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A systematic review and meta-analysis of surgical morbidity of primary versus patch repaired congenital diaphragmatic hernia patients.

ABSTRACT: Large studies comparing the surgical outcome of primary versus patch repair in congenital diaphragmatic hernia (CDH) patients are rare. This study aims to evaluate the incidence of surgical complications in both types of CDH repair. PubMed, EMBASE, Cochrane and Web of Science were searched for peer-reviewed articles. Studies on CDH between 1991 and August 2020 were systematically screened and meta-analyses were performed. Primary outcomes of this review were: haemorrhage, chylothorax, recurrences and small bowel obstruction (SBO). A total of 6436 abstracts were screened, after which 25 publications were included (2910 patients). Patch repaired patients have a 2.8 times higher risk on developing a recurrence (20 studies) and a 2.5 times higher risk on developing a chylothorax (five studies). Moreover, they have a two times higher risk on developing a SBO. No studies could be included that evaluated the incidence of surgical haemorrhage between these patients. Although the quality of the studies was relatively low, patch repaired patients have a higher risk on developing a recurrence, chylothorax and small bowel obstruction. Large prospective studies are required to adjust for severity of disease, to reveal the true causative factors in order to minimize the risk on these surgical complications in both types of patients.

SUBMITTER: Heiwegen K

PROVIDER: S-EPMC8209041 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Comprehensive meta-analysis of surgical procedure for congenital diaphragmatic hernia: thoracoscopic versus open repair.

Project description:PurposePrevious studies have shown a higher recurrence rate and longer operative times for thoracoscopic repair (TR) of congenital diaphragmatic hernia (CDH) compared to open repair (OR). An updated meta-analysis was conducted to re-evaluate the surgical outcomes of TR.MethodsA comprehensive literature search comparing TR and OR in neonates was performed in accordance with the PRISMA statement (PROSPERO: CRD42020166588).ResultsFourteen studies were selected for quantitative analysis, including a total of 709 patients (TR: 308 cases, OR: 401 cases). The recurrence rate was higher [Odds ratio: 4.03, 95% CI (2.21, 7.36), p < 0.001] and operative times (minutes) were longer [Mean Difference (MD): 43.96, 95% CI (24.70, 63.22), p < 0.001] for TR compared to OR. A significant reduction in the occurrence of postoperative bowel obstruction was observed in TR (5.0%) compared to OR (14.8%) [Odds ratio: 0.42, 95% CI (0.20, 0.89), p = 0.02].ConclusionsTR remains associated with higher recurrence rates and longer operative times. However, the reduced risk of postoperative bowel obstruction suggests potential long-term benefits. This study emphasizes the importance of meticulous patient selection for TR to mitigate detrimental effects on patients with severe disease.

| S-EPMC11233350 | biostudies-literature

Congenital diaphragmatic hernia.

Project description:Congenital Diaphragmatic Hernia (CDH) is defined by the presence of an orifice in the diaphragm, more often left and posterolateral that permits the herniation of abdominal contents into the thorax. The lungs are hypoplastic and have abnormal vessels that cause respiratory insufficiency and persistent pulmonary hypertension with high mortality. About one third of cases have cardiovascular malformations and lesser proportions have skeletal, neural, genitourinary, gastrointestinal or other defects. CDH can be a component of Pallister-Killian, Fryns, Ghersoni-Baruch, WAGR, Denys-Drash, Brachman-De Lange, Donnai-Barrow or Wolf-Hirschhorn syndromes. Some chromosomal anomalies involve CDH as well. The incidence is < 5 in 10,000 live-births. The etiology is unknown although clinical, genetic and experimental evidence points to disturbances in the retinoid-signaling pathway during organogenesis. Antenatal diagnosis is often made and this allows prenatal management (open correction of the hernia in the past and reversible fetoscopic tracheal obstruction nowadays) that may be indicated in cases with severe lung hypoplasia and grim prognosis. Treatment after birth requires all the refinements of critical care including extracorporeal membrane oxygenation prior to surgical correction. The best hospital series report 80% survival but it remains around 50% in population-based studies. Chronic respiratory tract disease, neurodevelopmental problems, neurosensorial hearing loss and gastroesophageal reflux are common problems in survivors. Much more research on several aspects of this severe condition is warranted.

| S-EPMC3261088 | biostudies-literature

Functional and structural evaluation in the lungs of children with repaired congenital diaphragmatic hernia.

