulmonary artery remodeling, right ventricular hypertrophy), and functional abnormalities (decreased pulmonary artery relaxation in response to the NO donor 2-(N,N-diethylamino)-diazenolate-2-oxide). Antenatal sildenafil administered to the pregnant rat from embryonic day 11.5 to embryonic day 20.5 crossed the placenta, increased fetal lung cyclic GMP and decreased active PDE5 expression. Antenatal sildenafil improved lung structure, increased pulmonary vessel density, reduced right ventricular hypertrophy, and improved postnatal NO donor 2-(N,N-diethylamino)-diazenolate-2-oxide-induced pulmonary artery relaxation. This was associated with increased lung endothelial NO synthase and vascular endothelial growth factor protein expression. Antenatal sildenafil had no adverse effect on retinal structure/function and brain development. Antenatal sildenafil improves pathological features of persistent pulmonary hypertension of the newborn in experimental CDH and does not alter the development of other PDE5-expressing organs. Given the high mortality/morbidity of CDH, the potential benefit of prenatal PDE5 inhibition in improving the outcome for infants with CDH warrants further studies.

Effects of estrogen on lung development in a rat model of diaphragmatic hernia

Purpose The study aimed to observe the influence of estradiol on rat models with congenital diaphragmatic hernia (CDH) and understand the potential mechanism. Methods Eleven pregnant female Sprague-Dawley rats were randomly divided into 3 groups on day 9.5 of gestation: group C (n = 2) was administered 2 mL of olive oil, whereas group N (n = 3) and group E (n = 6) were administered 200 mg of nitrofen. Antenatal estradiol was given subcutaneously to group E on days 18.5, 19.5, and 20.5 of gestation. Histologic evaluations, incidence of CDH, and the immunoreactivity of transforming growth factor (TGF)- β1 in lung were observed. In addition, the mRNA levels of TGF- β1, type I TGF- β receptor (T βRI), and type II TGF- β receptor (T βRII) were determined. Results Histologically, the lungs of group N fetuses were hypoplastic compared with those of group C and had thick-walled septa with poorly developed saccules. Group E showed improved mesenchymal differentiation with well-developed saccules. There was no significant difference between the incidence of CDH in group N and that in group E. The expression of TGF- β1 in lung tissue and arterioles in group N were significantly higher than those in group C and E. Moreover, relative mRNA expression levels of TGF- β1 and T βRI in group N were markedly higher than those in group C, whereas those in group E were significantly decreased compared with group N. Conclusions Estradiol can promote lung development in rats with CDH. The down-regulation of TGF- β1 and its signaling pathway may play a role in this effect.

964 Outcome of Liveborn Infants with Congenital Diaphragmatic Hernia in a Tertiary Neonatal Unit

Background and aims: The incidence of congenital diaphragmatic hernia (CDH) is approximately 1 in 3000 with reported survival of 60-70%[1]. The aim of this study was to review management and outcome of all live born babies with CDH delivered at Birmingham Women's Hospital from January 2000-December 2009. Methods: Retrospective case-note review of babies with CDH identified by their clinical diagnosis code. Trust audit department approval was obtained. Results: 60 live newborns with CDH were identified. Median gestational age at birth was 39 weeks (range 33-42 weeks). The median birth weight was 2970g (range 1080-4090g). There were 35 boys (58%). 53 cases were left sided. 3 babies died in delivery suite despite active resuscitation. 18 babies died on the neonatal unit despite full intensive care. 6 were referred for ECMO(5 of these survived). 33 babies were transferred to PICU but 3 babies died before surgery (1 unknown). 29 babies had surgery and 2 died post-surgery. The 30 day surgical survival rate was 93%. 32 babies are alive to date (1 unknown). Conclusion: In our experience, 35% of babies died in the immediate neonatal period. Transfer for ECMO has been a recent practice with 83% survival. 30 day surgical survival was 93%. However, the overall survival of a live born baby with CDH was 53%. Therefore, when counselling parents in the antenatal period, it is important to quote all figures rather than surgical survival alone.