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The Cochrane Database of Systematic... Nov 2015Congenital diaphragmatic hernia (CDH), is an uncommon but severe condition in which there is a developmental defect in the fetal diaphragm, resulting in liver and bowel... (Review)
Review
BACKGROUND
Congenital diaphragmatic hernia (CDH), is an uncommon but severe condition in which there is a developmental defect in the fetal diaphragm, resulting in liver and bowel migrating to the chest cavity and impairing lung development and function for the neonate. This condition can be diagnosed during pregnancy and as such, is potentially amenable to in-utero prenatal intervention. Neonatal surgical repair is possible, but even with early surgical repair and improving neonatal management, neonatal morbidity and mortality is high. Prenatal interventions described to date have included maternal antenatal corticosteroid administration and fetal tracheal occlusion, with both methods aiming to improve lung growth and maturity. However surgical procedures have potential maternal complications, as the uterus and amniotic sac are breached in order to gain access to the fetus.
OBJECTIVES
To compare the effects of prenatal versus postnatal interventions for CDH on perinatal mortality and morbidity, longer-term infant outcomes and maternal morbidity, and to compare the effects of different prenatal interventions with each other.
SEARCH METHODS
We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (31 August 2015) and reference lists of retrieved studies.
SELECTION CRITERIA
All published (including those published in abstract form), unpublished, and ongoing randomised controlled trials comparing prenatal and postnatal interventions for fetuses with CDH. Quasi-RCTs were eligible for inclusion but none were identified. Trials using a cross-over design are not eligible for inclusion.
DATA COLLECTION AND ANALYSIS
Two review authors evaluated trials for inclusion and methodological quality without consideration of their results according to the stated eligibility criteria and extracted data independently. Data were checked for accuracy.
MAIN RESULTS
We identified 11 studies for potential inclusion. Of those, we included three studies involving 97 women. Two additional studies are ongoing.Two trials examined in-utero fetal tracheal occlusion with standard (postnatal) care in fetuses with severe diaphragmatic hernia. Whilst the trials utilised fetal interventions that were similar, there were important differences in how access was gained to the fetus and in the timing and mode of delivery. Therefore, we did not combine these trials in meta-analysis and the results are examined in separate comparisons. One trial examined the effect of antenatal corticosteroids versus placebo. Overall, the methodological quality of the trials was variable and no data were available for a number of this review's secondary outcomes. In-utero fetal occlusion by maternal laparotomy versus standard postnatal management (one trial, 24 women)For the primary infant outcome (perinatal mortality), there were no data suitable for inclusion in the analysis. There was no difference between groups in terms of long-term infant survival (risk ratio (RR) 1.06, 95% confidence interval (CI) 0.66 to 1.69). In-utero fetal occlusion by minimally invasive fetoscopy versus standard postnatal management (one trial, 41 women)The primary infant outcome (perinatal mortality) was not reported. Minimally invasive fetoscopy was associated with a small reduction in the mean gestational age at birth (mean difference (MD) -1.80 weeks, 95% CI -3.13 to -0.47), but there was no clear difference in the risk of preterm birth before 37 weeks (RR 1.75, 95% CI 0.78 to 3.92). Long-term infant survival (three to six months) (RR 10.50, 95% CI 1.48 to 74.71) was increased with the intervention when compared with standard management, and there was a corresponding reduction in pulmonary hypertension (RR 0.58, 95% CI 0.36 to 0.93) associated with the intervention. There was no difference between groups in terms of preterm ruptured membranes (< 37 weeks) (RR 1.47, 95% CI 0.56 to 3.88) or maternal infectious morbidity (RR 3.14, 95% CI 0.14 to 72.92), and there were no maternal blood transfusions. Antenatal corticosteroids versus placebo (one trial, 32 women)We also included one trial (involving 32 women) examining the effect of antenatal corticosteroids versus placebo. There was no clear difference in the incidence of perinatal mortality (our primary infant outcome) between the group of women who received antenatal corticosteroids and the placebo control (RR 1.24, 95% CI 0.50 to 3.08). Data (mean only) were reported for two of our secondary outcomes (mechanical ventilation and days of hospital admission) but standard deviations (SDs) were not provided. For the purposes of this review and to permit further analysis we have estimated the SDs based on the reported P values reported in the trial report, although our estimation does assume that the SD is the same in both the intervention and control groups. There were no differences between the antenatal corticosteroid group and the placebo control in terms of days of mechanical ventilation (MD 18.00 days, 95% CI -14.77 to 50.77) or days of hospital admission (MD 17.00 days, 95% CI -13.93 to 47.93) .