Project description:BackgroundTo evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors.MethodsChildren with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Main bronchus diameters and lung volumes (total, left/right) were measured. According to total lung volume (TLV) relative to body surface area, children were grouped into TLV ≥ 50 group and TLV < 50 group and the associations with perinatal-neonatal factors were analyzed.ResultsOf the 28 children (mean age, 6.2 ± 0.2 years) with left-sided CDH, 7 (25%) had abnormal pulmonary function, of whom 6 (87%) showed restrictive patterns. All pulmonary functions except FEF25-75% were worse than those in matched healthy control group. Worse pulmonary function was significantly associated with small head and abdominal circumferences at birth. The mean TLV was 1339.1 ± 363.9 mL and LLV/TLV was 47.9 ± 2.5 mL. Children with abnormal pulmonary function were more likely to have smaller lung volumes. In multivariate analysis, abdominal circumference at birth was significantly associated with abnormal lung volume.ConclusionsA quarter of children with repaired CDH showed abnormal pulmonary function. Small abdominal circumference at birth was associated with abnormal pulmonary function and lower TLV. .

| S-EPMC7947149 | biostudies-literature

Surgical management of the diaphragmatic defect in congenital diaphragmatic hernia: a contemporary review.

Project description:Worldwide, 150 children are born each day with congenital diaphragmatic hernia (CDH), a diaphragmatic defect with concomitant abnormal lung development. Patients with CDH with large defects are particularly challenging to treat, have the highest mortality, and are at significant risk of long-term complications. Advances in prenatal and neonatal treatments have improved survival in high-risk patients with CDH, but surgical treatment of large defects lacks standardization. Open repair by an abdominal approach has long been considered the traditional procedure, but the type of defect repair (patch or muscle flap) and patch material (non-absorbable, synthetic or absorbable, biological) remain subjects of debate. Increased experience and improved techniques in minimally invasive surgery (MIS) have expanded selection criteria for thoracoscopic defect repair in cardiopulmonary stable patients with small defects. However, the application of MIS to repair large defects remains controversial due to increased recurrence rates and unknown long-term effects of perioperative hypercapnia and acidosis resulting from capnothorax and reduced ventilation. Current recommendations on the surgical management rely on cohort studies of varying patient numbers and data on the long-term outcomes are sparse. Here, we discuss surgical approaches for diaphragmatic defect repair highlighting advancements, and knowledge gaps in surgical techniques (open surgery and MIS), patch materials and muscle flaps for large defects, as well as procedural adjuncts and management of CDH variants.

| S-EPMC11340723 | biostudies-literature

Congenital Diaphragmatic hernia - a review.

Project description:Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes. Delivery of infants with CDH is recommended close to term gestation. Immediate management at birth includes bowel decompression, avoidance of mask ventilation and endotracheal tube placement if required. The main focus of management includes gentle ventilation, hemodynamic monitoring and treatment of pulmonary hypertension followed by surgery. Although inhaled nitric oxide is not approved by FDA for the treatment of PPHN induced by CDH, it is commonly used. Extracorporeal membrane oxygenation (ECMO) is typically considered after failure of conventional medical management for infants ≥ 34 weeks' gestation or with weight >2 kg with CDH and no associated major lethal anomalies. Multiple factors such as prematurity, associated abnormalities, severity of PPHN, type of repair and need for ECMO can affect the survival of an infant with CDH. With advances in the management of CDH, the overall survival has improved and has been reported to be 70-90% in non-ECMO infants and up to 50% in infants who undergo ECMO.

| S-EPMC5356475 | biostudies-literature

Rabbit Model of Fetal Tracheal Occlusion for Congenital Diaphragmatic Hernia

Project description:Analysis of embronic day 30.5 (E30.5) fetal rabbit left lung after creation of diapragmatic hernia (DH) at E25 and tracheal occlusion (TO) at E27. The fetuses of timed pregnant New Zealand rabbits were subjected to creation of a left diaphragmatic hernia at E25 with or without tracheal occlusion at E27. At E30.5, the fetuses were sacrificed with collection of the lateral aspect of the left upper lobe for NextGen mRNA sequencing.

2016-07-26 | E-GEOD-84811 | biostudies-arrayexpress

Genetic causes of congenital diaphragmatic hernia.

Project description:Congenital diaphragmatic hernia (CDH) is a moderately prevalent birth defect that, despite advances in neonatal care, is still a significant cause of infant death, and surviving patients have significant morbidity. The goal of ongoing research to elucidate the genetic causes of CDH is to develop better treatment and ultimately prevention. CDH is a complex developmental defect that is etiologically heterogeneous. This review summarizes the recurrent genetic causes of CDH including aneuploidies, chromosome copy number variants, and single gene mutations. It also discusses strategies for genetic evaluation and genetic counseling in an era of rapidly evolving technologies in clinical genetic diagnostics.

| S-EPMC4259843 | biostudies-literature

Genetic factors in congenital diaphragmatic hernia.

Project description:Congenital diaphragmatic hernia (CDH) is a relatively common birth defect associated with high mortality and morbidity. Although the exact etiology of most cases of CDH remains unknown, there is a growing body of evidence that genetic factors play an important role in the development of CDH. In this review, we examine key findings that are likely to form the basis for future research in this field. Specific topics include a short overview of normal and abnormal diaphragm development, a discussion of syndromic forms of CDH, a detailed review of chromosomal regions recurrently altered in CDH, a description of the retinoid hypothesis of CDH, and evidence of the roles of specific genes in the development of CDH.

| S-EPMC1852742 | biostudies-literature

FBN1 contributing to familial congenital diaphragmatic hernia.

Project description:Congenital diaphragmatic hernia (CDH) is a relatively common, life--threatening birth defect. We present a family with recurrent CDH--paraesophageal and central--for whom exome sequencing (ES) revealed a frameshift mutation (c.4969_4970insA, p.Ile1657Asnfs*30) in the fibrillin 1 gene (FBN1) that causes Marfan syndrome. A diagnosis of Marfan syndrome had not been considered previously in this family. However, a review of the literature demonstrated that FBN1 mutations have an unusual pattern of CDH in which paraesophageal hernias are particularly common. Subsequent clinical evaluations revealed evidence for ectopia lentis in affected family members supporting a clinical diagnosis of Marfan syndrome. Since only two other cases of familial CDH have been described in association with FBN1 mutations, we investigated an oligogenic hypothesis by examining ES data for deleterious sequence changes in other CDH-related genes. This search revealed putatively deleterious sequence changes in four other genes that have been shown to cause diaphragm defects in humans and/or mice--FREM1, DES, PAX3 and MET. It is unclear whether these changes, alone or in aggregate, are contributing to the development of CDH in this family. However, their individual contribution is likely to be small compared to that of the frameshift mutation in FBN1. We conclude that ES can be used to identify both major and minor genetic factors that may contribute to CDH. These results also suggest that ES should be considered in the diagnostic evaluation of individuals and families with CDH, particularly when other diagnostic modalities have failed to reveal a molecular etiology.

| S-EPMC4522925 | biostudies-literature

One-year outcomes for congenital diaphragmatic hernia.

Project description:BackgroundCongenital diaphragmatic hernia (CDH) is a congenital anomaly with high mortality and long-term morbidity. The aim of this study was to benchmark trends in 1-year and hospital volume outcomes for this condition.MethodsThis study included all infants born with CDH in England between 2003 and 2016. This was a retrospective analysis of the Hospital Episode Statistics database. The main outcomes were: 1-year mortality, neonatal length of hospital stay (nLOS), total bed-days at 1 year and readmission rate. The association between hospital volume and outcomes was assessed for specialist paediatric surgery centres.ResultsA total of 2336 infants were included (incidence 2·5 per 10 000 live births). No significant time trends were found in incidence and main outcomes. Some 1491 infants (63·8 per cent) underwent surgical repair. The 1-year mortality rate was 31·2 per cent. Median nLOS and total bed-days were 17 and 19 days respectively. The readmission rate in specialist paediatric centres was 6·3 per cent. Higher mortality was associated with birthweight lower than 1 kg (OR 5·90, 95 per cent c.i. 1·03 to 33·75), gestational age of 36 weeks or less (OR 1·75, 1·12 to 2·75) and black ethnicity (OR 2·13, 1·03 to 4·48). Only 4·0 per cent had extracorporeal membrane oxygenation, which was associated with higher mortality (OR 5·34, 3·01 to 9·46), longer nLOS (OR 3·70, 2·14 to 6·14) and longer total bed-days (OR 3·87, 2·19 to 6·83). Specialist paediatric centres showed variation in 30-day mortality (4·6 per cent with 84 per cent coefficient of variation), nLOS (median 25 (i.q.r. 15-42) days) and total bed-days (median 28 (i.q.r. 16-51) days), but no significant volume-outcome relationship.ConclusionKey outcomes for CDH were similar to those of other developed countries. High variation among specialist paediatric centres was found and should be investigated further to explore the value of regionalization of care.

| S-EPMC6551417 | biostudies-literature

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