AUTHORS' CONCLUSIONS
There is currently insufficient evidence to recommend in-utero intervention for fetuses with CDH as a part of routine clinical practice. We identified three small studies, with only one study adequately reporting on the primary outcome of this review - perinatal mortality, and there were few data pertaining to many of this review's secondary outcomes.WIth regard to the administration of antenatal corticosteroids, there remains a gap in current research, and a large multicentre trial with adequate statistical power should be undertaken to answer this unresolved question. More studies are needed to further examine the effect of in-utero fetal tracheal occlusion on important neonatal outcomes and long-term infant survival and health. Long-term follow-up is of particular importance, and should include morbidity and mortality measures. Further studies should examine the benefits of an in-utero intervention on subgroups with moderate and severe congenital diaphragmatic hernia. Indeed, there are three ongoing studies, being conducted by European, North and South American fetal medicine centres, which will contribute to this gap. Ongoing research and any implementation into clinical practice should include standardisation of the procedure, inclusion criteria and long-term childhood follow-up.
Topics: Adrenal Cortex Hormones; Female; Fetal Membranes, Premature Rupture; Fetoscopy; Gestational Age; Hernias, Diaphragmatic, Congenital; Hospitalization; Humans; Hypertension; Infant, Newborn; Laparoscopy; Perinatal Mortality; Pregnancy; Prenatal Care; Randomized Controlled Trials as Topic; Respiration, Artificial; Trachea
PubMed: 26611822
DOI: 10.1002/14651858.CD008925.pub2 -
Pediatric Critical Care Medicine : a... Aug 2018The goal of this systematic review of the literature was to summarize neurologic outcomes following neonatal and pediatric extracorporeal membrane oxygenation.
OBJECTIVES
The goal of this systematic review of the literature was to summarize neurologic outcomes following neonatal and pediatric extracorporeal membrane oxygenation.
DATA SOURCES
We conducted electronic searches of PubMed, Scopus, Web of Science, CINAHL, Cochrane, and EMBASE.
STUDY SELECTION
Inclusion criteria included publication dates 2000-2016, patient ages 0-18 years, and use of standardized measures to evaluate outcomes after extracorporeal membrane oxygenation.
DATA EXTRACTION
We identified 3,497 unique citations; 60 full-text articles were included in the final review.
DATA SYNTHESIS
Studies evaluated patients with congenital diaphragmatic hernia (7), cardiac disease (8), cardiac arrest (13), and mixed populations (32). Follow-up was conducted at hospital discharge in 10 studies (17%) and at a median of 26 months (interquartile range, 8-61 mo) after extracorporeal membrane oxygenation in 50 studies (83%). We found 55 outcome measures that assessed overall health and function (4), global cognitive ability (7), development (4), motor function (5), adaptive function (2), behavior/mood (6), hearing (2), quality of life (2), school achievement (5), speech and language (6), learning and memory (4), and attention and executive function (8). Overall, 10% to as many as 50% of children scored more than 2 SDS below the population mean on cognitive testing. Behavior problems were identified in 16-46% of children tested, and severe motor impairment was reported in 12% of children. Quality of life of former extracorporeal membrane oxygenation patients evaluated at school age or adolescence ranged from similar to healthy peers, to 31-53% having scores more than 1 SD below the population mean.
CONCLUSIONS
This systematic review of the literature suggests that children who have undergone extracorporeal membrane oxygenation suffer from a wide range of disabilities. A meta-analysis was not feasible due to heterogeneity in pathologies, outcome measures, and age at follow-up, underscoring the importance of developing and employing a core set of outcomes measures in future extracorporeal membrane oxygenation studies.
Topics: Adolescent; Child; Child, Preschool; Extracorporeal Membrane Oxygenation; Humans; Infant; Infant, Newborn; Neurocognitive Disorders; Outcome Assessment, Health Care; Quality of Life
PubMed: 29894448
DOI: 10.1097/PCC.0000000000001612 -
Scientific Reports Jun 2021Large studies comparing the surgical outcome of primary versus patch repair in congenital diaphragmatic hernia (CDH) patients are rare. This study aims to evaluate the... (Meta-Analysis)
Meta-Analysis
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.
Topics: Chylothorax; Hernias, Diaphragmatic, Congenital; Herniorrhaphy; Humans; Incidence; Infant, Newborn; Intestinal Obstruction; Male; Morbidity; Postoperative Complications; Risk Factors; Treatment Outcome
PubMed: 34135386
DOI: 10.1038/s41598-021-91908-7 -
BMJ Paediatrics Open Jul 2023Neonatal care is commonly regionalised, meaning specialist services are only available at certain units. Consequently, infants with surgical conditions needing...
BACKGROUND
Neonatal care is commonly regionalised, meaning specialist services are only available at certain units. Consequently, infants with surgical conditions needing specialist care who are born in non-surgical centres require postnatal transfer. Best practice models advocate for colocated maternity and surgical services as the place of birth for infants with antenatally diagnosed congenital conditions to avoid postnatal transfers. We conducted a systematic review to explore the association between location of birth and short-term outcomes of babies with gastroschisis, congenital diaphragmatic hernia (CDH) and oesophageal atresia with or without tracheo-oesophageal fistula (TOF/OA).
METHODS
We searched MEDLINE, CINAHL, Web of Science and SCOPUS databases for studies from high income countries comparing outcomes for infants with gastroschisis, CDH or TOF/OA based on their place of delivery. Outcomes of interest included mortality, length of stay, age at first feed, comorbidities and duration of parenteral nutrition. We assessed study quality using the Newcastle-Ottawa Scale. We present a narrative synthesis of our findings.
RESULTS
Nineteen cohort studies compared outcomes of babies with one of gastroschisis, CDH or TOF/OA. Heterogeneity across the studies precluded meta-analysis. Eight studies carried out case-mix adjustments. Overall, we found conflicting evidence. There is limited evidence to suggest that birth in a maternity unit with a colocated surgical centre was associated with a reduction in mortality for CDH and decreased length of stay for gastroschisis.
CONCLUSIONS
There is little evidence to suggest that delivery in colocated maternity-surgical services may be associated with shortened length of stay and reduced mortality. Our findings are limited by significant heterogeneity, potential for bias and paucity of strong evidence. This supports the need for further research to investigate the impact of birth location on outcomes for babies with congenital surgical conditions and inform future design of neonatal care systems.
PROSPERO REGISTRATION NUMBER
CRD42022329090.
Topics: Infant; Infant, Newborn; Humans; Female; Pregnancy; Hernias, Diaphragmatic, Congenital; Gastroschisis; Cohort Studies; Esophageal Atresia; Tracheoesophageal Fistula
PubMed: 37474200
DOI: 10.1136/bmjpo-2023-002007 -
Ultrasound in Obstetrics & Gynecology :... Sep 2021To compare the perinatal outcomes between pregnancies with and those without iatrogenic chorioamniotic separation (iCAS) following fetoscopic intervention. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To compare the perinatal outcomes between pregnancies with and those without iatrogenic chorioamniotic separation (iCAS) following fetoscopic intervention.
METHODS
We performed a search in PubMed, EMBASE, Scopus, Web of Science and Google Scholar from inception up to December 2020 for studies comparing perinatal outcomes between pregnancies that developed and those that did not develop iCAS after fetoscopic intervention for twin-to-twin transfusion syndrome (TTTS), open neural tube defect (ONTD) or congenital diaphragmatic hernia. A random-effects model was used to pool the mean differences (MD) or odds ratios (OR) and the corresponding 95% CI. The primary outcome was neonatal survival. Secondary outcomes included gestational age (GA) at intervention and at delivery, interval from intervention to delivery and incidence of preterm prelabor rupture of membranes (PPROM) and preterm delivery. The methodological quality of the included studies was evaluated using the Newcastle-Ottawa scale.
RESULTS
The search identified 348 records, of which seven studies (six on fetoscopic laser photocoagulation (FLP) for TTTS and one on fetoscopic repair for ONTD) assessed the perinatal outcomes of pregnancies that developed iCAS after fetoscopic intervention. Given that only one study reported on fetoscopic ONTD repair, the meta-analysis was limited to TTTS pregnancies and included six studies (total of 1881 pregnancies). Pregnancies that developed iCAS after FLP for TTTS, compared with those that did not, had significantly lower GA at the time of intervention (weeks) (MD, -1.07 (95% CI, -1.89 to -0.24); P = 0.01) and at delivery (weeks) (MD, -1.74 (95% CI, -3.13 to -0.34); P = 0.01) and significantly lower neonatal survival (OR, 0.41 (95% CI, 0.24-0.70); P = 0.001). In addition, development of iCAS after FLP for TTTS increased significantly the risk for PPROM < 34 weeks' gestation (OR, 3.98 (95% CI, 1.76-9.03); P < 0.001) and preterm delivery < 32 weeks (OR, 1.80 (95% CI, 1.16-2.80); P = 0.008).
CONCLUSIONS
iCAS is a common complication after FLP for TTTS. In patients undergoing FLP for TTTS, iCAS develops more often with earlier GA at intervention and is associated with earlier GA at delivery, higher risk of PPROM < 34 weeks' gestation and preterm delivery < 32 weeks and lower neonatal survival. Given the limitations of this meta-analysis and lack of literature reporting on other types of fetoscopic intervention, the presented findings should be interpreted with caution and should not be generalized to fetoscopic procedures used to treat other fetal conditions. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Adult; Delivery, Obstetric; Female; Fetal Membranes, Premature Rupture; Fetofetal Transfusion; Fetoscopy; Gestational Age; Hernias, Diaphragmatic, Congenital; Humans; Iatrogenic Disease; Incidence; Infant, Newborn; Neural Tube Defects; Odds Ratio; Postoperative Complications; Pregnancy; Pregnancy Outcome; Pregnancy, Twin; Premature Birth
PubMed: 33428299
DOI: 10.1002/uog.23588 -
Langenbeck's Archives of Surgery Jun 2024The objective of this study is to compare the operative time, intraoperative complications, length of stay, readmission rates, overall complications, mortality, and cost... (Meta-Analysis)
Meta-Analysis Comparative Study
PURPOSE
The objective of this study is to compare the operative time, intraoperative complications, length of stay, readmission rates, overall complications, mortality, and cost associated with Robotic Surgery (RS) and Laparascopic Surgery (LS) in anti-reflux and hiatal hernia surgery.
METHODS
A comprehensive literature search was conducted using MEDLINE (via PubMed), Web of Science and Scopus databases. Studies comparing short-term outcomes and cost between RS and LS in patients with anti-reflux and hiatal hernia were included. Data on operative time, complications, length of stay, readmission rates, overall complications, mortality, and cost were extracted. Quality assessment of the included studies was performed using the MINORS scale.
RESULTS
Fourteen retrospective observational studies involving a total of 555,368 participants were included in the meta-analysis. The results showed no statistically significant difference in operative time, intraoperative complications, length of stay, readmission rates, overall complications, and mortality between RS and LS. However, LS was associated with lower costs compared to RS.
CONCLUSION
This systematic review and meta-analysis demonstrates that RS has non-inferior short-term outcomes in anti-reflux and hiatal hernia surgery, compared to LS. LS is more cost-effective, but RS offers potential benefits such as improved visualization and enhanced surgical techniques. Further research, including randomized controlled trials and long-term outcome studies, is needed to validate and refine these findings.
Topics: Humans; Hernia, Hiatal; Robotic Surgical Procedures; Laparoscopy; Gastroesophageal Reflux; Operative Time; Herniorrhaphy; Treatment Outcome; Length of Stay; Fundoplication; Patient Readmission; Postoperative Complications
PubMed: 38842610
DOI: 10.1007/s00423-024-03368-y -
International Journal of Hyperthermia :... 2023The treatment of hepatic hemangioma includes surgical resection, radiofrequency ablation and Transarterial embolization. However, complications, mortality and... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The treatment of hepatic hemangioma includes surgical resection, radiofrequency ablation and Transarterial embolization. However, complications, mortality and compromised effectiveness limit their applications. Microwaves with effective heating generation and short ablation time become a promising treatment. The aim of this study is to conduct systematic review and meta-analyses to evaluate the effectiveness of Microwave Ablation (MWA) for the treatment of hepatic hemangioma.
METHODS
A systematic literature review was conducted in PubMed. Main outcomes were defined as hemangioma decreases in diameters and volume changes post-MWA. Conventional random-effect meta-analysis technique was applied to analyze the pooled data, and meta-regression model was established to explore the association among factors.
RESULTS
There were nine studies with a total of 501 patients retrieved. The pooled estimate of mean differences and 95% CI of hemangioma decreases after MWA treatment in diameter and in volume change (%) were 3.009 cm and (1.856, 4.161), and 53.169% and (51.274, 55.065), respectively. The pooled estimates of liver enzyme, ALT and AST, elevation were 219.905 with 95%CI (160.860, 278.949) and 315.679 with 95%CI (226.961, 404.397), respectively. Major complications were defined as acute kidney injury (AKI), pleural effusion, diaphragmatic hernia, and jaundice that needed to be treated, and the pooled incidence was 0.017 with 95% CI of (0.006, 0.029). No mortality related to MWA was reported. Meta-regression showed ablation time was associated with pre-operative lesion size ( = .001).
CONCLUSION
MWA is effective and safe in treatment of hepatic hemangioma, and our study suggests that hemangioma size should be investigated in the future MWA pretreatment difficulty scoring system study.
Topics: Humans; Microwaves; Catheter Ablation; Liver Neoplasms; Radiofrequency Ablation; Hemangioma; Treatment Outcome
PubMed: 36535918
DOI: 10.1080/02656736.2022.2146214 -
International Journal of Surgery... Jun 2022
Meta-Analysis
A commentary on "Postoperative hiatal herniation after open vs. minimally invasive esophagectomy; a systematic review and meta-analysis" (Int J Surg 2021;93:106046). Paraconduit hiatal hernia: A relevant complication after radical esophagectomy with reconstruction via gastric pull-up. Suggestions...
Topics: Esophageal Neoplasms; Esophagectomy; Hernia, Hiatal; Humans; Laparoscopy; Minimally Invasive Surgical Procedures; Postoperative Complications; Postoperative Period; Retrospective Studies
PubMed: 35589050
DOI: 10.1016/j.ijsu.2022.106659 -
Genes Aug 2023In 1990, Gorlin et al. described four types of craniofacial duplications: (1) single mouth with duplication of the maxillary arch; (2) supernumerary mouth laterally...
In 1990, Gorlin et al. described four types of craniofacial duplications: (1) single mouth with duplication of the maxillary arch; (2) supernumerary mouth laterally placed with rudimentary segments; (3) single mouth with replication of the mandibular segments; and (4) true facial duplication, namely diprosopus. We describe a newborn born with wide-spaced eyes, a very broad nose, and two separate mouths. Workup revealed the absence of the corpus callosum and the presence of a brain midline lipoma, wide sutures, and a Chiari I malformation with cerebellar herniation. We conducted a systematic review of the literature and compared all the cases described as diprosopus. In 96% of these, the central nervous system is affected, with anencephaly being the most commonly associated abnormality. Other associated anomalies include cardiac malformations (86%), cleft palate (63%), diaphragmatic hernia (13%), and disorder of sex development (DSD) (13%). Although the facial features are those that first strike the eye, the almost obligate presence of cerebral malformations suggests a disruptive event in the cephalic pole of the forming embryo. No major monogenic contribution has been recognized today for this type of malformation.
Topics: Infant, Newborn; Humans; Cleft Palate; Face; Brain; Central Nervous System
PubMed: 37761885
DOI: 10.3390/genes14091745 -
PLoS Medicine Sep 2020Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing.... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing. Although infant survival of children born with congenital anomalies has improved for many anomaly types in recent decades, there is less evidence on survival beyond infancy. We aimed to systematically review, summarise, and quantify the existing population-based data on long-term survival of individuals born with specific major congenital anomalies and examine the factors associated with survival.
METHODS AND FINDINGS
Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, and citations of the included articles for studies published 1 January 1995 to 30 April 2020 were searched. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate. We included original population-based studies that reported long-term survival (beyond 1 year of life) of children born with a major congenital anomaly with the follow-up starting from birth that were published in the English language as peer-reviewed papers. Studies on congenital heart defects (CHDs) were excluded because of a recent systematic review of population-based studies of CHD survival. Meta-analysis was performed to pool survival estimates, accounting for trends over time. Of 10,888 identified articles, 55 (n = 367,801 live births) met the inclusion criteria and were summarised narratively, 41 studies (n = 54,676) investigating eight congenital anomaly types (spina bifida [n = 7,422], encephalocele [n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6,176], gastroschisis [n = 4,845], Down syndrome by presence of CHD [n = 22,317], and trisomy 18 [n = 2,174]) were included in the meta-analysis. These studies covered birth years from 1970 to 2015. Survival for children with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, and Down syndrome with an associated CHD has significantly improved over time, with the pooled odds ratios (ORs) of surviving per 10-year increase in birth year being OR = 1.34 (95% confidence interval [95% CI] 1.24-1.46), OR = 1.50 (95% CI 1.38-1.62), OR = 1.62 (95% CI 1.28-2.05), OR = 1.57 (95% CI 1.37-1.81), OR = 1.24 (95% CI 1.02-1.5), and OR = 1.99 (95% CI 1.67-2.37), respectively (p < 0.001 for all, except for gastroschisis [p = 0.029]). There was no observed improvement for children with encephalocele (OR = 0.98, 95% CI 0.95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96, 95% CI 0.88-1.03, p = 0.26). The presence of additional structural anomalies, low birth weight, and earlier year of birth were the most commonly reported predictors of reduced survival for any congenital anomaly type. The main limitation of the meta-analysis was the small number of studies and the small size of the cohorts, which limited the predictive capabilities of the models resulting in wide confidence intervals.
CONCLUSIONS
This systematic review and meta-analysis summarises estimates of long-term survival associated with major congenital anomalies. We report a significant improvement in survival of children with specific congenital anomalies over the last few decades and predict survival estimates up to 20 years of age for those born in 2020. This information is important for the planning and delivery of specialised medical, social, and education services and for counselling affected families. This trial was registered on the PROSPERO database (CRD42017074675).
Topics: Adult; Age Factors; Child; Child Mortality; Child, Preschool; Congenital Abnormalities; Female; Humans; Infant; Live Birth; Parturition; Pregnancy; Registries; Young Adult
PubMed: 32986711
DOI: 10.1371/journal.pmed.1003356